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PubMed1994

Origin of both coronary arteries from the pulmonary artery.

Origin of both coronary arteries from the pulmonary artery is generally a lethal condition from progressive ventricular failure. We report the clinical and surgical course of two infants, ages 3 and 6 months, with this anomaly. One patient had normal intracardiac anatomy with low pulmonary artery pressures (30/12 mm Hg). The second patient had a restrictive subpulmonic ventricular septal defect with a moderately elevated pulmonary artery pressure (50/13 mm Hg). Left ventricular ejection and shortening fractions were profoundly depressed in both patients. The common coronary trunk arose from the right anterior facing sinus in one patient and from the left posterior facing sinus in the other. Both patients underwent repair by direct coronary implantation to the aorta. Left ventricular function improved with shortening fractions near normal at a follow-up of 6 months for one patient and 1 year for the other. Early diagnosis and prompt repair is compatible with survival and return of normal shortening fraction.

Coronary Vessel Anomalies↗

PubMed1994

Improving results with first-stage palliation for hypoplastic left heart syndrome.

Between January 1990 and February 1993, 73 patients underwent first-stage reconstruction for hypoplastic left heart syndrome at the University of Michigan Medical Center. During this period, surgical reconstruction remained essentially constant and consisted of a pulmonary artery-to-aorta anastomosis with allograft augmentation of the ascending, transverse, and proximal descending aorta, restriction of pulmonary blood flow with a polytetrafluoroethylene shunt from the innominate artery to the central pulmonary artery confluence, and atrial septectomy. Hospital survival was 62 of 73 patients, 85% (70% confidence limits: 80% to 89%). These results stand in marked contrast to those obtained during the earlier years of our experience from 1986 to 1989 when only 21 of 50 patients (42%, 70% confidence limits: 35% to 49%) survived (p = 0.001). Among the most recent group of patients, only 2 of 7 patients older than 1 month of age at operation survived, whereas 60 of 66 (91%, 70% confidence limits: 87% to 94%) patients younger than 1 month of age survived (p = 0.0001). Anatomic subtype and ascending aortic diameter were not predictive of survival. Actuarial survivals for those patients younger than 1 month of age at the first-stage operation, including hospital deaths and subsequent operative procedures, were 81%, 74%, and 74% at 6 months, 1 year, and 2 years, respectively. These results indicate that survival for patients after first-stage reconstruction for hypoplastic left heart syndrome has significantly improved in recent years. Older age was a strong risk factor, with a hospital survival of 91% for those patients undergoing first-stage palliation within the first month of life. These data have important implications for the type of operative intervention and its timing.

Actuarial Analysis↗