Anomalous origin of the left coronary artery from the pulmonary artery: results of surgical correction in five infants.

BACKGROUND: Five infants operated on for anomalous origin of the left coronary artery from the pulmonary artery were retrospectively analyzed. The mean age at operation was 12 +/- 6.7 weeks (95% confidence interval, 3.5 to 20 weeks), and mean weight at operation was 4.43 +/- 0.68 kg (95% confidence interval, 3.7 to 5.27 kg). All babies presented in infancy with left ventricular failure. Three had evidence of ischemia with left ventricular strain, and two had Q waves in anterolateral leads on electrocardiograph. Cross-sectional echocardiography showed dilated left ventricles with poor contractility in all babies with fractional shortening of 15.8% +/- 4.02% (95% confidence interval, 12% to 20%); moderate mitral regurgitation was seen in all babies.

METHODS: All babies underwent operation as soon as the diagnosis was made. Four babies had direct reimplantation of left coronary artery into the aorta, and 1 had tunnel repair. Intraaortic balloon counterpulsation was used in 1 baby for hemodynamic instability and as prophylaxis in the remaining 4 babies postoperatively for 115 +/- 26.2 hours (95% confidence interval, 72 to 144 hours).

RESULTS: All babies had delayed closure of the chest. There was no operative mortality. One baby was reoperated on for tunnel stenosis as well as pulmonary stenosis 4 months after primary repair. All babies were followed for 192 patient-months and show an improved fractional shortening.

CONCLUSIONS: Early operation, early institution of intraaortic balloon counter pulsation for left ventricular support, and delayed sternal closure are the key to good results.