Case Presentation

An 8.8-pound infant of a diabetic mother with prenatally suspected congenital heart disease was delivered via caesarean section at 35+2 weeks gestation and immediately intubated for severe hypoxemia. Initial transthoracic echocardiogram (TTE) demonstrated double inlet left ventricle (DILV) with a moderate sized bulboventricular foramen (BVF) and normally related great arteries, aka Holmes heart (Figure 1A-1B, Video 1). Additionally, there was severe biventricular hypertrophy (Figure 1C, Video 1), which confounded the initial echocardiographic diagnosis in multiple ways: creating the appearance of a more restrictive BVF (Figure 1D), a somewhat narrowed left ventricular outflow tract (LVOT, Figure 1E) and severe, bilateral atrioventricular valve regurgitation (AVVR, Figure 1A), the latter of which prompted the question of underlying cardiomyopathy.

Figure 1A: DILV. 2D and Color TTE, Four chamber view demonstrates a right sided right atrium and left sided left atrium with their two respective atrioventricular valves that both empty into a dilated, left-sided morphologic left ventricle. The Color panel shows severe atrioventricular valve regurgitation, which when taken with the severe hypertrophy prompted consideration of a possible underlying cardiomyopathy.

Figure 1B: Outflow tracts. 2D and Color TTE, Subcostal long axis view shows the normally related great artery relationship: the more anterior pulmonary artery arising from the posterior right ventricle and the more posterior aorta arising from the anterior left ventricle.

Video 1: DILV: 2D, TTE 4C Posterior to anterior sweep demonstrating the right and left atrioventricular valves both emptying into a large, dilated, left-sided morphologic left ventricle. The aorta is seen arising from this left ventricle first followed by a larger, more anterior pulmonary artery, which arises from a more posterior right ventricle, which is not well seen. There is severe biventricular hypertrophy, which is best appreciated during the sweep from the left ventricle into the aorta with near-complete obstruction of the left ventricular cavity.

Figure 1C: Severe hypertrophy. 2D TTE, PSAX view of the left ventricle at the level of the papillary muscles demonstrating the severe hypertrophy in the setting of being an infant of a diabetic mother. Of note, the very posterior right ventricle cannot be seen in this view.