Between 1988 and 2008, 35 consecutive patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) underwent surgery. The anomalous left coronary artery (LCA) was reimplanted in 29 and it was ligated in 6. Patients were evaluated during a median follow up of 82 months.There was one early death in the reimplanted group. (3,4%) All survivors showed significant improvement of LV function. Shortening fraction increased from X=21% (10-44%) to X=39% (29-49%). MR was identified in 80% of patients preoperatively and it improved in 53% of them after the LCA reimplantation. Fifteen patients were cathetherized. Five showed obstruction of the LCA. Three of these a had normal exercise test and only two showed abnormal ECG changes. Four of the five patients with occluded LCA had abnormal scintigraphy. Ninety one percent are asymptomatic. Only two patients with the LCA occluded had chest pain.
Two of the 6 patients with a ligated LCA died. One had severe LV disfunction and died shortly after surgery and the other died suddenly at the age of 15. All 4 survivors have normal shortening fraction. Conclusions: Reestablishment of a dual coronary artery system showed less early and late mortality than ligation. LV systolic function improved in all survivors despite some had occlusion of the LCA. Echocardiography and exercise test were good tools to assess the potential myocardial insult of survivors but did not predict the two coronary artery patency. Thus, coronary angiography is mandatory during follow up.