AN INTERESTING CASE OF BIVENTRICULAR NONCOMPACTION

Isolated noncompaction of ventricular myocardium is a form of cardiomyopathy characterized by a pattern of prominent trabecular meshwork and deep intertrabecular recesses.It is thought to be caused by arrest of normal endomyocardial morphogenesis.Previous reports show in biventricular noncompaction, left ventricle is involved predominantly than right ventricle.Here we present a case of biventricular noncompaction where right ventricle is involved more than left ventricleassociated with skeletal deformity and developmental delay.The 23 year old man presented with complaints suggestive of congestive heart failure of 9 month duration.Examination revealed raised JVP with normal auscultatory findings.Echo showed prominent trabeculations involving both ventricles with right ventricle being involved more.Colour Doppler shoed entry of blood into these recesses in continuity with ventricular cavity.Contrast Echo showed free entry into and out of these recesses.MRI showed prominent trabeculations involving both ventricles.It also showed an end-systolic ratio of noncompacted to compacted myocardium ratio of more than 2.0.