Screening for Fabry disease in patients with left ventricular noncompaction | Revista Portuguesa de Cardiologia (English edition)

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Portugal" "etiqueta" => "l" "identificador" => "aff0060" ] 12 => array:3 [ "entidad" => "Genetics Department, Reference Center on Lysosomal Storage Disorders, Hospital Senhora da Oliveira, Guimarães, Portugal" "etiqueta" => "m" "identificador" => "aff0065" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Rastreio de doença de Fabry em doentes com não compactação do ventrículo esquerdo" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 698 "Ancho" => 755 "Tamanyo" => 31421 ] ] "descripcion" => array:1 [ "en" => "Left ventricular noncompaction in a Fabry disease patient: cardiac magnetic resonance imaging showing hypertrabeculation mostly affecting the left ventricular apical region and lateral wall in apical 4-chamber view." ] ] ] "textoCompleto" => "IntroductionFabry disease (OMIM 301500) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene, which is located on Xq22.1 and codes for the enzyme alpha-galactosidase A (α-Gal A). Deficiency in the enzyme activity of α-Gal A leads to the lysosomal accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids, causing multiorgan damage.<a class="elsevierStyleCrossRef" href="#bib0230">1</a>Fabry disease is a rare condition, estimated to occur in 1 in 3100 hemizygous males.<a class="elsevierStyleCrossRef" href="#bib0235">2</a> The diagnosis in males is based on biochemical determination of α-Gal A activity. In heterozygous females, α-Gal A activity may be normal, so diagnosis requires molecular analysis of the GLA gene.<a class="elsevierStyleCrossRef" href="#bib0230">1</a> Clinical manifestations may arise early, in childhood or adolescence, and include acroparesthesias, cornea verticillata, angiokeratomas and microalbuminuria or proteinuria. In adulthood, patients may develop sensorineural deafness, renal failure, cerebrovascular events and cardiac manifestations.<a class="elsevierStyleCrossRef" href="#bib0230">1</a>Fabry disease affects the heart, causing left ventricular hypertrophy (LVH) and fibrosis, heart failure, angina, valve thickening or regurgitation, dysrhythmias, cardiac conduction abnormalities and sudden death.<a class="elsevierStyleCrossRefs" href="#bib0230">1,3</a> The main cardiac manifestation is hypertrophic cardiomyopathy, which occurs in 43% of male patients (mean age of onset 39±10 years) and 26% of female patients (mean age of onset 50±11 years).<a class="elsevierStyleCrossRef" href="#bib0245">4</a> LVH in Fabry disease is usually non-obstructive and symmetrical<a class="elsevierStyleCrossRef" href="#bib0240">3</a> with prominent papillary muscles<a class="elsevierStyleCrossRef" href="#bib0250">5</a> and, in nearly half of cases, myocardial late gadolinium enhancement is typically observed in the mid-wall of the inferolateral basal segments of the left ventricle.<a class="elsevierStyleCrossRef" href="#bib0255">6</a>Left ventricular noncompaction (LVNC) is morphologically characterized by prominent left ventricular (LV) trabeculations and deep intertrabecular recesses communicating with the LV cavity, located in the apical and lateral regions of the left ventricle, distal to the papillary muscles. The myocardium has a two-layer appearance, composed of a thin compacted epicardial layer and a thick spongy noncompacted endocardial layer.<a class="elsevierStyleCrossRef" href="#bib0260">7</a>LVNC has been found in 0.045% of adult individuals referred for echocardiography<a class="elsevierStyleCrossRef" href="#bib0265">8</a> and 0.029% of children admitted for heart failure due to cardiomyopathy.<a class="elsevierStyleCrossRef" href="#bib0270">9</a> It predominantly affects males<a class="elsevierStyleCrossRef" href="#bib0265">8</a> and individuals of African origin.<a class="elsevierStyleCrossRef" href="#bib0275">10</a> First described on autopsy in 1969 by Feldt et al.<a class="elsevierStyleCrossRef" href="#bib0280">11</a> and on echocardiography by Engberding et al. in 1984,<a class="elsevierStyleCrossRef" href="#bib0285">12</a> it is most commonly diagnosed by transthoracic echocardiography or cardiac magnetic resonance imaging (MRI). Several diagnostic criteria have been proposed using echocardiography or cardiac MRI<a class="elsevierStyleCrossRefs" href="#bib0290">13–19</a> (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>).<elsevierMultimedia ident="tbl0005"></elsevierMultimedia>Patients with LVNC may be asymptomatic or develop complications such as heart failure, thromboembolism or ventricular dysrhythmias and sudden death.<a class="elsevierStyleCrossRef" href="#bib0325">20</a>The etiology and pathogenesis of LVNC are unknown. It has been hypothesized to result from a disturbance of the compaction process of the LV myocardium during early development, but growing evidence suggests that, although the papillary muscles are formed by compaction of pre-existing trabeculations, these trabeculations contribute little, if anything, to the compacted layer of the ventricular walls.<a class="elsevierStyleCrossRef" href="#bib0330">21</a> Moreover, not all cases are congenital and LVNC may be acquired at any stage of life after birth.<a class="elsevierStyleCrossRef" href="#bib0260">7</a>LVNC is characterized by marked genetic heterogeneity. Mutations have been described in genes that code for proteins in the mitochondria (tafazzins), Z line (ZASP/Cypher), cytoskeleton (alpha-dystrobrevin), nuclear lamina (lamin A/C) and sarcomere (beta-myosin heavy chain, alpha-cardiac actin and cardiac troponin T).<a class="elsevierStyleCrossRef" href="#bib0260">7</a> However, the causal relationship between these mutations and LVNC remains to be established.LVNC has been reported in patients with congenital cardiac abnormalities, such as LV outflow tract abnormalities, Ebstein anomaly, tetralogy of Fallot and double outlet right ventricle; chromosomal defects, such as trisomy 13, Cornelia de Lange, DiGeorge, Turner and 5q minus syndromes; and neuromuscular disorders, such as mitochondrial myopathies, Barth syndrome, zaspopathy, myotonic dystrophy type 1, dystrobrevinopathy, and Emery-Dreifuss muscular dystrophy.<a class="elsevierStyleCrossRef" href="#bib0260">7</a> LVNC has also been reported in patients with storage disorders such as glycogen storage disease types Ib and IV,<a class="elsevierStyleCrossRefs" href="#bib0335">22,23</a> Danon disease<a class="elsevierStyleCrossRef" href="#bib0345">24</a> and amyloidosis.<a class="elsevierStyleCrossRef" href="#bib0350">25</a>In 2011, we reported the first case of LVNC in a Fabry disease patient.<a class="elsevierStyleCrossRef" href="#bib0355">26</a> This was a young asymptomatic female who fulfilled the Jenni echocardiographic criteria and the Petersen cardiac MRI criteria for LVNC. She had been diagnosed with Fabry disease following family screening, and the pathogenic mutation of the GLA gene detected was a missense mutation (p.F113L) that is known to be associated with a predominantly cardiac later-onset phenotype. However, thorough diagnostic study revealed none of the known clinical manifestations of Fabry disease, including LVH, late gadolinium enhancement or other cardiac or extracardiac manifestations, nor did she present LV dilation or dysfunction or any clinical complication of LVNC, such as heart failure, dysrhythmias or thromboembolism. Moreover, all of her relatives with Fabry disease underwent echocardiography and cardiac MRI and none presented LVNC. In this report, we pointed out the limitations of the current diagnostic criteria of LVNC and raised the question whether this was an overdiagnosis of LVNC, a coincidental finding of LVNC and Fabry disease, or if indeed the two conditions were clinically related.<a class="elsevierStyleCrossRefs" href="#bib0355">26,27</a>More recently, two additional cases of LVNC in Fabry disease patients have been reported,<a class="elsevierStyleCrossRefs" href="#bib0365">28–31</a> also in young females. In the second case, the diagnosis of LVNC was based only on cardiac MRI (Petersen criteria), without clear echocardiographic criteria of LVNC due to insufficient quality of the acoustic window. She had classical Fabry disease due to a nonsense GLA gene mutation (p.R220X). As in our case, she also did not present LVH, dilation, dysfunction, late gadolinium enhancement or other cardiac abnormalities, and there was no history of heart failure, dysrhythmias or thromboembolism. There was also no family history of LVNC. She had no clinical manifestations of Fabry disease besides cornea verticillata and angiokeratomas. An endomyocardial biopsy showed deposition of glycosphingolipids in cardiomyocytes and based on this finding the patient was referred for enzyme replacement therapy.<a class="elsevierStyleCrossRefs" href="#bib0365">28,29</a>In the third case, the diagnosis of LVNC was based on cardiac MRI (Petersen criteria) and echocardiography (although it was not reported which specific criteria were fulfilled). She was diagnosed with Fabry disease based on low levels of α-Gal A, but the pathogenic GLA gene mutation was never found. The only clinical manifestations of Fabry disease were angiokeratomas and acroparesthesias. As in the two previous cases, there was no LVH, dilation or dysfunction. Cardiac MRI showed subendocardial late gadolinium enhancement, but its location and extension was not reported. Interestingly, she had Wolff-Parkinson-White syndrome, interpreted by the authors as an unrelated finding, and underwent ablation of a para-Hisian accessory pathway, which was followed by disappearance of the short PR interval and delta waves. However, as in the two previous cases, she had no history of heart failure or thromboembolism. Moreover, there were no available data consistent with a family history of LVNC or Fabry disease.<a class="elsevierStyleCrossRefs" href="#bib0375">30,31</a>Taken together, it remains essential to clarify whether LVNC could be a cardiac manifestation of Fabry disease, given the implications for therapeutic decisions on whether to start enzyme replacement or chaperone therapy, particularly in young asymptomatic females with no other known clinical manifestations of Fabry disease.The only published study to date on Fabry disease screening in patients with LVNC included 26 male patients who fulfilled the Stöllberger echocardiographic criteria for LVNC. Fabry disease screening was based on the enzymatic assay of α-Gal A and found no patients with the disease.<a class="elsevierStyleCrossRef" href="#bib0385">32</a>Herein, we report the results of Fabry disease screening in a larger cohort of patients with LVNC.MethodsPatient populationBetween October 2015 and October 2017, 78 consecutive patients with LVNC were recruited from the cardiology consultation of eight Portuguese hospital centers. The inclusion criteria were adult patients (aged ≥18 years) diagnosed with LVNC based on the presence of any of the current diagnostic criteria of LVNC, by either echocardiography or cardiac MRI, as described in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>. All patients fulfilling the inclusion criteria underwent Fabry disease screening.Fabry disease screeningFabry disease screening was performed at a specialized laboratory (Centogene AG, Rostock, Germany). α-Gal A enzyme activity was measured in dried blood spot samples by fluorometry. Molecular analysis of the GLA gene was performed in males with reduced α-Gal A enzyme activity, i.e., below the normal reference range (<15.3 μmol/l/h), and in all females, independently of α-Gal A activity. The molecular analysis was performed by classical bidirectional Sanger sequencing of the GLA gene and included all exons, exon/intron boundaries and the promoter region.Ethical issuesThis research project was approved by the ethics committees of all the hospital centers included in this study and all patients provided written informed consent.ResultsPatients with LVNC were predominantly males (62.8%). Only four patients (5.1%) were identified as being of African origin. Mean age at diagnosis of LVNC was 64.4±14.1 years. A family history of LVNC was recorded in five patients (6.4%). The patients did not present concomitant neuromuscular disorders or any other medical conditions reported to be associated with LVNC.The diagnosis of LVNC was established by echocardiography in 91.0% of the cases, based on the Jenni criteria in 84.6%, the Stöllberger criteria in 46.2%, the Chin criteria in 21.8% and the three echocardiographic criteria in 20.5% of the cases. Nearly half of the patients presented LV systolic dysfunction (48.7%) and mean LV ejection fraction was 47.2±15.2%. Cardiac MRI was performed in 68 patients (87.2%) and the diagnosis of LVNC was established in 58 patients (85.3%), mainly based on the Petersen criteria (83.8%). The trabeculated LV mass was calculated in only 11 patients, all of them fulfilling the Jacquier criteria of LVNC. The presence of the Stacey or Captur criteria was not assessed in any cardiac MRI. The diagnosis of LVNC was established by both echocardiography and cardiac MRI in 53 of the 68 patients who underwent cardiac MRI (77.9%).LVNC was an incidental finding in 44.9% of the patients. Heart failure was the most common clinical manifestation at the time of diagnosis (34.6%). In the follow-up (53±33 months), heart failure remained the most frequent clinical complication (60.3%), with New York Heart Association functional class ranging from I to IV (I: 8.5%; II: 59.6%; III: 23.4%; IV: 8.5%). Dysrhythmias were also common (35.9%). Twenty-four-hour Holter monitoring was performed in 74 patients (94.9%) and revealed atrial fibrillation in 10 patients (13.5%) and ventricular tachycardia (VT) in 16 patients (21.6%), although only one case exhibited sustained VT. Thromboembolism occurred in nine patients (11.5%), as stroke in eight and as myocardial infarction in one (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>).<elsevierMultimedia ident="tbl0010"></elsevierMultimedia>Twenty-eight patients (35.9%) had undergone genetic study that included a wide range of genes (MYH7: 32.1%; MYBPC3: 30.8%; TNNT2: 28.2%; TNNI3: 19.2%; ACTC1: 28.2%; TPM1: 28.2%; MYL2: 11.5%; MYL3: 11.5%; DTNA: 7.7%; DMD: 10.3%; LDB3: 28.2%; TAZ: 26.9%; LMNA: 24.4%; CSRP3: 24.4%; TCAP: 20.5%; SGCD: 21.8%; PLN: 23.1%; TTN: 2.6%; SCN5A: 1.3%). A pathogenic mutation was found in the MYH7 gene in one patient (c.1003G>C) (unpublished data). Eight genetic variants of uncertain significance were found in six cases: four in MYBPC3 (c.1505G>A; c.2762A>T; c.1624G>C; c.3676C>T), the last two in the same patient; two in TTN (c.16425T>G; c.60259C>G); one in SCN5A (c.393-5C>A); and one in SGCD (c.772-774delAAG) in the same patient carrying two genetic variants of the MYBPC3 gene.The screening of 78 patients with LVNC revealed no additional cases of Fabry disease besides the one already identified.<a class="elsevierStyleCrossRef" href="#bib0355">26</a> This Fabry patient was never under enzyme replacement or chaperone therapy. During the eight-year follow-up, this patient has remained asymptomatic and without known clinical manifestation of Fabry disease or complication of LVNC. Moreover, there were no significant changes regarding the location or extension of LVNC or LV chamber size, mass or function during follow-up (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>).