Case report
Uhl's disease: An uncommon presentation of a rare disease
Doença de Uhl, uma doença rara com uma apresentação incomum
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Pinto, Rui Cruz Ferreira" "autores" => array:10 [ 0 => array:4 [ "nombre" => "Sílvia" "apellidos" => "Aguiar Rosa" "email" => array:1 [ 0 => "silviaguiarosa@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Ana Figueiredo" "apellidos" => "Agapito" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Marta" "apellidos" => "António" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 3 => array:3 [ "nombre" => "Lídia" "apellidos" => "de Sousa" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "José Alberto" "apellidos" => "Oliveira" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 5 => array:3 [ "nombre" => "Sérgio" "apellidos" => "Laranjo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 6 => array:3 [ "nombre" => "Susana" "apellidos" => "Martins" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">d</span>" "identificador" => "aff0020" ] ] ] 7 => array:3 [ "nombre" => "Nuno Jalles" "apellidos" => "Tavares" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 8 => array:3 [ "nombre" => "Fátima F." "apellidos" => "Pinto" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 9 => array:3 [ "nombre" => "Rui Cruz" "apellidos" => "Ferreira" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:4 [ 0 => array:3 [ "entidad" => "Cardiology Department, Santa Marta Hospital, Lisbon, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Paediatric Cardiology Department, Santa Marta Hospital, Lisbon, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Centro de Ressonância Caselas, Lisbon, Portugal" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Cardiology Department, Santa Maria Hospital, Lisbon, Portugal" "etiqueta" => "d" "identificador" => "aff0020" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Doença de Uhl, uma doença rara com uma apresentação incomum" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 896 "Ancho" => 2917 "Tamanyo" => 149784 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Late gadolinium enhancement (LGE) acquired 10 minutes after intravenous gadolinium administration. LGE is evident in the right ventricular and right atrium walls, in the four-chamber view (A), right ventricle view (B) and short axis view (C).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Uhl's anomaly was first described by Osler in 1905. The exact incidence of this rare condition is unknown. In 1993, Gerlis et al. concluded that many cases of arrhythmogenic right ventricular dysplasia (ARVD) were incorrectly classified as Uhl's anomaly. In said paper, the authors discussed 84 real Uhl's disease cases, and since then fewer cases have been reported.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">1</span></a> Uhl's anomaly is a rare disease secondary to the selective but uncontrolled apoptosis of right ventricular myocytes during the perinatal period, following complete cardiac development. This explains the absence of the right ventricular myocardium and the direct apposition of endocardium to epicardium without a myocardial layer in between, resulting in right ventricular failure.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Furthermore, some studies suggest that the overexpression of vascular endothelial growth factor may play a role in impairing the development of the right ventricular myocardium.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">In Uhl's disease, the absence of the right ventricular myocardium leads to a lack of contraction and, consequently, the chamber acts as a transition zone between the right atrium and pulmonary artery. Pulmonary circulation is maintained by the pumping action of the right atrium, whereas the right ventricle (RV) balloons aneurysmally in systole. The right atrium is enormous in size.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">In the past, diagnosis was performed during autopsy. However, nowadays, given the development of imaging techniques, diagnosis is carried out via echocardiography or more sensitive and specific cardiac magnetic resonance (CMR) imaging.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">4</span></a> Moreover, there are also published cases where diagnosis took place during prenatal screening.<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">5</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0025" class="elsevierStylePara elsevierViewall">The authors report the case of a 28-year-old man, with no known heart disease and an insignificant personal and family medical history. However, he did note that he had suffered cyanosis over the past three years, which was never investigated, and had no other symptoms. The patient was admitted to the hospital due to a respiratory infection, presenting with fever, dyspnea and hypoxemia (peripheral oxygen saturation of 55%).