Background  

Anomalous left coronary artery arising from the pulmonary artery is a rare congenital heart disease. Mortality is in excess of 90% if left untreated during the first year of life. Surgical management has evolved from coronary ligation to anatomical repair by coronary button translocation to the aorta. We report our recent experience with surgical correction of this rare condition.

Methods  

We retrospectively studied the case records of 14 consecutive patients operated in our institute between Jan 1998 to Aug 2004. The mean age was 65 months ±114 (Range 1 to 420). The male to female ratio was 2.5:1. Coronary ligation ws performed in two patients, coronary artery bypass grafting with saphenous vein graft was done in three, the last nine patients underwent coronary button transfer, one patient required concomitant mitral valve replacement. Clinical profile, surgical techniques and operative outcomes as well as long-term results were analyzed.

Results  

Hospital mortality was 28.5% (4/14), Follow up was 100% complete with no late mortality, ranging from 1 months to five years. Ischaemic mitral regurgitation progressed in only one patient, remained the same in one and improved in all the others. All patients except one are in functional class I at last follow up.

Conclusion  

In conclusion, infants presenting with a diagnosis of anomalous left coronary artery arising from pulmonary artery with congestive cardiac failure are a difficult subgroup of patients. Coronary button translocation has emerged as the single most effective surgical modality and is associated with good long-term results.