<elsevierMultimedia ident="fig0005"></elsevierMultimedia>DiscussionThis multicenter study of Fabry disease screening in 78 consecutive patients with LVNC revealed no additional Fabry patients besides the single case that had been previously reported by our center, which argues against the hypothesis that LVNC is a clinical manifestation of Fabry disease.The most likely explanation for the finding of LVNC in this Fabry patient is an overdiagnosis of LVNC. Studies have increasingly exposed the lack of specificity of the current diagnostic criteria for LVNC, potentially leading to overdiagnosis.<a class="elsevierStyleCrossRefs" href="#bib0275">10,33,34</a> Moreover, there is growing evidence that LVNC has no independent prognostic impact compared to conventional risk factors, such as LV dilation, dysfunction or fibrosis, and that patients with LVNC without LV dilation, dysfunction or fibrosis have an excellent prognosis.<a class="elsevierStyleCrossRefs" href="#bib0395">34–36</a>In a study of 199 patients referred to a heart failure clinic and 60 healthy controls, Kohli et al. found that 23.6% of the patients and 8.3% of the controls fulfilled one or more echocardiographic criteria for LVNC. Among the heart failure patients with LVNC criteria, 29.8% fulfilled all three echocardiographic criteria and 36.2% fulfilled only a single criterion, with the frequency of each criterion ranging from 12.1% to 18.6%.<a class="elsevierStyleCrossRef" href="#bib0275">10</a> In a large population-based cohort of 1000 subjects from the Multi-Ethnic Study of Atherosclerosis (MESA) registry who underwent cardiac MRI, the Petersen criteria for LVNC were fulfilled in 43% of the subjects without cardiac disease.<a class="elsevierStyleCrossRef" href="#bib0390">33</a> Another study based on the MESA registry including 2742 subjects undergoing cardiac MRI showed that a greater extent of LV trabeculations in asymptomatic individuals appears to be benign and is not associated with deterioration in LV volumes or function over an almost 10-year period.<a class="elsevierStyleCrossRef" href="#bib0405">36</a> In a study including 700 patients referred for cardiac MRI, Ivanov et al. found an LVNC prevalence of 39%, 23%, 25% and 3%, according to the Petersen, Stacey, Jacquier and Captur criteria, respectively. In this study, during a median follow-up of seven years, there were no statistically significant differences in the assessed outcomes (death, ischemic stroke, ventricular tachycardia/fibrillation, and heart failure hospitalization) between patients with and without LVNC, irrespective of the criteria applied.<a class="elsevierStyleCrossRef" href="#bib0395">34</a> Finally, in a study including 113 patients with LVNC according to the Jenni and Petersen criteria, Andreini et al. found that the degree of hypertrabeculation had no prognostic impact over and above LV dilation, dysfunction or late gadolinium enhancement. Indeed, patients without LV dilation, dysfunction or fibrosis had an excellent prognosis at four-year follow-up.<a class="elsevierStyleCrossRef" href="#bib0400">35</a>In light of this evidence, the possibility of an overdiagnosis of LVNC, with no associated prognostic impact, must be considered in these three cases of Fabry disease published in the literature. In fact, all three case reports of LVNC occurred in young females with no family history of LVNC in Fabry or non-Fabry relatives, no LV hypertrophy, dilation or dysfunction, no late gadolinium enhancement suggestive of Fabry disease involvement, no or few clinical manifestations of Fabry disease, and no clinical complications of LVNC.<a class="elsevierStyleCrossRefs" href="#bib0355">26–31</a>Appropriate follow-up would also be required to correctly interpret the significance of LVNC in these Fabry patients. In our case, the patient was never under enzyme replacement or chaperone therapy, because at baseline she did not present any known manifestation of Fabry disease that would constitute a clinical indication to start specific therapy according to the guidelines. During the eight-year follow-up of this patient, no significant change has been documented in the location or extension of LVNC or in LV chamber size, mass or function. Moreover, this patient has remained asymptomatic and with no known clinical manifestation of Fabry disease or complication of LVNC, which supports the hypothesis of an overdiagnosis of LVNC in this Fabry patient.Another possibility is that LVNC and Fabry disease in these patients are coincidental and unrelated conditions. Although the simultaneous finding of two unrelated rare conditions may seem unlikely, it is now known that LVNC has a much higher prevalence<a class="elsevierStyleCrossRefs" href="#bib0275">10,33,34</a> than the previous estimate of 0.045%<a class="elsevierStyleCrossRef" href="#bib0265">8</a> and the currently estimated Fabry disease prevalence of 1 in 3100<a class="elsevierStyleCrossRef" href="#bib0235">2</a> is much higher than the previous estimate of 1 in 117 000.<a class="elsevierStyleCrossRef" href="#bib0410">37</a>The finding of LVNC in a wide variety of congenital cardiac abnormalities, chromosomal defects, neuromuscular disorders<a class="elsevierStyleCrossRef" href="#bib0260">7</a> and storage disorders,<a class="elsevierStyleCrossRefs" href="#bib0335">22–24</a> as well as in elite athletes<a class="elsevierStyleCrossRef" href="#bib0415">38</a> and pregnant women,<a class="elsevierStyleCrossRef" href="#bib0420">39</a> has raised the question of whether LVNC is indeed a distinct cardiomyopathy or simply a common epiphenomenon in cardiac overload conditions. In our case, this young woman was not an athlete, but she had been pregnant once, although we have no record of echocardiograms before, during or just after pregnancy. A history of pregnancy or vigorous exercise was not reported in the other two cases.Finally, there is the possibility that LVNC could be a cardiac manifestation of Fabry disease. However, there is no evidence that Fabry disease causes LVNC by either compensatory or genetic mechanisms. Indeed, although they had Fabry disease, none of these patients had concomitant LVH, dilation or dysfunction that could have led to cardiac overload and hence to LVNC. Furthermore, there is no evidence that the GLA gene mutation, the mutated α-Gal A, or the intramyocardial deposition of glycosphingolipids due to Fabry disease are the cause of or are in any way related to the development of LVNC, and any assumption on this point remains speculative. Lysosomal accumulation of Gb3 begins in utero and has been demonstrated as early as the 17th week of gestation in various fetal cell types, such as cardiomyocytes,<a class="elsevierStyleCrossRef" href="#bib0425">40</a> kidney podocytes,<a class="elsevierStyleCrossRefs" href="#bib0425">40–42</a> hepatocytes,<a class="elsevierStyleCrossRefs" href="#bib0430">41,43</a> corneal epithelial cells<a class="elsevierStyleCrossRef" href="#bib0445">44</a> and myenteric plexus cells.<a class="elsevierStyleCrossRef" href="#bib0435">42</a> Although Gb3 storage begins in utero, clinical manifestations only develop several years later. Therefore, Gb3 deposits are expected findings in biopsies, including endomyocardial biopsies, of all Fabry patients independently of the presence of clinical manifestations, and such deposition should not be interpreted as evidence that LVNC is due to Fabry disease.The only study screening for Fabry disease in patients with LVNC did not reveal any positive case. However, this was a small cohort of 26 male patients and the only criteria for LVNC diagnosis were the Stöllberger echocardiographic criteria.<a class="elsevierStyleCrossRef" href="#bib0385">32</a> Our study, on the other hand, included a larger cohort of patients and the inclusion criteria allowed (LVNC) diagnosis based on one or more criteria, by echocardiography or cardiac MRI. Our cohort of patients appears to be representative of LVNC and its wide clinical spectrum. Although a significant proportion of cases were incidental findings, there was good agreement between echocardiography and cardiac MRI regarding the diagnosis of LVNC, nearly half of the patients presented LV systolic dysfunction, and in the follow-up most patients developed complications. Even so, no Fabry disease patient was diagnosed besides the single case already reported in the literature. Nevertheless, our study is also limited by its small sample size, which may have prevented the detection of Fabry disease if LVNC was a rare manifestation of this disease. However, the common finding of LVNC in other patient populations, such as heart failure, also argues against the hypothesis of LVNC being a rare clinical manifestation of Fabry disease. The fact that our patient remained stable and did not develop any complications during an eight-year follow-up without specific therapy for Fabry disease also argues against the hypothesis that LVNC is a clinical manifestation or a risk marker for the development of other cardiac manifestations of Fabry disease.Based on recent evidence exposing the low specificity of the current diagnostic criteria and the increasing rate of overdiagnosis of LVNC,<a class="elsevierStyleCrossRefs" href="#bib0275">10,33,34</a> it is plausible that more cases of LVNC will be diagnosed among Fabry patients, raising concerns about its therapeutic implications. This is particularly important in Fabry females, who, in part due to the inactivation pattern of the X chromosome, present a highly variable prognosis, some of them exhibiting a benign disease course and a lifelong prognosis without significant clinical manifestations in the absence of specific therapy.<a class="elsevierStyleCrossRef" href="#bib0450">45</a>ConclusionIn summary, our study found no evidence to support the hypothesis that LVNC is a clinical manifestation of Fabry disease. Taken together with the available evidence, our results do not support the use of the current diagnostic criteria of LVNC for the decision to start specific therapy for Fabry disease, either enzyme replacement or chaperone therapy, in young asymptomatic females presenting LVNC criteria in the absence of other cardiac abnormalities or known manifestations of Fabry disease that would constitute a clinical indication to start therapy according to the current guidelines.DisclosuresOlga Azevedo, MD, and Gabriel Miltenberger-Miltenyi, PhD, have received educational and/or research grants from Shire Human Genetic Therapies, Inc., Genzyme Corporation and Amicus Nuno Marques, MD, Hugo Antunes, MD, and Liliana Reis, MD, have received educational grants from Shire Human Genetic Therapies, Inc. and Genzyme Corporation. Nuno Craveiro, MD, Rui Azevedo Guerreiro, MD, and Rui Pontes dos Santos, MD, have received educational grants from Shire Human Genetic Therapies, Inc. The other authors have no disclosures.FundingThis research was supported by a research grant from Shire Human Genetic Therapies, Inc.Shire Human Genetic Therapies, Inc. had no influence on the design and conduct of the study, in the collection, analysis, and interpretation of the data, or in the preparation, review, or approval of the manuscript.Conflicts of interestThe authors have no conflicts of interest to declare." "textoCompletoSecciones" => array:1 [ "secciones" => array:13 [ 0 => array:3 [ "identificador" => "xres1309695" "titulo" => "Abstract" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction and Aim" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusion" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1209264" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1309694" "titulo" => "Resumo" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Introdução e objetivo" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusão" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1209265" "titulo" => "Palavras-chave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:3 [ "identificador" => "sec0010" "titulo" => "Methods" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0015" "titulo" => "Patient population" ] 1 => array:2 [ "identificador" => "sec0020" "titulo" => "Fabry disease screening" ] 2 => array:2 [ "identificador" => "sec0025" "titulo" => "Ethical issues" ] ] ] 6 => array:2 [ "identificador" => "sec0030" "titulo" => "Results" ] 7 => array:2 [ "identificador" => "sec0035" "titulo" => "Discussion" ] 8 => array:2 [ "identificador" => "sec0040" "titulo" => "Conclusion" ] 9 => array:2 [ "identificador" => "sec0045" "titulo" => "Disclosures" ] 10 => array:2 [ "identificador" => "sec0050" "titulo" => "Funding" ] 11 => array:2 [ "identificador" => "sec0055" "titulo" => "Conflicts of interest" ] 12 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2018-09-23" "fechaAceptado" => "2019-02-03" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1209264" "palabras" => array:5 [ 0 => "Fabry disease" 1 => "Noncompaction" 2 => "Hypertrabeculation" 3 => "Cardiomyopathy" 4 => "Screening" ] ] ] "pt" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palavras-chave" "identificador" => "xpalclavsec1209265" "palabras" => array:5 [ 0 => "Doença de Fabry" 1 => "Não compactação" 2 => "Hipertrabeculação" 3 => "Cardiomiopatia" 4 => "Rastreio" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "Introduction and AimIt is unclear whether left ventricular noncompaction (LVNC) is a distinct cardiomyopathy or a morphologic manifestation of different cardiomyopathies. We previously reported a case of LVNC in a Fabry disease (FD) patient, but it remains to be clarified whether LVNC is a cardiac manifestation of FD, a coincidental finding or an overdiagnosis, which has major therapeutic implications. This study aims to determine the prevalence of FD among patients with LVNC. MethodsWe performed a retrospective study including all patients diagnosed with LVNC in eight hospital centers. Diagnosis of LVNC was based on at least one echocardiographic or cardiac magnetic resonance criterion. FD screening was performed by combined enzyme and genetic testing. ResultsThe study included 78 patients diagnosed with LVNC based on the Jenni (84.6%), Stöllberger (46.2%), Chin (21.8%), Petersen (83.8%) and Jacquier (16.2%) criteria. Left ventricular systolic dysfunction was present in 48.7%. Heart failure was found in 60.3%, ventricular dysrhythmias in 21.6% and embolic events in 11.5%. FD screening found no additional cases among patients with LVNC, besides the previously described case. ConclusionNo additional FD cases were found among patients with LVNC, which argues against the hypothesis that LVNC is a cardiac manifestation of FD." "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction and Aim" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusion" ] ] ] "pt" => array:3 [ "titulo" => "Resumo" "resumen" => "Introdução e objetivoNão está esclarecido se a não compactação do ventrículo esquerdo (NCVE) é uma miocardiopatia distinta ou uma manifestação morfológica de várias miocardiopatias. Nós reportamos previamente um caso de NCVE num doente com doença de Fabry (DF), mas permanece por esclarecer se a NCVE é uma manifestação cardíaca de DF, um achado coincidente ou um sobrediagnóstico, o que tem importantes implicações terapêuticas. Este estudo pretende determinar a prevalência de DF em doentes com NCVE. MétodosEstudo retrospetivo incluindo todos os doentes diagnosticados com NCVE em oito centros hospitalares. O diagnóstico de NCVE foi baseado em pelo menos um dos critérios de ecocardiografia ou ressonância cardíaca. O rastreio de DF foi realizado por teste enzimático e genético. ResultadosO estudo incluiu 78 doentes diagnosticados com NCVE com base nos critérios de Jenni (84,6%), Stöllberger (46,2%), Chin (21,8%), Petersen (83,8%) e Jacquier (16,2%). A disfunção sistólica do VE estava presente em 48,7%. A insuficiência cardíaca foi encontrada em 60,3%, as disritmias ventriculares em 21,6% e os eventos embólicos em 11,5%. O rastreio de DF não encontrou casos adicionais nos doentes com NCVE, para além do caso previamente descrito. ConclusãoNão foram encontrados casos adicionais de DF nos doentes com NCVE, o que argumenta contra a hipótese de a NCVE ser uma manifestação cardíaca de DF." "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Introdução e objetivo" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusão" ] ] ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 698 "Ancho" => 755 "Tamanyo" => 31421 ] ] "descripcion" => array:1 [ "en" => "Left ventricular noncompaction in a Fabry disease patient: cardiac magnetic resonance imaging showing hypertrabeculation mostly affecting the left ventricular apical region and lateral wall in apical 4-chamber view." ] ] 1 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "LV: left ventricular; MRI: magnetic resonance imaging." "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Echocardiographic criteria</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Jenni et al.