Selective aortic root angiography in the hypoplastic left heart syndrome.

Selective aortic root angiography was performed in 18 patients with the clinical diagnosis of hypoplastic left heart syndrome in order to allow more precise correlation with echocardiography. Aortic valve atresia and severe hypoplasia of the left heart chambers subsequently were substaniated at necropsy in each instance. Wide variation in the size and shape of the aortic root and ascending aorta was demonstrated angiographically in these patients. In some patients, despite aortic valve atresia, the dimensions of the aortic root and sinuses of Valsalva at angiography were almost normal. In addition, this technique allowed the demonstration of primitive vascular communications between the coronary artery and left ventricular cavity in 1 patient (coronary-cameral fistula). The realization that the aortic root may approach nearly normal size in some patients with the hypoplastic left heart syndrome is essential to the echocardiographic diagnosis of this disease and to the differential diagnosis from other cardiac and noncardiac causes of severe neonatal congestive heart failure.