Editorial comment
Left ventricular noncompaction and Fabry disease: An unlikely association
Não-compactação do ventrículo esquerdo e doença de Fabry: uma associação improvável
José Ribeiro
Centro Hospitalar de Vila Nova de Gaia/Espinho EPE, Serviço de Cardiologia, Vila Nova de Gaia, Portugal
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"https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2174204920300106?idApp=UINPBA00004E" "url" => "/21742049/0000003800000010/v2_202002180803/S2174204920300106/v2_202002180803/en/main.assets" ] "en" => array:12 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Editorial comment</span>" "titulo" => "Left ventricular noncompaction and Fabry disease: An unlikely association" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "717" "paginaFinal" => "719" ] ] "autores" => array:1 [ 0 => array:3 [ "autoresLista" => "José Ribeiro" "autores" => array:1 [ 0 => array:3 [ "nombre" => "José" "apellidos" => "Ribeiro" "email" => array:1 [ 0 => "cardiogaia@gmail.com" ] ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Centro Hospitalar de Vila Nova de Gaia/Espinho EPE, Serviço de Cardiologia, Vila Nova de Gaia, Portugal" "identificador" => "aff0005" ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Não-compactação do ventrículo esquerdo e doença de Fabry: uma associação improvável" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Left ventricular noncompaction (LVNC) describes a ventricular wall anatomy characterized by prominent left ventricular (LV) trabeculae, forming a thin compacted layer, and deep intertrabecular recesses that are continuous with the LV cavity and separated from the epicardial coronary arteries.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">1</span></a> In the normal heart, trabeculae actively provide mechanical leverage by contracting during early systolic ejection.<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">2</span></a> Trabeculae are formed during early embryonic development. The origin of LVNC is attributed to an arrest in compaction of the endomyocardial layer of the heart during early embryogenesis.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The diagnostic criteria for LVNC are based on ratios between thickness, mass or volume of noncompacted and compacted left ventricle. The number of noncompacted segments provides information on the extension of LVNC. However, this approach is highly investigator-dependent; diagnosis is based on two-dimensional planes using semi-quantitative or qualitative criteria and specificity is low.<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">3</span></a> Grothoff et al. demonstrated that, on cardiovascular magnetic resonance imaging (CMRI), trabeculation in segments 4-6 indicates a high probability of LVNC and distinguishes LVNC from other cardiomyopathies and normal hearts.<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">4</span></a> Alternative CMRI-based methods include assessment of the global trabeculation index.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">5</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Some authors suggest that the established diagnostic criteria are too sensitive and that LVNC is over-diagnosed.<a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">6</span></a> LVNC can be an incidental finding in screening studies, such as for athletes,<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">7</span></a> and is not associated with deterioration in LV volumes or function during long-term follow-up in the asymptomatic population.<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">8</span></a> When faced with isolated LVNC morphology, cardiologists must decide whether what they are observing is a cardiomyopathy or a variant LV wall anatomy. In most cases, especially in adult patients, the key element in the diagnostic decision is not the LVNC itself, but the associated LV dilation and/or dysfunction, hypertrophy, right ventricular involvement, arrhythmias and conduction disturbances. The genetic basis of LVNC is an issue of ongoing research, severely limited by the enrollment criteria, which reflect the current heterogeneous diagnostic definitions of LVNC.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">1</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The weakness of a diagnosis based solely on proportions or ratios is demonstrated by using Fabry disease (FD) as an example. The hearts of patients with FD may exhibit prominent papillary muscles and trabeculae<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">9</span></a> which may reach LVNC criteria,<a class="elsevierStyleCrossRefs" href="#bib0175"><span class="elsevierStyleSup">10,11</span></a> which are fulfilled for trabecular thickness rather than for a thin compacted layer. Similarly, in patients with Danon disease with LVNC,<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">12</span></a> the criteria for diagnosis of LVNC seem to be met due to the prominent trabeculae with a thickened compacted layer.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The occurrence of hypertrabeculation and left ventricular noncompaction (LVNC) is increasingly reported in large echocardiographic series.<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">8</span></a> LVNC can be regarded as an isolated entity or as one of the traits that may recur in cardiac and noncardiac diseases.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">1</span></a> On the basis of current definitions and terminology, LVNC can occur in various settings, including as an isolated finding, or associated with LV dilatation and dysfunction or with cardiomyopathies or congenital heart disease, or acquired and potentially reversible, as has been reported in athletes.<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">1,7</span></a> However, the association of Fabry disease (FD) with LVNC has been reported in a few cases.<a class="elsevierStyleCrossRefs" href="#bib0175"><span class="elsevierStyleSup">10,11</span></a> It is possible that unidentified mutations in genes other than <span class="elsevierStyleItalic">GLA</span> (responsible for FD) may coexist, which would explain the association found.</p><p id="par0030" class="elsevierStylePara elsevierViewall">FD is a progressive multisystemic X-linked genetic sphingolipidosis caused by deficient activity of lysosomal alpha-galactosidase A (α-Gal A).<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">13</span></a> Accumulation of α-Gal A substrates in various cells and organs produces the clinical phenotype in FD.<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">14</span></a> FD can mimic other myocardial diseases, including sarcomeric hypertrophic cardiomyopathy (HCM) and amyloid cardiomyopathy. Cardiac imaging, in particular echocardiography and CMRI, plays an important role in detecting this peculiar disease.