<a class="elsevierStyleCrossRef" href="#bib0290">13</a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">(1) Numerous and prominent trabeculations and deep intertrabecular recesses that are localized predominantly in the apical and mid-ventricular segments of the inferior and lateral walls of the left ventricle;(2) Two-layered myocardium, with a thin compacted layer that is adjacent to the epicardium and a much thicker noncompacted layer that is near the endocardium, resulting in a maximum end-systolic ratio of the thickness of noncompacted to compacted layer >2 in short-axis parasternal view;(3) Color Döppler evidence of the perfusion of the intertrabecular recesses directly from the LV cavity;(4) Absence of other cardiac abnormalities. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Chin et al.<a class="elsevierStyleCrossRef" href="#bib0295">14</a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">(1) The quotient between the distance from the epicardial surface to the trough of the intertrabecular recess and the distance from the epicardial surface to the peak of the trabeculation decreases from the level of the papillary muscles to the apex and is ≤0.5, at end-diastole, at the level of the LV apex. Measurements are performed in parasternal long-axis view at the level of the mitral valve and papillary muscles and in subxiphoid or apical 4-chamber views at the level of the LV apex. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Stöllberger et al.<a class="elsevierStyleCrossRef" href="#bib0300">15</a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">(1) More than three trabeculations protruding from the LV wall, apically to the papillary muscles, are visualized in one imaging plane;(2) The intertrabecular recesses are perfused directly from the LV cavity as shown by color Döppler. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cardiac MRI criteria</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Petersen et al.<a class="elsevierStyleCrossRef" href="#bib0305">16</a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">The maximal end-diastolic ratio of the thickness of the noncompacted to the compacted layer, measured in any of the three long-axis views, is >2.3. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Jacquier et al.<a class="elsevierStyleCrossRef" href="#bib0310">17</a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">The LV trabeculated mass is over 20% of the total LV mass (papillary muscles are excluded from the trabeculated mass). \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Stacey et al.<a class="elsevierStyleCrossRef" href="#bib0315">18</a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">The maximal end-systolic ratio of the thickness of the noncompacted to compacted layer in short-axis views is ≥2. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Captur et al.<a class="elsevierStyleCrossRef" href="#bib0320">19</a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Maximal apical fractal dimension >1.3, measured at end-diastole in short-axis views of the apical third of the left ventricle. \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2244596.png" ] ] ] ] "descripcion" => array:1 [ "en" => "Diagnostic criteria of left ventricular noncompaction." ] ] 2 => array:8 [ "identificador" => "tbl0010" "etiqueta" => "Table 2" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at2" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "AV: atrioventricular; C: compacted; ECG: electrocardiogram; IVS: interventricular septum; LA: left atrial; LBBB: left bundle branch block; LV: left ventricular; LVNC: left ventricular noncompaction; MRI: magnetic resonance imaging; NC: non-compacted; PW: posterior wall; RBBB: right bundle branch block; VT: ventricular tachycardia." "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Gender (male/female) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">49 (62.8%)/29 (37.2%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Race (Caucasian/African) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">74 (94.9%)/4 (5.1%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Age at diagnosis of LVNC, years (mean±SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">47±17 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Age at Fabry disease screening, years (mean±SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">53±17 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Family history of LVNC \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5 (6.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Family history of Fabry disease \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1 (1.3%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Clinical presentation</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Incidental finding \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">35 (44.9%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Heart failure \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">27 (34.6%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Dysrhythmia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">9 (11.5%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Thromboembolism \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 (5.1%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Other \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8 (10.3%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Clinical complications in follow-up</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Heart failure \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">47 (60.3%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Dysrhythmias \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">28 (35.9%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Thromboembolism \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">9 (11.5%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Diagnosis of LVNC</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Echocardiography \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">71/78 (91.0%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cardiac MRI \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">58/68 (85.3%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Echocardiographic criteria</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Jenni \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">66 (84.6%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Stöllberger \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">36 (46.2%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Chin \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">17 (21.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cardiac MRI criteria</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Petersen \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">57 (83.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Jacquier \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">11 (16.2%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Stacey \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 (0.0%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Captur \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 (0.0%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Echocardiography</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LV ejection fraction, % (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">47.2±15.2 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LV systolic dysfunction \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">38 (48.7%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LV diastolic dysfunction \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">42 (56.0%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Mean Sa, cm/s (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">7.7±2.8 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Mean Ea, cm/s (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">9.2±4.1 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Mean E/Ea ratio \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8.9±2.7 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">IVS/PW thickness, mm (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">9.8±2.9/8.7±1.9 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LV mass, g/m2 (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">121.5±82.3 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LA volume, ml/m2 (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">32.9±13.9 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Valve disease \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">15 (19.2%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cardiac MRI</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Ratio of thickness of NC:C layer \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3.4±3.9 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LV mass, g/m2 (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">99.5±56.0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LV end-systolic volume, ml/m2 (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">71.8±50.0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LV end-diastolic volume, ml/m2 (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">130.1±75.1 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LV ejection fraction, % (mean ± SD) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">46.2±16.2 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Late gadolinium enhancement \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 (15.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">ECG</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Sinus rhythm \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">70 (89.7%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Atrial fibrillation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6 (7.7%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Atrial flutter \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2 (2.6%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">AV block \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 (5.1%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LBBB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 (12.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RBBB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3 (3.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Bifascicular block \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2 (2.6%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">24-hour Holter</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Sinus rhythm \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">67 (90.5%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Atrial fibrillation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 (13.5%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Atrial flutter \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1 (1.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">AV block \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">12 (16.2%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LBBB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">11 (14.9%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RBBB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3 (4.1%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Bifascicular block \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2 (2.7%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Supraventricular tachycardia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">13 (17.6%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">VT \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">16 (21.6%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Non-sustained VT \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">15 (20.3%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Sustained VT \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1 (1.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"></td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Clinical manifestations of Fabry disease</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Renal failure \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">7 (9.0%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Microalbuminuria \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1 (1.3%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Proteinuria \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 (0.0%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Stroke \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8 (10.3%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Acroparesthesias \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1 (1.3%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Deafness \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2 (2.6%) \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2244595.png" ] ] ] ] "descripcion" => array:1 [ "en" => "Baseline characteristics of patients with left ventricular noncompaction (n=78)." ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:45 [ 0 => array:3 [ "identificador" => "bib0230" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Fabry disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "D.P. 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Original Article

