Anomalous origin of the left coronary artery from the pulmonary artery: new electrocardiographic, echocardiographic and surgical observations.

The case of a two year old girl with anomalous origin of the left coronary artery from the pulmonary artery is described. She was never in heart failure but had cardiomegaly and anginal pain. The ECG showed a typical infarct pattern with left ventricular hypertrophy. An unusual finding was a prolonged QTc of 0.52. During cardiac catheterization and twice 24 hours later she developed ventricular fibrillation treated with electroshock and prevented later with propranolol. The QTc returned to normal after surgery. Echocardiography showed diastolic flutter and early systolic closure of the pulmonary valve. This disappeared after surgical correction. Transverse 2D echo of the aortic root showed a large right coronary artery which decreased in size after surgery. The left coronary artery was not seen on echocardiography. At cardiac catheterization the diagnosis of an anomalous origin of the left coronary artery from the pulmonary artery was established, with a large shunt to the pulmonary artery through the anomalous artery. Mild pulmonary hypertension and mild mitral regurgitation were present. At surgery, since direct implantation was technically impossible, the left coronary artery was successfully connected to the aorta via a 6 mm expanded Poly-Tetra-Fluoro-Ethylene (P.T.F.E.) graft.