Isolated right ventricular non-compaction: A rare form of dilated cardiomyopathy

Noncompaction of the ventricular myocardium is a cardiomyopathy thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. This abnormality is often associated with other congenital cardiac defects but it is also seen in the absence of other cardiac anomalies. Isolated ventricular non-compaction is a morphological abnormality of excessive (atleast four) trabeculation and deep inter-trabecular spaces communicating with the ventricular cavity, with typical bilaminar structure of compacted and noncompacted segment of ventricle. It is often complicated by ventricular dysfunction, arrhythmias and cardioembolism. Although the usual site of involvement is the left ventricle, the right ventricle (RV) can rarely be affected. Isolated right ventricle involvement is very rare and to the best of our knowledge only few cases of isolated RV noncompaction are reported. Thus herein we report a case of 40-year-old male patient who presented with features of right sided heart faiure, atrial fibrillation and history of syncope. ECG showed RBBB with junctional rhythm. Echocardiography showed enlarged right atrium and right ventricle showed double layered structure with prominent trabecula and deep intertrabecular recesses. Left ventricle was normal. Cardiac MRI further confirmed the diagnosis of isolated right ventricle noncompaction. Thus clinician should keenly look for this rare cardiomyopathy as an important differential diagnosis of patient presenting with right sided heart failure.