Screening for Fabry disease in patients with left ventricular noncompaction

Rastreio de doença de Fabry em doentes com não compactação do ventrículo esquerdo

Olga Azevedoa,b,c,

Corresponding author

olgazevedo@yahoo.com.br

Corresponding author.

, Nuno Marquesd,e,f, Nuno Craveirog, Ana Rita Pereirah, Hugo Antunesi, Liliana Reisj, Rui Azevedo Guerreirok, Rui Pontes dos Santosl, Gabriel Miltenberger-Miltenyib,c,m, Nuno Sousab,c, Damião Cunhab,c

a Cardiology Department, Reference Center on Lysosomal Storage Disorders, Hospital Senhora da Oliveira, Guimarães, Portugal

b Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal

c ICVS/3Bs PT Government Associate Laboratory, Braga/Guimarães, Portugal

d Cardiology Department, Centro Hospitalar Universitário do Algarve, Faro, Portugal

e Algarve Biomedical Center, Faro, Portugal

f Biomedical Science and Medicine Department, Algarve University, Faro, Portugal

g Cardiology Department, Hospital de Santarém, Santarém, Portugal

h Cardiology Department, Hospital Garcia de Orta, Almada, Portugal

i Cardiology Department, Centro Hospitalar de Tondela e Viseu, Viseu, Portugal

j Cardiology Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal

k Cardiology Department, Hospital do Espírito Santo, Évora, Portugal

l Cardiology Department, Centro Hospitalar do Tâmega e Sousa, Penafiel, Portugal

m Genetics Department, Reference Center on Lysosomal Storage Disorders, Hospital Senhora da Oliveira, Guimarães, Portugal

