Early Result of Surgical Management of the Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

Yoon, Yoo Sang; Park, Jeong Jun; Yun, Tae Jin; Kim, Young Hwue; Ko, Jae Kon; Park, In Sook; Seo, Dong Man

Abstract

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, but is one of the most common causes of myocardial ischemia which would result in high mortality within the first year of life. This is our early result of the surgical management for these patients. MATERIAL AND METHOD: From June 1989 to July 2003, 6 patients with ALCAPA and one patient with ARCAPA (Anomalous origin of the Right coronary artery from the pulmonary artery) underwent surgical repair. We have reviewed the all medical records, electrocardiogram, chest X-ray and echocardiography retrospectively. RESULT: Three of the patients were boys and four were girls. The median age at the operation was 5.4 months (Range: 3~33 months). The average body weight of at the operation was 6.7 kg (Range: 3.7~11.3 kg). A mean follow up period was 18 months. Only 3 patients were initially diagnosed as ALCAPA. And 3 patients had moderate mitral regurgitation. Immediate coronary artery reimplantation on diagnosis with the aim of restoring a two-coronary system circulation was done. The average bypass time was 114+/-37 minutes, and the average aortic cross clamping time was 55+/-22 minutes. The average stay of intensive care unit was 5+/-3 days, the mean mechanical ventilator time was 38+/-45 hours and the hospital stay after operation was 12+/-5 days. There were significant improvements in electrocardiogram and chest X-ray of the all patients except one late death patient. The ventricular function showed almost normal recovery after operation; the EF (Ejection Fraction) increased from 41.2+/-10.3% to 60.5+/-15.8% within 1 month and to 59.8+/-13.9% within 1 year after operation, the SF (Shortening Fraction) increased from 23.6+/-4.7% to 38.6+/-8.4% within 1 month and to 37.4+/-7.9% within 1 year after operation, LVEDDI (Left Ventricular End-diastolic Dimension Index) decreased from 100.8+/-25.6 mm/m2 to 90.3+/-19.2 mm/m2 within 1 month and to 79.3+/-15.8 mm/m2 within 1 year after operation. Concomitant mitral repair was done in two patients with anterior mitral leaflet prolapse. In every patient, mitral valve showed less than mild regurgitation during follow up. One late death occurred in which patient Dor procedure was applied 10 months after initial operation due to the dilated cardiomyopathy.
CONCLUSION
In the management of this rare and could be fatal Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), early suspicion and correct diagnosis is of most important.But, after diagnosis, immediate restoration of 2 coronary systems could result in good outcome.