'Caterpillar right ventricle: unusual manifestation of a rare disease

A 66-year-old man presented with abdominal distension and pedal oedema for the past 10 years. He had history of right heart failure on several occasions in the past, and one of these admissions prompted referral to a cardiac specialist. On examination, he had markedly elevated jugular venous pressure with prominent ‘y-descent’, a tricuspid regurgitation murmur, gross ascites and pedal oedema. A full blood count, routine biochemical screen and serum bicarbonate levels were normal. Right ventricular angiogram revealed a ‘caterpillar’-like aneurysm of the right ventricle (RV) apex (figure 1 arrow) with a dilated right ventricular outflow tract and significant tricuspid regurgitation with a dilated right atrium (see online supplementary video 1)supplementary video 1). Figure 1 Acine angiographic frame of the right ventricle in posteroanterior view, showing a ‘caterpillar’-like right ventricle aneurysm (arrow). Question On the basis of the clinical and right ventriculography features, what is the most likely diagnosis for this patient?Arrhythmogenic right ventricular cardiomyopathy (ARVC) Right ventricular endomyocardial fibrosis (RV-EMF) with an RV aneurysm Cardiac sarcoidosis Congenital diverticulum of the right ventricle
Source: Heart Asia - Category: Cardiology Authors: Tags: Image challenge Source Type: research