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          "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Left ventricular noncompaction in a Fabry disease patient&#58; cardiac magnetic resonance imaging showing hypertrabeculation mostly affecting the left ventricular apical region and lateral wall in apical 4-chamber view&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Fabry disease &#40;OMIM 301500&#41; is an X-linked lysosomal storage disorder caused by mutations in the <span class="elsevierStyleItalic">GLA</span> gene&#44; which is located on Xq22&#46;1 and codes for the enzyme alpha-galactosidase A &#40;&#945;-Gal A&#41;&#46; Deficiency in the enzyme activity of &#945;-Gal A leads to the lysosomal accumulation of globotriaosylceramide &#40;Gb3&#41; and related glycosphingolipids&#44; causing multiorgan damage&#46;<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Fabry disease is a rare condition&#44; estimated to occur in 1 in 3100 hemizygous males&#46;<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">2</span></a> The diagnosis in males is based on biochemical determination of &#945;-Gal A activity&#46; In heterozygous females&#44; &#945;-Gal A activity may be normal&#44; so diagnosis requires molecular analysis of the <span class="elsevierStyleItalic">GLA</span> gene&#46;<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">1</span></a> Clinical manifestations may arise early&#44; in childhood or adolescence&#44; and include acroparesthesias&#44; cornea verticillata&#44; angiokeratomas and microalbuminuria or proteinuria&#46; In adulthood&#44; patients may develop sensorineural deafness&#44; renal failure&#44; cerebrovascular events and cardiac manifestations&#46;<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">1</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Fabry disease affects the heart&#44; causing left ventricular hypertrophy &#40;LVH&#41; and fibrosis&#44; heart failure&#44; angina&#44; valve thickening or regurgitation&#44; dysrhythmias&#44; cardiac conduction abnormalities and sudden death&#46;<a class="elsevierStyleCrossRefs" href="#bib0230"><span class="elsevierStyleSup">1&#44;3</span></a> The main cardiac manifestation is hypertrophic cardiomyopathy&#44; which occurs in 43&#37; of male patients &#40;mean age of onset 39&#177;10 years&#41; and 26&#37; of female patients &#40;mean age of onset 50&#177;11 years&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0245"><span class="elsevierStyleSup">4</span></a> LVH in Fabry disease is usually non-obstructive and symmetrical<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">3</span></a> with prominent papillary muscles<a class="elsevierStyleCrossRef" href="#bib0250"><span class="elsevierStyleSup">5</span></a> and&#44; in nearly half of cases&#44; myocardial late gadolinium enhancement is typically observed in the mid-wall of the inferolateral basal segments of the left ventricle&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">6</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Left ventricular noncompaction &#40;LVNC&#41; is morphologically characterized by prominent left ventricular &#40;LV&#41; trabeculations and deep intertrabecular recesses communicating with the LV cavity&#44; located in the apical and lateral regions of the left ventricle&#44; distal to the papillary muscles&#46; The myocardium has a two-layer appearance&#44; composed of a thin compacted epicardial layer and a thick spongy noncompacted endocardial layer&#46;<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">7</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">LVNC has been found in 0&#46;045&#37; of adult individuals referred for echocardiography<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">8</span></a> and 0&#46;029&#37; of children admitted for heart failure due to cardiomyopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">9</span></a> It predominantly affects males<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">8</span></a> and individuals of African origin&#46;<a class="elsevierStyleCrossRef" href="#bib0275"><span class="elsevierStyleSup">10</span></a> First described on autopsy in 1969 by Feldt et al&#46;<a class="elsevierStyleCrossRef" href="#bib0280"><span class="elsevierStyleSup">11</span></a> and on echocardiography by Engberding et al&#46; in 1984&#44;<a class="elsevierStyleCrossRef" href="#bib0285"><span class="elsevierStyleSup">12</span></a> it is most commonly diagnosed by transthoracic echocardiography or cardiac magnetic resonance imaging &#40;MRI&#41;&#46; Several diagnostic criteria have been proposed using echocardiography or cardiac MRI<a class="elsevierStyleCrossRefs" href="#bib0290"><span class="elsevierStyleSup">13&#8211;19</span></a> &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Patients with LVNC may be asymptomatic or develop complications such as heart failure&#44; thromboembolism or ventricular dysrhythmias and sudden death&#46;<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">20</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The etiology and pathogenesis of LVNC are unknown&#46; It has been hypothesized to result from a disturbance of the compaction process of the LV myocardium during early development&#44; but growing evidence suggests that&#44; although the papillary muscles are formed by compaction of pre-existing trabeculations&#44; these trabeculations contribute little&#44; if anything&#44; to the compacted layer of the ventricular walls&#46;<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">21</span></a> Moreover&#44; not all cases are congenital and LVNC may be acquired at any stage of life after birth&#46;<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">7</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">LVNC is characterized by marked genetic heterogeneity&#46; Mutations have been described in genes that code for proteins in the mitochondria &#40;tafazzins&#41;&#44; Z line &#40;ZASP&#47;Cypher&#41;&#44; cytoskeleton &#40;alpha-dystrobrevin&#41;&#44; nuclear lamina &#40;lamin A&#47;C&#41; and sarcomere &#40;beta-myosin heavy chain&#44; alpha-cardiac actin and cardiac troponin T&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">7</span></a> However&#44; the causal relationship between these mutations and LVNC remains to be established&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">LVNC has been reported in patients with congenital cardiac abnormalities&#44; such as LV outflow tract abnormalities&#44; Ebstein anomaly&#44; tetralogy of Fallot and double outlet right ventricle&#59; chromosomal defects&#44; such as trisomy 13&#44; Cornelia de Lange&#44; DiGeorge&#44; Turner and 5q minus syndromes&#59; and neuromuscular disorders&#44; such as mitochondrial myopathies&#44; Barth syndrome&#44; zaspopathy&#44; myotonic dystrophy type 1&#44; dystrobrevinopathy&#44; and Emery-Dreifuss muscular dystrophy&#46;<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">7</span></a> LVNC has also been reported in patients with storage disorders such as glycogen storage disease types Ib and IV&#44;<a class="elsevierStyleCrossRefs" href="#bib0335"><span class="elsevierStyleSup">22&#44;23</span></a> Danon disease<a class="elsevierStyleCrossRef" href="#bib0345"><span class="elsevierStyleSup">24</span></a> and amyloidosis&#46;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">25</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">In 2011&#44; we reported the first case of LVNC in a Fabry disease patient&#46;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">26</span></a> This was a young asymptomatic female who fulfilled the Jenni echocardiographic criteria and the Petersen cardiac MRI criteria for LVNC&#46; She had been diagnosed with Fabry disease following family screening&#44; and the pathogenic mutation of the <span class="elsevierStyleItalic">GLA</span> gene detected was a missense mutation &#40;p&#46;F113L&#41; that is known to be associated with a predominantly cardiac later-onset phenotype&#46; However&#44; thorough diagnostic study revealed none of the known clinical manifestations of Fabry disease&#44; including LVH&#44; late gadolinium enhancement or other cardiac or extracardiac manifestations&#44; nor did she present LV dilation or dysfunction or any clinical complication of LVNC&#44; such as heart failure&#44; dysrhythmias or thromboembolism&#46; Moreover&#44; all of her relatives with Fabry disease underwent echocardiography and cardiac MRI and none presented LVNC&#46; In this report&#44; we pointed out the limitations of the current diagnostic criteria of LVNC and raised the question whether this was an overdiagnosis of LVNC&#44; a coincidental finding of LVNC and Fabry disease&#44; or if indeed the two conditions were clinically related&#46;<a class="elsevierStyleCrossRefs" href="#bib0355"><span class="elsevierStyleSup">26&#44;27</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">More recently&#44; two additional cases of LVNC in Fabry disease patients have been reported&#44;<a class="elsevierStyleCrossRefs" href="#bib0365"><span class="elsevierStyleSup">28&#8211;31</span></a> also in young females&#46; In the second case&#44; the diagnosis of LVNC was based only on cardiac MRI &#40;Petersen criteria&#41;&#44; without clear echocardiographic criteria of LVNC due to insufficient quality of the acoustic window&#46; She had classical Fabry disease due to a nonsense <span class="elsevierStyleItalic">GLA</span> gene mutation &#40;p&#46;R220X&#41;&#46; As in our case&#44; she also did not present LVH&#44; dilation&#44; dysfunction&#44; late gadolinium enhancement or other cardiac abnormalities&#44; and there was no history of heart failure&#44; dysrhythmias or thromboembolism&#46; There was also no family history of LVNC&#46; She had no clinical manifestations of Fabry disease besides cornea verticillata and angiokeratomas&#46; An endomyocardial biopsy showed deposition of glycosphingolipids in cardiomyocytes and based on this finding the patient was referred for enzyme replacement therapy&#46;<a class="elsevierStyleCrossRefs" href="#bib0365"><span class="elsevierStyleSup">28&#44;29</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">In the third case&#44; the diagnosis of LVNC was based on cardiac MRI &#40;Petersen criteria&#41; and echocardiography &#40;although it was not reported which specific criteria were fulfilled&#41;&#46; She was diagnosed with Fabry disease based on low levels of &#945;-Gal A&#44; but the pathogenic <span class="elsevierStyleItalic">GLA</span> gene mutation was never found&#46; The only clinical manifestations of Fabry disease were angiokeratomas and acroparesthesias&#46; As in the two previous cases&#44; there was no LVH&#44; dilation or dysfunction&#46; Cardiac MRI showed subendocardial late gadolinium enhancement&#44; but its location and extension was not reported&#46; Interestingly&#44; she had Wolff-Parkinson-White syndrome&#44; interpreted by the authors as an unrelated finding&#44; and underwent ablation of a para-Hisian accessory pathway&#44; which was followed by disappearance of the short PR interval and delta waves&#46; However&#44; as in the two previous cases&#44; she had no history of heart failure or thromboembolism&#46; Moreover&#44; there were no available data consistent with a family history of LVNC or Fabry disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0375"><span class="elsevierStyleSup">30&#44;31</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Taken together&#44; it remains essential to clarify whether LVNC could be a cardiac manifestation of Fabry disease&#44; given the implications for therapeutic decisions on whether to start enzyme replacement or chaperone therapy&#44; particularly in young asymptomatic females with no other known clinical manifestations of Fabry disease&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">The only published study to date on Fabry disease screening in patients with LVNC included 26 male patients who fulfilled the St&#246;llberger echocardiographic criteria for LVNC&#46; Fabry disease screening was based on the enzymatic assay of &#945;-Gal A and found no patients with the disease&#46;<a class="elsevierStyleCrossRef" href="#bib0385"><span class="elsevierStyleSup">32</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Herein&#44; we report the results of Fabry disease screening in a larger cohort of patients with LVNC&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Methods</span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Patient population</span><p id="par0080" class="elsevierStylePara elsevierViewall">Between October 2015 and October 2017&#44; 78 consecutive patients with LVNC were recruited from the cardiology consultation of eight Portuguese hospital centers&#46; The inclusion criteria were adult patients &#40;aged &#8805;18 years&#41; diagnosed with LVNC based on the presence of any of the current diagnostic criteria of LVNC&#44; by either echocardiography or cardiac MRI&#44; as described in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#46; All patients fulfilling the inclusion criteria underwent Fabry disease screening&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Fabry disease screening</span><p id="par0085" class="elsevierStylePara elsevierViewall">Fabry disease screening was performed at a specialized laboratory &#40;Centogene AG&#44; Rostock&#44; Germany&#41;&#46; &#945;-Gal A enzyme activity was measured in dried blood spot samples by fluorometry&#46; Molecular analysis of the <span class="elsevierStyleItalic">GLA</span> gene was performed in males with reduced &#945;-Gal A enzyme activity&#44; i&#46;e&#46;&#44; below the normal reference range &#40;&#60;15&#46;3 &#956;mol&#47;l&#47;h&#41;&#44; and in all females&#44; independently of &#945;-Gal A activity&#46; The molecular analysis was performed by classical bidirectional Sanger sequencing of the <span class="elsevierStyleItalic">GLA</span> gene and included all exons&#44; exon&#47;intron boundaries and the promoter region&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Ethical issues</span><p id="par0090" class="elsevierStylePara elsevierViewall">This research project was approved by the ethics committees of all the hospital centers included in this study and all patients provided written informed consent&#46;</p></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Results</span><p id="par0095" class="elsevierStylePara elsevierViewall">Patients with LVNC were predominantly males &#40;62&#46;8&#37;&#41;&#46; Only four patients &#40;5&#46;1&#37;&#41; were identified as being of African origin&#46; Mean age at diagnosis of LVNC was 64&#46;4&#177;14&#46;1 years&#46; A family history of LVNC was recorded in five patients &#40;6&#46;4&#37;&#41;&#46; The patients did not present concomitant neuromuscular disorders or any other medical conditions reported to be associated with LVNC&#46;</p><p id="par0100" class="elsevierStylePara elsevierViewall">The diagnosis of LVNC was established by echocardiography in 91&#46;0&#37; of the cases&#44; based on the Jenni criteria in 84&#46;6&#37;&#44; the St&#246;llberger criteria in 46&#46;2&#37;&#44; the Chin criteria in 21&#46;8&#37; and the three echocardiographic criteria in 20&#46;5&#37; of the cases&#46; Nearly half of the patients presented LV systolic dysfunction &#40;48&#46;7&#37;&#41; and mean LV ejection fraction was 47&#46;2&#177;15&#46;2&#37;&#46; Cardiac MRI was performed in 68 patients &#40;87&#46;2&#37;&#41; and the diagnosis of LVNC was established in 58 patients &#40;85&#46;3&#37;&#41;&#44; mainly based on the Petersen criteria &#40;83&#46;8&#37;&#41;&#46; The trabeculated LV mass was calculated in only 11 patients&#44; all of them fulfilling the Jacquier criteria of LVNC&#46; The presence of the Stacey or Captur criteria was not assessed in any cardiac MRI&#46; The diagnosis of LVNC was established by both echocardiography and cardiac MRI in 53 of the 68 patients who underwent cardiac MRI &#40;77&#46;9&#37;&#41;&#46;</p><p id="par0105" class="elsevierStylePara elsevierViewall">LVNC was an incidental finding in 44&#46;9&#37; of the patients&#46; Heart failure was the most common clinical manifestation at the time of diagnosis &#40;34&#46;6&#37;&#41;&#46; In the follow-up &#40;53&#177;33 months&#41;&#44; heart failure remained the most frequent clinical complication &#40;60&#46;3&#37;&#41;&#44; with New York Heart Association functional class ranging from I to IV &#40;I&#58; 8&#46;5&#37;&#59; II&#58; 59&#46;6&#37;&#59; III&#58; 23&#46;4&#37;&#59; IV&#58; 8&#46;5&#37;&#41;&#46; Dysrhythmias were also common &#40;35&#46;9&#37;&#41;&#46; Twenty-four-hour Holter monitoring was performed in 74 patients &#40;94&#46;9&#37;&#41; and revealed atrial fibrillation in 10 patients &#40;13&#46;5&#37;&#41; and ventricular tachycardia &#40;VT&#41; in 16 patients &#40;21&#46;6&#37;&#41;&#44; although only one case exhibited sustained VT&#46; Thromboembolism occurred in nine patients &#40;11&#46;5&#37;&#41;&#44; as stroke in eight and as myocardial infarction in one &#40;<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>&#41;&#46;</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0110" class="elsevierStylePara elsevierViewall">Twenty-eight patients &#40;35&#46;9&#37;&#41; had undergone genetic study that included a wide range of genes &#40;<span class="elsevierStyleItalic">MYH7</span>&#58; 32&#46;1&#37;&#59; <span class="elsevierStyleItalic">MYBPC3</span>&#58; 30&#46;8&#37;&#59; <span class="elsevierStyleItalic">TNNT2</span>&#58; 28&#46;2&#37;&#59; <span class="elsevierStyleItalic">TNNI3</span>&#58; 19&#46;2&#37;&#59; <span class="elsevierStyleItalic">ACTC1</span>&#58; 28&#46;2&#37;&#59; <span class="elsevierStyleItalic">TPM1</span>&#58; 28&#46;2&#37;&#59; <span class="elsevierStyleItalic">MYL2</span>&#58; 11&#46;5&#37;&#59; <span class="elsevierStyleItalic">MYL3</span>&#58; 11&#46;5&#37;&#59; <span class="elsevierStyleItalic">DTNA</span>&#58; 7&#46;7&#37;&#59; <span class="elsevierStyleItalic">DMD</span>&#58; 10&#46;3&#37;&#59; <span class="elsevierStyleItalic">LDB3</span>&#58; 28&#46;2&#37;&#59; <span class="elsevierStyleItalic">TAZ</span>&#58; 26&#46;9&#37;&#59; <span class="elsevierStyleItalic">LMNA</span>&#58; 24&#46;4&#37;&#59; <span class="elsevierStyleItalic">CSRP3</span>&#58; 24&#46;4&#37;&#59; <span class="elsevierStyleItalic">TCAP</span>&#58; 20&#46;5&#37;&#59; <span