<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">15</span></a> Although increased left ventricular (LV) wall thickness has traditionally been the hallmark feature of FD, several other structural and functional abnormalities have been uncovered by conventional and novel echocardiographic techniques. Among patients with increased LV wall thickness due to various etiologies, those with FD tend to have more prominent papillary muscles, in both thickness and hyperechogenicity. Systolic function is generally preserved, but can be reduced in advanced disease, usually associated with extensive fibrosis.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">16</span></a> CMRI is an excellent method to reveal the presence of fibrosis in FD, which is commonly located in the posterolateral basal and mid-level or subepicardial layers.<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">13</span></a> Magnetic resonance noncontrast myocardial T1 mapping may show glycosphingolipid deposits before the onset of left ventricular hypertrophy (LVH), and is also a useful method for differentiating FD from other causes of LVH.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">17</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">FD is a multisystemic disease, and depending on the affected organs it can result in neurological, ocular, skin, renal or cardiac manifestations. Therefore, cardiologists, neurologists, dermatologists, nephrologists and ophthalmologists should all be aware of the possibility of FD, depending on the patient's clinical presentation. In a cardiological setting, a diagnosis of FD should be considered systematically in cases of unexplained LVH, particularly when it is concentric, symmetric, homogeneous or non-obstructive.<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">18</span></a> LVH is a key feature in FD and is reported in up to 50% of male patients and one-third of female patients.<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">19</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">LVH in FD typically combines concentric thickening without LV obstruction and normal LV ejection fraction. However, asymmetric septal or apical hypertrophy has also been described, along with subaortic obstruction, which may mimic the phenotypical and clinical features of sarcomeric HCM.<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">20</span></a> Right ventricular hypertrophy with preserved systolic function, impaired left atrial function and moderate aortic dilatation may also be observed, and LV hypertrabeculation and noncompaction have been described.<a class="elsevierStyleCrossRefs" href="#bib0205"><span class="elsevierStyleSup">16,21</span></a> LV function may deteriorate with time, leading to a restrictive cardiomyopathy pattern. Other echocardiographic features include prominent papillary muscles<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">22</span></a> and a binary appearance of the LV endocardial border,<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">16</span></a> although the diagnostic value of these findings is controversial.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Specific therapy should be initiated at the earliest stage, before cardiac fibrosis develops, when the first structural or functional cardiac abnormalities are detected. Options include enzyme replacement therapy (ERT) or chaperone therapy. ERT with recombinant human α-Gal (rh-α-Gal) has been available to treat FD since 2001 and may improve ventricular morphology and function.<a class="elsevierStyleCrossRefs" href="#bib0190"><span class="elsevierStyleSup">13,23</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">In 2003, Stöllberger et al. showed that LVNC does not appear to be a manifestation of cardiac FD.<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">11</span></a> They studied 26 patients with LVNC diagnosed by echocardiographic criteria and ruled out FD by assessment of clinical systemic manifestations and blood tests for α-Gal A. The most common detection method is the measurement of α-Gal A activity by dried blood spot (DBS) analysis. Although decreased α-Gal A activity in DBS can confirm the diagnosis of FD in homozygous males, this is not the most reliable diagnostic method in heterozygous females, since enzyme activity may be within the normal range in around 40% of this patient group. Therefore, women with high clinical suspicion should undergo genotyping to confirm the diagnosis. Endomyocardial biopsy is the gold standard for diagnosis of cardiac involvement in doubtful cases. Caution should be exercised in clinical assessment because certain drugs such as amiodarone, chloroquine, and tamoxifen have a storage pattern mimicking FD.<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">13</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">There are three cases in the literature documenting an association of LVNC and FD in the same patient, all three of them female.<a class="elsevierStyleCrossRefs" href="#bib0175"><span class="elsevierStyleSup">10,24</span></a> In this issue of the <span class="elsevierStyleItalic">Journal</span>, Azevedo et al. report a multicenter study screening for FD in 78 patients with LVNC.<a class="elsevierStyleCrossRef" href="#bib0250"><span class="elsevierStyleSup">25</span></a> The diagnosis of LVNC was established by echocardiography in 91.0% of the patients and that of FD by α-Gal A activity measured in DBS samples by fluorometry. Molecular analysis of the <span class="elsevierStyleItalic">GLA</span> gene was performed in males with reduced α-Gal A activity. Screening of these 78 patients with LVNC did not identify any additional patients with FD. This supports the hypothesis that although LVNC may occur in patients with FD, this does not represent more than a coincidence of findings.</p><p id="par0060" class="elsevierStylePara elsevierViewall">This article, therefore, strengthens the evidence that there is no pathophysiological link between these two conditions and that LV noncompaction is unlikely to be a phenotypical expression of FD.</p><p id="par0065" class="elsevierStylePara elsevierViewall">In clinical practice, for the management of secondary cardiomyopathies such as FD, echocardiographic study has an important role in screening and diagnosis and in assessment of cardiac function and hemodynamics. Since ERT is usually a major component of treatment for FD, early diagnosis is essential for effective treatment. Based on recent evidence, caution should be exercised when using the current diagnostic criteria of LVNC as a basis for the decision to start specific therapy for FD.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The author has no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:25 [ 0 => array:3 [ "identificador" => "bib0130" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Left ventricular noncompaction: a distinct genetic cardiomyopathy?" 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| 2021 January | 29 | 16 | 45 |
| 2020 December | 40 | 15 | 55 |
| 2020 November | 21 | 21 | 42 |
| 2020 October | 15 | 14 | 29 |
| 2020 September | 24 | 16 | 40 |
| 2020 August | 12 | 6 | 18 |
| 2020 July | 14 | 15 | 29 |
| 2020 June | 12 | 10 | 22 |
| 2020 May | 19 | 12 | 31 |
| 2020 April | 13 | 14 | 27 |
| 2020 March | 29 | 12 | 41 |
| 2020 February | 60 | 11 | 71 |
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