Surgical treatment of anomalous origin of coronary artery from the pulmonary artery.

BACKGROUND

Anomalous origin of coronary artery from the pulmonary artery is a rare congenital cardiac malformation with a mortality rate of up to 90% within the first year of life without surgical intervention. Direct implantation of the anomalous coronary artery (ACA) into the aorta is successful in early life, but it may have increased surgical difficulty and risk with age. This retrospective study summarized our operative experience in direct implantation for treatment of this coronary anomaly in pediatric and adult patients.

METHODS

From August 2000 to January 2003, 4 consecutive patients aged from 9 months to 41 years underwent dual coronary repair. Among them, two children and one infant with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and one adult was anomalous origin of right coronary artery from the pulmonary artery (ARCAPA). Coronary arteries were directly implanted into the ascending aorta in 4 patients. In a boy with ALCAPA associated with moderate mitral insufficiency (MI), whose ACA arose remotely from the ascending aorta, we created a tube-shaped graft using part of the pulmonary arterial wall in continuity with the origin of the left coronary artery (LCA). Concomitant moderate MI was repaired in 2 patients, including this boy, after a dual-coronary repair.

RESULTS

All patients survived. There were no hospital or late deaths and no major complications as well. Echocardiography revealed that the left ventricular (LV) function including LV end-diastolic dimension (EDD) and ejection fraction (EF) was markedly improved at hospital discharge. At 3 - 6 years follow-up after surgery all patients were asymptomatic and currently in NYHA class I.

CONCLUSIONS

The best results are achieved with direct implantation of the ACA into the ascending aorta and simultaneous mitral valve repair if needed. Direct implantation is feasible in pediatric and adult patients with ALCAPA or ARCAPA including the coronary artery in a location remote from the ascending aorta. It is a good procedure to lengthen the ACA by creating a tube-shape graft using part of the pulmonary arterial wall in continuity with the origin of ACA.