class="elsevierStyleItalic">SGCD</span>&#58; 21&#46;8&#37;&#59; <span class="elsevierStyleItalic">PLN</span>&#58; 23&#46;1&#37;&#59; <span class="elsevierStyleItalic">TTN</span>&#58; 2&#46;6&#37;&#59; <span class="elsevierStyleItalic">SCN5A</span>&#58; 1&#46;3&#37;&#41;&#46; A pathogenic mutation was found in the <span class="elsevierStyleItalic">MYH7</span> gene in one patient &#40;c&#46;1003G&#62;C&#41; &#40;unpublished data&#41;&#46; Eight genetic variants of uncertain significance were found in six cases&#58; four in <span class="elsevierStyleItalic">MYBPC3</span> &#40;c&#46;1505G&#62;A&#59; c&#46;2762A&#62;T&#59; c&#46;1624G&#62;C&#59; c&#46;3676C&#62;T&#41;&#44; the last two in the same patient&#59; two in <span class="elsevierStyleItalic">TTN</span> &#40;c&#46;16425T&#62;G&#59; c&#46;60259C&#62;G&#41;&#59; one in <span class="elsevierStyleItalic">SCN5A</span> &#40;c&#46;393-5C&#62;A&#41;&#59; and one in <span class="elsevierStyleItalic">SGCD</span> &#40;c&#46;772-774delAAG&#41; in the same patient carrying two genetic variants of the <span class="elsevierStyleItalic">MYBPC3</span> gene&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">The screening of 78 patients with LVNC revealed no additional cases of Fabry disease besides the one already identified&#46;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">26</span></a> This Fabry patient was never under enzyme replacement or chaperone therapy&#46; During the eight-year follow-up&#44; this patient has remained asymptomatic and without known clinical manifestation of Fabry disease or complication of LVNC&#46; Moreover&#44; there were no significant changes regarding the location or extension of LVNC or LV chamber size&#44; mass or function during follow-up &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Discussion</span><p id="par0120" class="elsevierStylePara elsevierViewall">This multicenter study of Fabry disease screening in 78 consecutive patients with LVNC revealed no additional Fabry patients besides the single case that had been previously reported by our center&#44; which argues against the hypothesis that LVNC is a clinical manifestation of Fabry disease&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">The most likely explanation for the finding of LVNC in this Fabry patient is an overdiagnosis of LVNC&#46; Studies have increasingly exposed the lack of specificity of the current diagnostic criteria for LVNC&#44; potentially leading to overdiagnosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0275"><span class="elsevierStyleSup">10&#44;33&#44;34</span></a> Moreover&#44; there is growing evidence that LVNC has no independent prognostic impact compared to conventional risk factors&#44; such as LV dilation&#44; dysfunction or fibrosis&#44; and that patients with LVNC without LV dilation&#44; dysfunction or fibrosis have an excellent prognosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">34&#8211;36</span></a></p><p id="par0130" class="elsevierStylePara elsevierViewall">In a study of 199 patients referred to a heart failure clinic and 60 healthy controls&#44; Kohli et al&#46; found that 23&#46;6&#37; of the patients and 8&#46;3&#37; of the controls fulfilled one or more echocardiographic criteria for LVNC&#46; Among the heart failure patients with LVNC criteria&#44; 29&#46;8&#37; fulfilled all three echocardiographic criteria and 36&#46;2&#37; fulfilled only a single criterion&#44; with the frequency of each criterion ranging from 12&#46;1&#37; to 18&#46;6&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0275"><span class="elsevierStyleSup">10</span></a> In a large population-based cohort of 1000 subjects from the Multi-Ethnic Study of Atherosclerosis &#40;MESA&#41; registry who underwent cardiac MRI&#44; the Petersen criteria for LVNC were fulfilled in 43&#37; of the subjects without cardiac disease&#46;<a class="elsevierStyleCrossRef" href="#bib0390"><span class="elsevierStyleSup">33</span></a> Another study based on the MESA registry including 2742 subjects undergoing cardiac MRI showed that a greater extent of LV trabeculations in asymptomatic individuals appears to be benign and is not associated with deterioration in LV volumes or function over an almost 10-year period&#46;<a class="elsevierStyleCrossRef" href="#bib0405"><span class="elsevierStyleSup">36</span></a> In a study including 700 patients referred for cardiac MRI&#44; Ivanov et al&#46; found an LVNC prevalence of 39&#37;&#44; 23&#37;&#44; 25&#37; and 3&#37;&#44; according to the Petersen&#44; Stacey&#44; Jacquier and Captur criteria&#44; respectively&#46; In this study&#44; during a median follow-up of seven years&#44; there were no statistically significant differences in the assessed outcomes &#40;death&#44; ischemic stroke&#44; ventricular tachycardia&#47;fibrillation&#44; and heart failure hospitalization&#41; between patients with and without LVNC&#44; irrespective of the criteria applied&#46;<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">34</span></a> Finally&#44; in a study including 113 patients with LVNC according to the Jenni and Petersen criteria&#44; Andreini et al&#46; found that the degree of hypertrabeculation had no prognostic impact over and above LV dilation&#44; dysfunction or late gadolinium enhancement&#46; Indeed&#44; patients without LV dilation&#44; dysfunction or fibrosis had an excellent prognosis at four-year follow-up&#46;<a class="elsevierStyleCrossRef" href="#bib0400"><span class="elsevierStyleSup">35</span></a></p><p id="par0135" class="elsevierStylePara elsevierViewall">In light of this evidence&#44; the possibility of an overdiagnosis of LVNC&#44; with no associated prognostic impact&#44; must be considered in these three cases of Fabry disease published in the literature&#46; In fact&#44; all three case reports of LVNC occurred in young females with no family history of LVNC in Fabry or non-Fabry relatives&#44; no LV hypertrophy&#44; dilation or dysfunction&#44; no late gadolinium enhancement suggestive of Fabry disease involvement&#44; no or few clinical manifestations of Fabry disease&#44; and no clinical complications of LVNC&#46;<a class="elsevierStyleCrossRefs" href="#bib0355"><span class="elsevierStyleSup">26&#8211;31</span></a></p><p id="par0140" class="elsevierStylePara elsevierViewall">Appropriate follow-up would also be required to correctly interpret the significance of LVNC in these Fabry patients&#46; In our case&#44; the patient was never under enzyme replacement or chaperone therapy&#44; because at baseline she did not present any known manifestation of Fabry disease that would constitute a clinical indication to start specific therapy according to the guidelines&#46; During the eight-year follow-up of this patient&#44; no significant change has been documented in the location or extension of LVNC or in LV chamber size&#44; mass or function&#46; Moreover&#44; this patient has remained asymptomatic and with no known clinical manifestation of Fabry disease or complication of LVNC&#44; which supports the hypothesis of an overdiagnosis of LVNC in this Fabry patient&#46;</p><p id="par0145" class="elsevierStylePara elsevierViewall">Another possibility is that LVNC and Fabry disease in these patients are coincidental and unrelated conditions&#46; Although the simultaneous finding of two unrelated rare conditions may seem unlikely&#44; it is now known that LVNC has a much higher prevalence<a class="elsevierStyleCrossRefs" href="#bib0275"><span class="elsevierStyleSup">10&#44;33&#44;34</span></a> than the previous estimate of 0&#46;045&#37;<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">8</span></a> and the currently estimated Fabry disease prevalence of 1 in 3100<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">2</span></a> is much higher than the previous estimate of 1 in 117 000&#46;<a class="elsevierStyleCrossRef" href="#bib0410"><span class="elsevierStyleSup">37</span></a></p><p id="par0150" class="elsevierStylePara elsevierViewall">The finding of LVNC in a wide variety of congenital cardiac abnormalities&#44; chromosomal defects&#44; neuromuscular disorders<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">7</span></a> and storage disorders&#44;<a class="elsevierStyleCrossRefs" href="#bib0335"><span class="elsevierStyleSup">22&#8211;24</span></a> as well as in elite athletes<a class="elsevierStyleCrossRef" href="#bib0415"><span class="elsevierStyleSup">38</span></a> and pregnant women&#44;<a class="elsevierStyleCrossRef" href="#bib0420"><span class="elsevierStyleSup">39</span></a> has raised the question of whether LVNC is indeed a distinct cardiomyopathy or simply a common epiphenomenon in cardiac overload conditions&#46; In our case&#44; this young woman was not an athlete&#44; but she had been pregnant once&#44; although we have no record of echocardiograms before&#44; during or just after pregnancy&#46; A history of pregnancy or vigorous exercise was not reported in the other two cases&#46;</p><p id="par0155" class="elsevierStylePara elsevierViewall">Finally&#44; there is the possibility that LVNC could be a cardiac manifestation of Fabry disease&#46; However&#44; there is no evidence that Fabry disease causes LVNC by either compensatory or genetic mechanisms&#46; Indeed&#44; although they had Fabry disease&#44; none of these patients had concomitant LVH&#44; dilation or dysfunction that could have led to cardiac overload and hence to LVNC&#46; Furthermore&#44; there is no evidence that the <span class="elsevierStyleItalic">GLA</span> gene mutation&#44; the mutated &#945;-Gal A&#44; or the intramyocardial deposition of glycosphingolipids due to Fabry disease are the cause of or are in any way related to the development of LVNC&#44; and any assumption on this point remains speculative&#46; Lysosomal accumulation of Gb3 begins in utero and has been demonstrated as early as the 17th week of gestation in various fetal cell types&#44; such as cardiomyocytes&#44;<a class="elsevierStyleCrossRef" href="#bib0425"><span class="elsevierStyleSup">40</span></a> kidney podocytes&#44;<a class="elsevierStyleCrossRefs" href="#bib0425"><span class="elsevierStyleSup">40&#8211;42</span></a> hepatocytes&#44;<a class="elsevierStyleCrossRefs" href="#bib0430"><span class="elsevierStyleSup">41&#44;43</span></a> corneal epithelial cells<a class="elsevierStyleCrossRef" href="#bib0445"><span class="elsevierStyleSup">44</span></a> and myenteric plexus cells&#46;<a class="elsevierStyleCrossRef" href="#bib0435"><span class="elsevierStyleSup">42</span></a> Although Gb3 storage begins in utero&#44; clinical manifestations only develop several years later&#46; Therefore&#44; Gb3 deposits are expected findings in biopsies&#44; including endomyocardial biopsies&#44; of all Fabry patients independently of the presence of clinical manifestations&#44; and such deposition should not be interpreted as evidence that LVNC is due to Fabry disease&#46;</p><p id="par0160" class="elsevierStylePara elsevierViewall">The only study screening for Fabry disease in patients with LVNC did not reveal any positive case&#46; However&#44; this was a small cohort of 26 male patients and the only criteria for LVNC diagnosis were the St&#246;llberger echocardiographic criteria&#46;<a class="elsevierStyleCrossRef" href="#bib0385"><span class="elsevierStyleSup">32</span></a> Our study&#44; on the other hand&#44; included a larger cohort of patients and the inclusion criteria allowed &#40;LVNC&#41; diagnosis based on one or more criteria&#44; by echocardiography or cardiac MRI&#46; Our cohort of patients appears to be representative of LVNC and its wide clinical spectrum&#46; Although a significant proportion of cases were incidental findings&#44; there was good agreement between echocardiography and cardiac MRI regarding the diagnosis of LVNC&#44; nearly half of the patients presented LV systolic dysfunction&#44; and in the follow-up most patients developed complications&#46; Even so&#44; no Fabry disease patient was diagnosed besides the single case already reported in the literature&#46; Nevertheless&#44; our study is also limited by its small sample size&#44; which may have prevented the detection of Fabry disease if LVNC was a rare manifestation of this disease&#46; However&#44; the common finding of LVNC in other patient populations&#44; such as heart failure&#44; also argues against the hypothesis of LVNC being a rare clinical manifestation of Fabry disease&#46; The fact that our patient remained stable and did not develop any complications during an eight-year follow-up without specific therapy for Fabry disease also argues against the hypothesis that LVNC is a clinical manifestation or a risk marker for the development of other cardiac manifestations of Fabry disease&#46;</p><p id="par0165" class="elsevierStylePara elsevierViewall">Based on recent evidence exposing the low specificity of the current diagnostic criteria and the increasing rate of overdiagnosis of LVNC&#44;<a class="elsevierStyleCrossRefs" href="#bib0275"><span class="elsevierStyleSup">10&#44;33&#44;34</span></a> it is plausible that more cases of LVNC will be diagnosed among Fabry patients&#44; raising concerns about its therapeutic implications&#46; This is particularly important in Fabry females&#44; who&#44; in part due to the inactivation pattern of the X chromosome&#44; present a highly variable prognosis&#44; some of them exhibiting a benign disease course and a lifelong prognosis without significant clinical manifestations in the absence of specific therapy&#46;<a class="elsevierStyleCrossRef" href="#bib0450"><span class="elsevierStyleSup">45</span></a></p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Conclusion</span><p id="par0170" class="elsevierStylePara elsevierViewall">In summary&#44; our study found no evidence to support the hypothesis that LVNC is a clinical manifestation of Fabry disease&#46; Taken together with the available evidence&#44; our results do not support the use of the current diagnostic criteria of LVNC for the decision to start specific therapy for Fabry disease&#44; either enzyme replacement or chaperone therapy&#44; in young asymptomatic females presenting LVNC criteria in the absence of other cardiac abnormalities or known manifestations of Fabry disease that would constitute a clinical indication to start therapy according to the current guidelines&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Disclosures</span><p id="par0175" class="elsevierStylePara elsevierViewall">Olga Azevedo&#44; MD&#44; and Gabriel Miltenberger-Miltenyi&#44; PhD&#44; have received educational and&#47;or research grants from Shire Human Genetic Therapies&#44; Inc&#46;&#44; Genzyme Corporation and Amicus Nuno Marques&#44; MD&#44; Hugo Antunes&#44; MD&#44; and Liliana Reis&#44; MD&#44; have received educational grants from Shire Human Genetic Therapies&#44; Inc&#46; and Genzyme Corporation&#46; Nuno Craveiro&#44; MD&#44; Rui Azevedo Guerreiro&#44; MD&#44; and Rui Pontes dos Santos&#44; MD&#44; have received educational grants from Shire Human Genetic Therapies&#44; Inc&#46; The other authors have no disclosures&#46;</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Funding</span><p id="par0180" class="elsevierStylePara elsevierViewall">This research was supported by a research grant from <span class="elsevierStyleGrantSponsor" id="gs1">Shire Human Genetic Therapies&#44; Inc</span>&#46;</p><p id="par0185" class="elsevierStylePara elsevierViewall">Shire Human Genetic Therapies&#44; Inc&#46; had no influence on the design and conduct of the study&#44; in the collection&#44; analysis&#44; and interpretation of the data&#44; or in the preparation&#44; review&#44; or approval of the manuscript&#46;</p></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Conflicts of interest</span><p id="par0190" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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    "fechaRecibido" => "2018-09-23"
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            1 => "N&#227;o compacta&#231;&#227;o"
            2 => "Hipertrabecula&#231;&#227;o"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Introduction and Aim</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">It is unclear whether left ventricular noncompaction &#40;LVNC&#41; is a distinct cardiomyopathy or a morphologic manifestation of different cardiomyopathies&#46; We previously reported a case of LVNC in a Fabry disease &#40;FD&#41; patient&#44; but it remains to be clarified whether LVNC is a cardiac manifestation of FD&#44; a coincidental finding or an overdiagnosis&#44; which has major therapeutic implications&#46; This study aims to determine the prevalence of FD among patients with LVNC&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We performed a retrospective study including all patients diagnosed with LVNC in eight hospital centers&#46; Diagnosis of LVNC was based on at least one echocardiographic or cardiac magnetic resonance criterion&#46; FD screening was performed by combined enzyme and genetic testing&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The study included 78 patients diagnosed with LVNC based on the Jenni &#40;84&#46;6&#37;&#41;&#44; St&#246;llberger &#40;46&#46;2&#37;&#41;&#44; Chin &#40;21&#46;8&#37;&#41;&#44; Petersen &#40;83&#46;8&#37;&#41; and Jacquier &#40;16&#46;2&#37;&#41; criteria&#46; Left ventricular systolic dysfunction was present in 48&#46;7&#37;&#46; Heart failure was found in 60&#46;3&#37;&#44; ventricular dysrhythmias in 21&#46;6&#37; and embolic events in 11&#46;5&#37;&#46; FD screening found no additional cases among patients with LVNC&#44; besides the previously described case&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusion</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">No additional FD cases were found among patients with LVNC&#44; which argues against the hypothesis that LVNC is a cardiac manifestation of FD&#46;</p></span>"
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      "pt" => array:3 [
