VACTERL association with double-chambered left ventricle: A rare occurrence
A 2-year-old male asymptomatic child came for echocardiogram referred from an orthopedic center. His past history was significant for various limb and urachal anomalies of VACTERL association including bilateral radial aplasia, club hand, absent thumbs, sacral agenesis, and urachal sinus. At 9 months of age, he was diagnosed to have apical muscular ventricular septal defects and secundum atrial septal defect by echocardiography done elsewhere. On examination, he had normal development and normal intelligence for his age. There were dysmorphic facial features with bilateral radial club hand, absent thumbs, and short upper and lower limbs []. Chest examination was normal with cardiac auscultation revealing regular heart sounds with no murmur. His ECG and Chest X-ray were unremarkable. Sacral X-ray showed partial sacral agenesis [].\nTransthoracic echocardiography demonstrated situs solitus with normal atrioventricular and ventriculoarterial concordance. There were no ventricular septal defects. The left ventricle (LV) was abnormally configured with a muscle band extending from the interventricular septum toward the apex dividing the LV into two separate contracting LV chambers. A typical “figure-of-eight” appearance was seen in the parasternal short-axis view in diastole indicating double-chambered left ventricle (DCLV) [ arrowheads, ]. During systole, the LV was seen leading to aorta []. There was laminar flow inside the LV with no intraventricular gradient. Rest of the cardiac examination was normal with good biventricular function.