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

Abstract

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.