</p><p id="par0030" class="elsevierStylePara elsevierViewall">Electrocardiogram (ECG) showed atrial fibrillation (±77 bpm), right bundle branch block and a fragmented QRS complex – despite normal left ventricular activation, right ventricular depolarization was delayed and prolonged (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">A chest X-ray revealed cardiomegaly, with a narrow vascular pedicle (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>). Computed tomography of the chest showed dilated right heart chambers, inferior vena cava and hepatic veins, with no dilatation of the pulmonary artery. Pulmonary ventilation/perfusion scintigraphy ruled out a pulmonary embolism and indicated a right-to-left shunt. Transthoracic echocardiography confirmed severe dilation of the right heart chambers and impaired right ventricular systolic function (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>); a tricuspid valve with normal implantation and moderate regurgitation; an estimated pulmonary artery systolic pressure of 36 mmHg; a normal pulmonary artery diameter; and a large <span class="elsevierStyleItalic">ostium secundum</span> atrial septal defect (ASD) (38 mm), confirmed by transesophageal echocardiography.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Cardiac catheterization was performed, revealing an absence of gradient between the RV and pulmonary artery and post-capillary pulmonary hypertension (mean pulmonary artery pressure of 32 mmHg, with an elevated pulmonary capillary wedge pressure of 19 mmHg). A pulmonary blood flow:systemic blood flow ratio of 0.88:1 was also calculated.</p><p id="par0045" class="elsevierStylePara elsevierViewall">At this point, the authors felt that ASD as the primary diagnosis could not explain the clinical features or hemodynamic data. On one hand, there was no anomalous venous return to justify the cyanosis and mild postcapillary pulmonary hypertension was not compatible with Eisenmenger's syndrome and the consequent right-to-left shunt. On the other hand, there was no evidence of a significant past or present left-to-right shunt, since the pulmonary artery was normal in size.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Primary right ventricular disease was the most likely hypothesis and CMR was performed, revealing severe dilation of the right atrium (91×72 mm) and right ventricle (end-diastolic volume 355 ml, 207.9 ml/m<span class="elsevierStyleSup">2</span>), an extremely thin-walled RV with almost complete absence of the right ventricular free wall myocardium, a paucity of apical trabeculations and impaired systolic function (ejection fraction [EF] 25%) (<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>). The left ventricle was dilated with normal wall thickness and an EF of 41%. An ASD measuring over 30 mm was identified and late gadolinium enhancement was observed in the right ventricle and right atrium walls (<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>). These findings were compatible with Uhl's anomaly.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">Cardiopulmonary exercise testing showed a moderate exercise capacity with a peak oxygen uptake of 27.6 ml/kg/min (55% of the value predicted for the patient's gender and age) as well as an oxygen uptake at anaerobic threshold of 20.5 ml/kg/min and a minute ventilation-to-carbon dioxide output slope of 46.2.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The patient initiated anticoagulation therapy with warfarin and received a subcutaneous implantable cardioverter-defibrillator for primary prevention of sudden cardiac death, due to severe right ventricular dysfunction. The patient will be closely monitored and heart transplantation will be considered in the event his functional capacity deteriorates.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0065" class="elsevierStylePara elsevierViewall">Uhl's disease is characterized by the absence of the RV myocardium and the direct apposition of endocardium to epicardium.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">2</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Histological examination confirms cardiac muscle wastage and replacement by fibrous tissue.<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">6</span></a> However, despite this anomaly in the RV myocardium, the septal and left ventricular myocardium is preserved.