CLINICAL INTRODUCTION: A 14-year-old boy presented with history of decreased appetite and bilateral swelling of feet for 6 months. He did not give any associated history of orthopnoea or paroxysmal nocturnal dyspnoea. He was born by a normal delivery after a non-consanguineous marriage. He had an unremarkable birth and childhood health history. There was no family history of significant cardiovascular illness or sudden death. Clinical examination showed an average built boy with elevated jugular venous pressure with prominent v wave and bilateral pitting pedal oedema. Cardiovascular examination showed normal first (S1) and second (S2) heart sounds and a short early systolic murmur over tricuspid region. Other systems examination was remarkable for soft tender hepatomegaly.ECG showed sinus rhythm with tall, peaked p waves. Chest X-ray revealed enlargement along the right cardiac border. Transthoracic echocardiographic images are shown in figure 1A (apical four-chamber view) and figure 1B (tricuspid inflow Doppler). There was no colour Doppler evidence of interatrial shunt.heartjnl;105/5/405/F1F1F1Figure 1(A) Transthoracic echocardiographic apical four-chamber view. (B) Tricuspid inflow continuous wave Doppler image. QUESTION: What is the most likely diagnosis of his condition? Endomyocardial fibrosis (EMF)Ebstein's anomalyArrhythmogenic right ventricular dysplasia (ARVD)Idiopathic dilatation of right atriumRestrictive cardiomyopathy.
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http://dx.doi.org/10.1136/heartjnl-2018-313646 | DOI Listing |
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