Echo showed large apical VSD with separation of RV apex from the remaining RV by excessive trabeculations, thereby eliminating any left to right shunt across the VSD; a rare and distinct type of morphology and physiology. Physiologically, there was no apparent hemodynamic disturbance. Right ventricular basal cavity size and inflow (tricuspid annular diameter: 16 mm Z score-1.86) and outflow tract were normal. Normal aorta and main pulmonary artery with confluent and good-sized branch pulmonary arteries. Apical segment distal to moderator band was integrated to LV and resultant apical dilatation of LV. Large apical muscular defect of ventricular septum closed by the moderator band.

Cyanosis and clubbing is due to resultant functional hypoplastic RV cavity with increased RV end-diastolic pressure and increased RA pressure with right to left shunt across large ASD. This type of apical VSD constitutes a rare and distinct type of morphology and physiology and has to be considered in patients with suspected LV aneurysm, absence of CAD and lack of any heart murmur.

Our patient was symptomatic with shortness of breath, cyanosed and failed to gain weight with a real challenge in planning his management.