<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">4,7</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">In severe forms, RV failure develops during childhood, with pulmonary circulation being dependent on the right atrium. Cyanosis is also present in the event of an atrial shunt.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">4</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Prior to the advent of current imaging methods, diagnosis was performed during autopsy. Nowadays, CMR is the method of choice for diagnosis, as well as for morphologic and functional evaluation.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">4</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">The association of Uhl's disease with other congenital heart defects had rarely been reported, namely with tricuspid valve dysplasia or absence, Ebstein's anomaly, pulmonary atresia or regurgitation, patent ductus arteriosus, ASD and hypoplasia of the mitral valve and left ventricle.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">1,8–12</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">A differential diagnosis should be performed with ARVD. From a histological point of view, ARVD is characterized by fibrofatty infiltration of the right ventricular free wall which is absent in Uhl's disease, where fibrosis predominates.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">13</span></a> Unlike ARVD, the major clinical finding in Uhl's disease is heart failure, rather than arrhythmic events and sudden death.<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">6,10</span></a> Family history is also more common in ARVD than in Uhl's disease.<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">10</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">Regarding electrocardiographic studies, in ARVD, T-wave inversions in V1 and V2 are noted, along with Epsilon waves. Uhl's disease, on the other hand, presents a fragmented QRS complex.<a class="elsevierStyleCrossRefs" href="#bib0120"><span class="elsevierStyleSup">7,13</span></a> In CMR, Uhl's disease is characterized by an extremely dilated thin-walled RV with severe hypokinesis, a paucity of trabeculations and no fat signal in the RV wall, as seen in ARVD. In ARVD, focal wall motion abnormalities are more frequent, contrasting with the global hypokinesis seen in Uhl's disease.<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">4,7,13</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Ebstein's anomaly should also be considered in differential diagnosis. In Uhl's disease the tricuspid valve hinges normally and is not dysplastic.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">4</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">With regard to surgical treatment, a right heart bypass can be performed. However, this procedure carries a risk of clots forming in the right ventricle as well as embolization, particularly in the presence of a bidirectional Glenn shunt.<a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">14</span></a> Another option involves almost entirely resecting the RV free wall.<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">15</span></a> Possible complications include recurrent ventricular dilatation and arrhythmias. In these cases, a residual RV free wall must be ruled out and a total cavopulmonary connection performed.<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">16</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">If clinical deterioration persists, the treatment approach should be orthotopic cardiac transplantation.<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">17</span></a></p><p id="par0115" class="elsevierStylePara elsevierViewall">In our case, the association of RV myocardium disease with a large ASD explains the patient's unique clinical evolution.</p><p id="par0120" class="elsevierStylePara elsevierViewall">Cyanosis in the presence of an ASD (with a “normal” RV) may occur in two circumstances that were not present in this case: severe pulmonary hypertension with Eisenmenger's syndrome and the consequent right-to-left shunt, or when an anomalous systemic venous return exists.</p><p id="par0125" class="elsevierStylePara elsevierViewall">Furthermore, the lack of pulmonary artery dilation shows that, although the ASD is large, there was probably no significant left-to-right shunt. The inexistence of such a shunt is due to the fact that a shunt is dependent of the compliance of both ventricles in ASDs, and in this case only the RV was affected. Over the years, progressive RV dysfunction led to a right-to-left shunt and consequent cyanosis. Moreover, the defect acts as an escape, which has until now prevented the occurrence of overt right-sided heart failure.