Hypoplastic left heart syndrome. Experience with an operation to establish functionally normal circulation.

A new operation to establish functionally normal circulation was performed in five neonates with hypoplastic left heart syndrome. With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the patent ductus arteriosus was ligated. The atrial septum was excised and the atrium was repartitioned with a pericardial baffle so that pulmonary veins were in continuity with the tricuspid valve and right ventricle. The pulmonary artery was divided above the sinuses of Valsalva, and the proximal end was connected to the aortic arch with a tubular Dacron prosthesis. The distal end of the pulmonary artery was brought in continuity with the right atrium by way of a second Dacron conduit. The right ventricle thereby became the systemic ventricle and blood passed through the lungs vis a tergo. Circulation was supported temporarily, but all of the patients died from inadequate right ventricular performance or compromised coronary blood flow. This experience is presented to stimulate thought and some hope for babies with a uniformly fatal cardiac anomaly.