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        "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Introdu&#231;&#227;o e objetivo</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">N&#227;o est&#225; esclarecido se a n&#227;o compacta&#231;&#227;o do ventr&#237;culo esquerdo &#40;NCVE&#41; &#233; uma miocardiopatia distinta ou uma manifesta&#231;&#227;o morfol&#243;gica de v&#225;rias miocardiopatias&#46; N&#243;s reportamos previamente um caso de NCVE num doente com doen&#231;a de Fabry &#40;DF&#41;&#44; mas permanece por esclarecer se a NCVE &#233; uma manifesta&#231;&#227;o card&#237;aca de DF&#44; um achado coincidente ou um sobrediagn&#243;stico&#44; o que tem importantes implica&#231;&#245;es terap&#234;uticas&#46; Este estudo pretende determinar a preval&#234;ncia de DF em doentes com NCVE&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">M&#233;todos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Estudo retrospetivo incluindo todos os doentes diagnosticados com NCVE em oito centros hospitalares&#46; O diagn&#243;stico de NCVE foi baseado em pelo menos um dos crit&#233;rios de ecocardiografia ou resson&#226;ncia card&#237;aca&#46; O rastreio de DF foi realizado por teste enzim&#225;tico e gen&#233;tico&#46;</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">O estudo incluiu 78 doentes diagnosticados com NCVE com base nos crit&#233;rios de Jenni &#40;84&#44;6&#37;&#41;&#44; St&#246;llberger &#40;46&#44;2&#37;&#41;&#44; Chin &#40;21&#44;8&#37;&#41;&#44; Petersen &#40;83&#44;8&#37;&#41; e Jacquier &#40;16&#44;2&#37;&#41;&#46; A disfun&#231;&#227;o sist&#243;lica do VE estava presente em 48&#44;7&#37;&#46; A insufici&#234;ncia card&#237;aca foi encontrada em 60&#44;3&#37;&#44; as disritmias ventriculares em 21&#44;6&#37; e os eventos emb&#243;licos em 11&#44;5&#37;&#46; O rastreio de DF n&#227;o encontrou casos adicionais nos doentes com NCVE&#44; para al&#233;m do caso previamente descrito&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclus&#227;o</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">N&#227;o foram encontrados casos adicionais de DF nos doentes com NCVE&#44; o que argumenta contra a hip&#243;tese de a NCVE ser uma manifesta&#231;&#227;o card&#237;aca de DF&#46;</p></span>"
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          3 => array:2 [
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          "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Left ventricular noncompaction in a Fabry disease patient&#58; cardiac magnetic resonance imaging showing hypertrabeculation mostly affecting the left ventricular apical region and lateral wall in apical 4-chamber view&#46;</p>"
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          "leyenda" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">LV&#58; left ventricular&#59; MRI&#58; magnetic resonance imaging&#46;</p>"
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                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t  " colspan="2" align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Echocardiographic criteria</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Jenni et al&#46;<a class="elsevierStyleCrossRef" href="#bib0290"><span class="elsevierStyleSup">13</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">&#40;1&#41; Numerous and prominent trabeculations and deep intertrabecular recesses that are localized predominantly in the apical and mid-ventricular segments of the inferior and lateral walls of the left ventricle&#59;&#40;2&#41; Two-layered myocardium&#44; with a thin compacted layer that is adjacent to the epicardium and a much thicker noncompacted layer that is near the endocardium&#44; resulting in a maximum end-systolic ratio of the thickness of noncompacted to compacted layer &#62;2 in short-axis parasternal view&#59;&#40;3&#41; Color D&#246;ppler evidence of the perfusion of the intertrabecular recesses directly from the LV cavity&#59;&#40;4&#41; Absence of other cardiac abnormalities&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Chin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0295"><span class="elsevierStyleSup">14</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">&#40;1&#41; The quotient between the distance from the epicardial surface to the trough of the intertrabecular recess and the distance from the epicardial surface to the peak of the trabeculation decreases from the level of the papillary muscles to the apex and is &#8804;0&#46;5&#44; at end-diastole&#44; at the level of the LV apex&#46; Measurements are performed in parasternal long-axis view at the level of the mitral valve and papillary muscles and in subxiphoid or apical 4-chamber views at the level of the LV apex&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">St&#246;llberger et al&#46;<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">15</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">&#40;1&#41; More than three trabeculations protruding from the LV wall&#44; apically to the papillary muscles&#44; are visualized in one imaging plane&#59;&#40;2&#41; The intertrabecular recesses are perfused directly from the LV cavity as shown by color D&#246;ppler&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Cardiac MRI criteria</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Petersen et al&#46;<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">16</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">The maximal end-diastolic ratio of the thickness of the noncompacted to the compacted layer&#44; measured in any of the three long-axis views&#44; is &#62;2&#46;3&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Jacquier et al&#46;<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">17</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">The LV trabeculated mass is over 20&#37; of the total LV mass &#40;papillary muscles are excluded from the trabeculated mass&#41;&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Stacey et al&#46;<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">18</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">The maximal end-systolic ratio of the thickness of the noncompacted to compacted layer in short-axis views is &#8805;2&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Captur et al&#46;<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">19</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Maximal apical fractal dimension &#62;1&#46;3&#44; measured at end-diastole in short-axis views of the apical third of the left ventricle&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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          "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Diagnostic criteria of left ventricular noncompaction&#46;</p>"
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          "leyenda" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">AV&#58; atrioventricular&#59; C&#58; compacted&#59; ECG&#58; electrocardiogram&#59; IVS&#58; interventricular septum&#59; LA&#58; left atrial&#59; LBBB&#58; left bundle branch block&#59; LV&#58; left ventricular&#59; LVNC&#58; left ventricular noncompaction&#59; MRI&#58; magnetic resonance imaging&#59; NC&#58; non-compacted&#59; PW&#58; posterior wall&#59; RBBB&#58; right bundle branch block&#59; VT&#58; ventricular tachycardia&#46;</p>"
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                  <table border="0" frame="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Gender &#40;male&#47;female&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">49 &#40;62&#46;8&#37;&#41;&#47;29 &#40;37&#46;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Race &#40;Caucasian&#47;African&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">74 &#40;94&#46;9&#37;&#41;&#47;4 &#40;5&#46;1&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Age at diagnosis of LVNC&#44; years &#40;mean</span><span class="elsevierStyleItalic">&#177;</span><span class="elsevierStyleItalic">SD&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">47&#177;17&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Age at Fabry disease screening&#44; years &#40;mean</span><span class="elsevierStyleItalic">&#177;</span><span class="elsevierStyleItalic">SD&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">53&#177;17&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Family history of LVNC</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">5 &#40;6&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Family history of Fabry disease</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">1 &#40;1&#46;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Clinical presentation</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Incidental finding&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">35 &#40;44&#46;9&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Heart failure&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">27 &#40;34&#46;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Dysrhythmia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">9 &#40;11&#46;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Thromboembolism&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">4 &#40;5&#46;1&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Other&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">8 &#40;10&#46;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Clinical complications in follow-up</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Heart failure&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">47 &#40;60&#46;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Dysrhythmias&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">28 &#40;35&#46;9&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Thromboembolism&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">9 &#40;11&#46;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Diagnosis of LVNC</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Echocardiography&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">71&#47;78 &#40;91&#46;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Cardiac MRI&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">58&#47;68 &#40;85&#46;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Echocardiographic criteria</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Jenni&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">66 &#40;84&#46;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>St&#246;llberger&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">36 &#40;46&#46;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Chin&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">17 &#40;21&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Cardiac MRI criteria</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Petersen&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">57 &#40;83&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Jacquier&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">11 &#40;16&#46;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Stacey&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0 &#40;0&#46;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Captur&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0 &#40;0&#46;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Echocardiography</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LV ejection fraction&#44; &#37; &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">47&#46;2&#177;15&#46;2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LV systolic dysfunction&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">38 &#40;48&#46;7&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LV diastolic dysfunction&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">42 &#40;56&#46;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Mean Sa&#44; cm&#47;s &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7&#46;7&#177;2&#46;8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Mean Ea&#44; cm&#47;s &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">9&#46;2&#177;4&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Mean E&#47;Ea ratio&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">8&#46;9&#177;2&#46;7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>IVS&#47;PW thickness&#44; mm &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">9&#46;8&#177;2&#46;9&#47;8&#46;7&#177;1&#46;9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LV mass&#44; g&#47;m<span class="elsevierStyleSup">2</span> &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">121&#46;5&#177;82&#46;3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LA volume&#44; ml&#47;m<span class="elsevierStyleSup">2</span> &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">32&#46;9&#177;13&#46;9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Valve disease&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">15 &#40;19&#46;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Cardiac MRI</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Ratio of thickness of NC&#58;C layer&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3&#46;4&#177;3&#46;9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LV mass&#44; g&#47;m<span class="elsevierStyleSup">2</span> &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">99&#46;5&#177;56&#46;0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LV end-systolic volume&#44; ml&#47;m<span class="elsevierStyleSup">2</span> &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">71&#46;8&#177;50&#46;0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LV end-diastolic volume&#44; ml&#47;m<span class="elsevierStyleSup">2</span> &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">130&#46;1&#177;75&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LV ejection fraction&#44; &#37; &#40;mean &#177; SD&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">46&#46;2&#177;16&#46;2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Late gadolinium enhancement&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">10 &#40;15&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">ECG</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Sinus rhythm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">70 &#40;89&#46;7&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Atrial fibrillation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6 &#40;7&#46;7&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Atrial flutter&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 &#40;2&#46;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>AV block&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 &#40;5&#46;1&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LBBB&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">10 &#40;12&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>RBBB&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 &#40;3&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Bifascicular block&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 &#40;2&#46;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">24-hour Holter</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Sinus rhythm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">67 &#40;90&#46;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Atrial fibrillation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">10 &#40;13&#46;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Atrial flutter&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;1&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>AV block&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">12 &#40;16&#46;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>LBBB&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">11 &#40;14&#46;9&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>RBBB&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 &#40;4&#46;1&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Bifascicular block&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 &#40;2&#46;7&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Supraventricular tachycardia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">13 &#40;17&#46;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>VT&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">16 &#40;21&#46;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Non-sustained VT&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">15 &#40;20&#46;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Sustained VT&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;1&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="2" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Clinical manifestations of Fabry disease</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Renal failure&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7 &#40;9&#46;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Microalbuminuria&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;1&#46;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Proteinuria&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0 &#40;0&#46;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Stroke&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">8 &#40;10&#46;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Acroparesthesias&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;1&#46;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Deafness&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 &#40;2&#46;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
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                0 => "xTab2244595.png"
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        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Baseline characteristics of patients with left ventricular noncompaction &#40;n&#61;78&#41;&#46;</p>"
        ]
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    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:45 [
            0 => array:3 [
              "identificador" => "bib0230"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Fabry disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "D&#46;P&#46; Germain"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:4 [
                        "tituloSerie" => "Orphanet J Rare Dis"
                        "fecha" => "2010"
                        "volumen" => "22"
                        "paginaInicial" => "30"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0235"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "High incidence of later-onset Fabry disease revealed by newborn screening"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
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Article information

