Arrhythmogenic Right Ventricular Dysplasia (ARVD) in Childhood: Case Report with a Review of the Literature

Abstract

AS Al Jarallah, Arrhythmogenic Right Ventricular Dysplasia (Arvd) in Childhood: Case Report with a Review of the Literature. 1997; 17(3): 350-353 Over the last few decades of advances in the field of cardiology, many newly identified diseases have been described. One of these newly recognized conditions was arrhythmogenic right ventricular dysplasia (ARVD), which was first described in 1978 by Frank et al. 1 The clinical spectrum of this condition was described by Marcus et al. in 1982. 2 Estimates of the prevalence of ARVD vary sharply in the literature. Thiene et al. found a startling 20% incidence of ARVD in a study of sudden deaths in young adults originating from northeast Italy. 3 In contrast, in two retrospective studies of sudden deaths in young American adults, the incidence of ARVD was found to be 0.5%-3.0%. 4 In this condition, areas of fatty and fibrous tissues replace the normal myocardium of the right ventricle. The involved myocardium evokes ventricular arrhythmias of right ventricular origin. The diagnosis of ARVD in the past was based on the presence of ventricular arrhythmia with left bundle branch block configuration and morphologic changes or motion abnormalities in the free wall of the right ventricle. 2 Recently, standardized diagnostic criteria have been proposed, based on the presence of major and minor criteria encompassing structural, histological, electrocardiographic, arrhythmia, and genetic factors. 5 Although the long-term prognosis of ARVD is generally good, the prevalence is unknown because of its rarity, especially in the pediatric age group. A male child with ARVD is reported here, with a review of the literature. Case ReportA 12-year-old boy presented with an 18-month history of progressive shortness of breath and recurrent vomiting. Nine months prior to admission, he was seen in a private clinic with severe dyspnea and tachycardia (heart rate of 216 beats/min), where he was diagnosed with heart failure and treated with digoxin, furosemide and spironolactone. There was no history of rheumatic fever or endocarditis and no family history of heart disease.General examination revealed a puffy child, mildly dyspneic, and pink in color. Weight was in the 50th percentile, height in the 10th percentile, temperature was normal, respiratory rate was 22 per minute. Pulse was 110 per minute, regular, good volume, with no radio-femoral delay. Blood pressure was 110/75 mmHg in the right upper arm. Precordial examination revealed no chest deformity, with quiet precordium, no thrill. First and second heart sounds were muffled, third heart sound was audible (gallop rhythm) with a grade 3/6 pansystolic murmur at the left lower sternal border and the lung fields were clear with equal air entry. Abdomen was slightly distended with mild ascites and tender hepatomegaly (6 cm below the right costal margin). There was pitting edema involving both lower limbs.Chest x-ray showed globular enlargement of the heart (cardiothoracic ratio 70%) (Figure 1). The 12-leads electrocardiography showed sinus rh...

Arrhythmogenic right ventricular cardiomyopathy with biventricular involvement and heart failure in a 9-year old girl

“…There was a case report from Saudi Arabia of a 12-year-old boy who presented with history of progressive shortness of breath and enlarged heart before developing ventricular arrhythmias [6]. Another 11-year-old Saudi girl presented with multiple ventricular ectopics, tiredness, and palpitations with typical features of ARVC on echocardiogram [7].…”