</p><p id="par0130" class="elsevierStylePara elsevierViewall">The ECG is also notable, with a fragmented QRS complex, reflecting the delay and prolongation of right ventricular depolarization.</p><p id="par0135" class="elsevierStylePara elsevierViewall">In conclusion, this case shows that clinical symptoms, hemodynamic data and imaging findings must be comprehensively and thoroughly reviewed in congenital heart disease in order to enable correct diagnosis. Primary RV disease should be considered when RV failure is present in the absence of valve disease and pulmonary hypertension. Uhl's anomaly is a rare disease that consists in the absence of the RV myocardium and progressive RV failure.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of interest</span><p id="par0140" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres1269357" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1174873" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1269358" "titulo" => "Resumo" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1174874" "titulo" => "Palavras-chave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflicts of interest" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-02-28" "fechaAceptado" => "2017-06-11" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1174873" "palabras" => array:3 [ 0 => "Uhl's disease" 1 => "Primary right ventricular disease" 2 => "Cardiac magnetic resonance imaging" ] ] ] "pt" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palavras-chave" "identificador" => "xpalclavsec1174874" "palabras" => array:3 [ 0 => "Doença de Uhl" 1 => "Doença primária do ventrículo direito" 2 => "Ressonância magnética cardíaca" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Uhl's disease, also known as Uhl anomaly, is a rare disease secondary to selective but uncontrolled apoptosis of right ventricular myocytes during the perinatal period, after complete cardiac development, leading to the absence of right ventricular myocardium and the direct apposition of endocardium to epicardium without a myocardial layer in between, resulting in right ventricular failure.</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">The present paper describes a case of Uhl's disease with an uncommon presentation. A 28-year-old man was admitted with dyspnea and cyanosis. Transthoracic echocardiography showed severe dilation of the right chambers, impaired right ventricular systolic function and a large <span class="elsevierStyleItalic">ostium secundum</span> atrial septal defect (ASD). Cardiac catheterization revealed pulmonary hypertension, with increased pulmonary capillary wedge pressure (mean 19<span class="elsevierStyleHsp" style=""></span>mmHg) and Qp:QS 0.88:1. At this point, the authors considered that a main diagnosis of ASD could not explain the clinical features and hemodynamic data. A primary disease of the right ventricle was the most likely hypothesis and cardiac magnetic resonance imaging was performed, which demonstrated an extremely thin-walled right ventricle, with almost complete absence of right ventricular free wall myocardium, compatible with Uhl's disease.</p></span>" ] "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A anomalia de Ulh é uma doença rara, secundária à seletiva, contudo descontrolada apoptose dos miócitos do ventrículo direito durante o período perinatal, após o desenvolvimento cardíaco completo, o que explica a ausência de miocárdio do ventrículo direito e a direta aposição do endocárdio e epicárdio, resultando em falência do ventrículo direito. O presente artigo descreve um caso de doença de Uhl com apresentação incomum. Homem de 28 anos admitido por dispneia e cianose. O ecocardiograma transtorácico documentou dilatação grave das cavidades direitas, compromisso da função sistólica do ventrículo direito e uma grande comunicação interauricular <span class="elsevierStyleItalic">ostium secundum.</span> O cateterismo cardíaco revelou hipertensão pulmonar com aumento da pressão capilar (média 19mmHg); Qp:Qs 0,88:1. Neste momento, os autores consideraram que a comunicação interauricular como diagnóstico principal não explicava a apresentação clínica e os dados hemodinâmicos. Doença primária do ventrículo direito tornou-se a hipótese mais provável, pelo que se realizou uma ressonância magnética cardíaca que demonstrou um ventrículo direito com parede extremamente fina, com ausência praticamente total do miocárdio da parede livre, compatível com doença de Uhl.</p></span>" ] ] "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" => 1777 "Ancho" => 2917 "Tamanyo" => 315363 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">A: Chest X-ray revealing cardiomegaly with no pulmonary artery dilatation; B: Electrocardiogram showing atrial fibrillation and right bundle branch block. C: Transthoracic echocardiography showing severe right chambers dilation, particularly of the right atrium, and a large <span class="elsevierStyleItalic">ostium secundum</span> atrial septal defect.