ISSN: 21742049
Original language: English

DOI:10.1016/j.repce.2020.01.008

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Year/Month	Html	Pdf	Total

2024 November	7	6	13
2024 October	43	26	69
2024 September	53	31	84
2024 August	211	41	252
2024 July	38	23	61
2024 June	40	33	73
2024 May	31	28	59
2024 April	28	30	58
2024 March	30	26	56
2024 February	23	19	42
2024 January	30	32	62
2023 December	30	29	59
2023 November	48	35	83
2023 October	23	11	34
2023 September	22	25	47
2023 August	19	17	36
2023 July	21	12	33
2023 June	25	17	42
2023 May	43	38	81
2023 April	30	11	41
2023 March	80	21	101
2023 February	45	19	64
2023 January	24	21	45
2022 December	69	32	101
2022 November	58	24	82
2022 October	115	28	143
2022 September	81	47	128
2022 August	73	27	100
2022 July	87	38	125
2022 June	40	30	70
2022 May	41	31	72
2022 April	43	40	83
2022 March	28	38	66
2022 February	44	41	85
2022 January	34	39	73
2021 December	22	41	63
2021 November	22	28	50
2021 October	27	29	56
2021 September	26	28	54
2021 August	28	21	49
2021 July	23	22	45
2021 June	20	24	44
2021 May	25	32	57
2021 April	52	45	97
2021 March	30	23	53
2021 February	27	13	40
2021 January	32	11	43
2020 December	28	10	38
2020 November	22	20	42
2020 October	29	19	48
2020 September	30	10	40
2020 August	12	6	18
2020 July	10	11	21
2020 June	16	14	30
2020 May	22	7	29
2020 April	19	5	24
2020 March	25	11	36
2020 February	78	13	91

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