Congenital absence of aortic valvar leaflets: a rare variant of the hypoplastic left heart syndrome.

Congenital absence of aortic valvar leaflets is a rare and fatal variant of the hypoplastic left heart syndrome. We describe a recent patient seen at our institution with this lesion, illustrating a combined echocardiographic and angiographic approach that delineates both anatomy and physiology. The early mortality experienced in previous reports, as well as unsuccessful surgical palliation in our case, should promote further discussion regarding the optimal treatment.