'Caterpillar right ventricle': unusual manifestation of a rare disease. Issue 2 (28th September 2017)

• Record Type: Journal Article
• Authors: Kalliath, Suneesh; Rajesh, Gopalan Nair
• Is Part Of: Heart Asia. Volume 9:Issue 2(2017)
• Publication Date: 2017-09-28
• Subjects: arrhythmogenic right ventricular dyplasia -- cardiomyopathy restrictive -- cardiomyopathy apical
• DOI: 10.1136/heartasia-2017-010957
• Languages: English
• ISSNs: 2398-5968

Abstract

Abstract : 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) . 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