Aortic atresia and hypoplastic left ventricle

The spectrum of hypoplastic left heart syndrome spans a continuum from borderline hypoplasia to extreme forms with retrograde blood flow from the ductus arteriosus to the cerebral and coronary arteries. A male neonate, born at 37 weeks’ gestation, had an antenatal diagnosis of hypoplastic left heart syndrome. Postnatal echocardiographic assessment confirmed the diagnosis of hypoplastic left heart syndrome with minimal antegrade flow in the aortic sinus, retrograde arch perfusion, and nondemonstrable ascending aorta. As a result of initial instability with recurrent infections, bilateral pulmonary artery banding was performed. Intraoperatively, a thread-like ascending aorta was found (Figure 1A), which warranted further investigation. Focused echocardiographic assessment could not visualize flow in the ascending aorta but demonstrated antegrade flow through a severely stenotic aortic valve with laminar color Doppler flow in the coronaries (Figure 2). Computed tomography angiography confirmed that the ascending aorta ended blindly above the sinotubular junction with antegrade coronary artery perfusion