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 928 "Ancho" => 2917 "Tamanyo" => 142308 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Cardiovascular magnetic resonance: Steady-state free precession imaging documenting severe dilation of the right atrium and right ventricle, an extremely thin-walled right ventricle with almost complete absence of the right ventricular free wall myocardium, with a paucity of apical trabeculations, in a four-chamber view in diastole (A) and systole (B), and coronal view (C). An atrial septal defect measuring over 30 mm was also identified.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 896 "Ancho" => 2917 "Tamanyo" => 149784 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Late gadolinium enhancement (LGE) acquired 10 minutes after intravenous gadolinium administration. LGE is evident in the right ventricular and right atrium walls, in the four-chamber view (A), right ventricle view (B) and short axis view (C).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:17 [ 0 => array:3 [ "identificador" => "bib0090" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Dysplastic conditions of the right ventricular myocardium: Uhl's anomaly vs arrhythmogenic right ventricular dysplasia" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "L.M. Gerlis" 1 => "S.C. Schmidt-Ott" 2 => "S.Y. Ho" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1136/hrt.69.2.142" "Revista" => array:6 [ "tituloSerie" => "Br Heart J" "fecha" => "1993" "volumen" => "69" "paginaInicial" => "142" "paginaFinal" => "150" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/8435240" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0095" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Images in cardiovascular medicine, fortuitous discovery of partial Uhl anomaly in a male adult" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "J.L. Hébert" 1 => "G. Duthoit" 2 => "F. Hidden-Lucet" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1161/CIRCULATIONAHA.110.960773" "Revista" => array:6 [ "tituloSerie" => "Circulation" "fecha" => "2010" "volumen" => "121" "paginaInicial" => "e426" "paginaFinal" => "e429" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20530016" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0100" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "VEGF induces cardiovascular malformation and embryonic lethality" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "M. Feucht" 1 => "B. Christ" 2 => "J. Wilting" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Am J Pathol" "fecha" => "1997" "volumen" => "151" "paginaInicial" => "1407" "paginaFinal" => "1416" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9358767" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0105" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "MRI of Uhl's anomaly" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "M.L. Greer" 1 => "C. MacDonald" 2 => "I. Adatia" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1161/01.cir.101.24.e230" "Revista" => array:6 [ "tituloSerie" => "Circulation" "fecha" => "2000" "volumen" => "101" "paginaInicial" => "E230" "paginaFinal" => "E232" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/10859296" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0110" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Prenatal diagnosis of uhl anomaly with autopsy correlation" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "S. Philip" 1 => "S. Bharati" 2 => "K.M. Cherian" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1055/s-0035-1566296" "Revista" => array:7 [ "tituloSerie" => "AJP Rep" "fecha" => "2016" "volumen" => "6" "paginaInicial" => "e91" "paginaFinal" => "e95" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26929879" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0140673609607315" "estado" => "S300" "issn" => "01406736" ] ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0115" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Uhl's anomaly complicated with critical ventricular arrhythmia in a 2-month-old infant" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "Y. Tanoue" 1 => "H. Kado" 2 => "Y. Shiokawa" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.ejcts.2003.09.016" "Revista" => array:6 [ "tituloSerie" => "Eur J Cardiothorac Surg" "fecha" => "2003" "volumen" => "24" "paginaInicial" => "1040" "paginaFinal" => "1042" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/14643833" "web" => "Medline" ] ] ] ] ] ] ] ] 6 => array:3 [ "identificador" => "bib0120" "etiqueta" => "7" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Images in cardiovascular medicine, electrocardiogram, echocardiography, and magnetic resonance imaging characteristics in Uhl's disease" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "A. Otmani" 1 => "L. Leborgne" 2 => "C. Renard" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1161/CIRCULATIONAHA.106.630061" "Revista" => array:6 [ "tituloSerie" => "Circulation" "fecha" => "2007" "volumen" => "115" "paginaInicial" => "e11" "paginaFinal" => "e12" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17228005" "web" => "Medline" ] ] ] ] ] ] ] ] 7 => array:3 [ "identificador" => "bib0125" "etiqueta" => "8" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Uhl's anomaly: clinical spectrum and pathophysiology" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "R.L. van der Palen" 1 => "A.C. van der Wal" 2 => "P.G. Robles de Medina" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.ijcard.2016.02.048" "Revista" => array:6 [ "tituloSerie" => "Int J Cardiol" "fecha" => "2016" "volumen" => "209" "paginaInicial" => "118" "paginaFinal" => "121" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26889594" "web" => "Medline" ] ] ] ] ] ] ] ] 8 => array:3 [ "identificador" => "bib0130" "etiqueta" => "9" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Uhl's anomaly with absent tricuspid valve in an infant" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "A.M. Taksande" 1 => "V. Gautami" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.4103/2211-4122.166086" "Revista" => array:7 [ "tituloSerie" => "J Cardiovasc Echogr" "fecha" => "2015" "volumen" => "25" "paginaInicial" => "90" "paginaFinal" => "92" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28465943" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0168822712003191" "estado" => "S300" "issn" => "01688227" ] ] ] ] ] ] ] 9 => array:3 [ "identificador" => "bib0135" "etiqueta" => "10" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Uhl's anomaly, differential diagnosis and indication for cardiac transplantation in an infant" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "N.M. Ikari" 1 => "E. Azeka" 2 => "V.D. Aiello" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1590/s0066-782x2001000700008" "Revista" => array:7 [ "tituloSerie" => "Arq Bras Cardiol" "fecha" => "2001" "volumen" => "77" "paginaInicial" => "69" "paginaFinal" => "76" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11500750" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0140673605663787" "estado" => "S300" "issn" => "01406736" ] ] ] ] ] ] ] 10 => array:3 [ "identificador" => "bib0140" "etiqueta" => "11" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Unusual right ventricle aneurysm and dysplastic pulmonary valve with mitral valve hypoplasia" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "O. Pamukcu" 1 => "A. Ozyurt" 2 => "M. Argun" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.4103/0974-2069.115272" "Revista" => array:6 [ "tituloSerie" => "Ann Pediatr Cardiol" "fecha" => "2013" "volumen" => "6" "paginaInicial" => "167" "paginaFinal" => "169" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24688237" "web" => "Medline" ] ] ] ] ] ] ] ] 11 => array:3 [ "identificador" => "bib0145" "etiqueta" => "12" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Isolated congenital pulmonary regurgitation with right ventricular outflow tract aneurysm – a rare variant of Uhl's anomaly" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "K. Vaidyanathan" 1 => "R. Agarwal" 2 => "R. Johari" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1540-8191.2010.01057.x" "Revista" => array:6 [ "tituloSerie" => "J Card Surg" "fecha" => "2010" "volumen" => "25" "paginaInicial" => "415" "paginaFinal" => "417" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20529159" "web" => "Medline" ] ] ] ] ] ] ] ] 12 => array:3 [ "identificador" => "bib0150" "etiqueta" => "13" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "F.I. Marcus" 1 => "W.J. McKenna" 2 => "D. Sherrill" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1161/CIRCEP.117.005959" "Revista" => array:4 [ "tituloSerie" => "Circulation" "fecha" => "2010" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/29752376" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0168822715004647" "estado" => "S300" "issn" => "01688227" ] ] ] ] ] ] ] 13 => array:3 [ "identificador" => "bib0155" "etiqueta" => "14" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Successful surgical repair of Uhl's anomaly" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "N. Azhari" 1 => "M. Assaqqat" 2 => "Z. Bulbul" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1017/s1047951102000410" "Revista" => array:6 [ "tituloSerie" => "Cardiol Young" "fecha" => "2002" "volumen" => "12" "paginaInicial" => "192" "paginaFinal" => "195" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12018730" "web" => "Medline" ] ] ] ] ] ] ] ] 14 => array:3 [ "identificador" => "bib0160" "etiqueta" => "15" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A case of Uhl anomaly treated with one and a half ventricle repair combined with partial right ventriculectomy in infancy" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "S. Yoshii" 1 => "S. Suzuki" 2 => "S. Hosaka" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1067/mtc.2001.116319" "Revista" => array:6 [ "tituloSerie" => "J Thorac Cardiovasc Surg" "fecha" => "2001" "volumen" => "122" "paginaInicial" => "1026" "paginaFinal" => "1028" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11689812" "web" => "Medline" ] ] ] ] ] ] ] ] 15 => array:3 [ "identificador" => "bib0165" "etiqueta" => "16" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "S. Sano" 1 => "K. Ishino" 2 => "M. Kawada" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "J Thorac Cardiovasc" "fecha" => "2002" "volumen" => "123" "paginaInicial" => "640" "paginaFinal" => "647" ] ] ] ] ] ] 16 => array:3 [ "identificador" => "bib0170" "etiqueta" => "17" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Uhl's anomaly, Differential diagnosis and indication for cardiac transplantation in an infant" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "N.M. Ikari" 1 => "E. Azeka" 2 => "V.D. Aiello" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1590/s0066-782x2001000700008" "Revista" => array:6 [ "tituloSerie" => "Arq Bras Cardiol" "fecha" => "2001" "volumen" => "77" "paginaInicial" => "69" "paginaFinal" => "76" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11500750" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/08702551/0000003700000012/v3_201911281020/S0870255117301555/v3_201911281020/en/main.assets" "Apartado" => array:4 [ "identificador" => "29263" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Casos Clínicos" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/08702551/0000003700000012/v3_201911281020/S0870255117301555/v3_201911281020/en/main.pdf?idApp=UINPBA00004E&text.app=https://revportcardiol.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0870255117301555?idApp=UINPBA00004E" ]
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| 2024 Abril | 87 | 35 | 122 |
| 2024 Maro | 118 | 29 | 147 |
| 2024 Fevereiro | 109 | 26 | 135 |
| 2024 Janeiro | 99 | 23 | 122 |
| 2023 Dezembro | 103 | 22 | 125 |
| 2023 Novembro | 123 | 42 | 165 |
| 2023 Outubro | 90 | 25 | 115 |
| 2023 Setembro | 74 | 20 | 94 |
| 2023 Agosto | 85 | 23 | 108 |
| 2023 Julho | 102 | 17 | 119 |
| 2023 Junho | 111 | 17 | 128 |
| 2023 Maio | 140 | 29 | 169 |
| 2023 Abril | 89 | 10 | 99 |
| 2023 Maro | 125 | 23 | 148 |
| 2023 Fevereiro | 105 | 20 | 125 |
| 2023 Janeiro | 66 | 21 | 87 |
| 2022 Dezembro | 100 | 23 | 123 |
| 2022 Novembro | 133 | 37 | 170 |
| 2022 Outubro | 131 | 33 | 164 |
| 2022 Setembro | 104 | 40 | 144 |
| 2022 Agosto | 115 | 41 | 156 |
| 2022 Julho | 118 | 43 | 161 |
| 2022 Junho | 110 | 34 | 144 |
| 2022 Maio | 64 | 38 | 102 |
| 2022 Abril | 83 | 37 | 120 |
| 2022 Maro | 59 | 43 | 102 |
| 2022 Fevereiro | 58 | 39 | 97 |
| 2022 Janeiro | 74 | 41 | 115 |
| 2021 Dezembro | 51 | 36 | 87 |
| 2021 Novembro | 70 | 39 | 109 |
| 2021 Outubro | 109 | 62 | 171 |
| 2021 Setembro | 84 | 40 | 124 |
| 2021 Agosto | 85 | 47 | 132 |
| 2021 Julho | 66 | 29 | 95 |
| 2021 Junho | 73 | 40 | 113 |
| 2021 Maio | 86 | 42 | 128 |
| 2021 Abril | 172 | 55 | 227 |
| 2021 Maro | 108 | 28 | 136 |
| 2021 Fevereiro | 82 | 29 | 111 |
| 2021 Janeiro | 73 | 21 | 94 |
| 2020 Dezembro | 78 | 25 | 103 |
| 2020 Novembro | 68 | 21 | 89 |
| 2020 Outubro | 90 | 25 | 115 |
| 2020 Setembro | 92 | 26 | 118 |
| 2020 Agosto | 62 | 12 | 74 |
| 2020 Julho | 62 | 17 | 79 |
| 2020 Junho | 75 | 22 | 97 |
| 2020 Maio | 101 | 13 | 114 |
| 2020 Abril | 75 | 26 | 101 |
| 2020 Maro | 79 | 13 | 92 |
| 2020 Fevereiro | 171 | 23 | 194 |
| 2020 Janeiro | 49 | 11 | 60 |
| 2019 Dezembro | 58 | 12 | 70 |
| 2019 Novembro | 61 | 11 | 72 |
| 2019 Outubro | 62 | 16 | 78 |
| 2019 Setembro | 67 | 12 | 79 |
| 2019 Agosto | 46 | 12 | 58 |
| 2019 Julho | 48 | 20 | 68 |
| 2019 Junho | 60 | 23 | 83 |
| 2019 Maio | 52 | 16 | 68 |
| 2019 Abril | 50 | 21 | 71 |
| 2019 Maro | 42 | 14 | 56 |
| 2019 Fevereiro | 66 | 18 | 84 |
| 2019 Janeiro | 118 | 33 | 151 |
| 2018 Dezembro | 64 | 38 | 102 |
| 2018 Novembro | 37 | 16 | 53 |
| 2018 Outubro | 62 | 24 | 86 |
| 2018 Setembro | 9 | 16 | 25 |
| 2018 Agosto | 13 | 26 | 39 |
| 2018 Julho | 6 | 24 | 30 |
Mostrar tudo
