Anomalous Left Coronary Artery Connected to the Pulmonary Artery / Single Center Experience Over 30 Years

Introduction: ALCAPA is a rare disease with almost 90% of spontaneous death and leads to ischemic cardiomyopathy and chronic mitral regurgitation. The purpose of this study is to evaluate the long-term follow-up of operated patients.
Methods: 48 patients were operated on between 1980 and 2013 including 36 less than 2 years old. A retrospective analysis of perioperative and long-term factors was performed on the clinical and ultrasonographic plan.
Results: Direct reimplantation was performed in 71% of cases; Meyer technique in 19% and Takeuchi technique in 6% .2 patients underwent a concomitant mitral valve surgery. 2 patients required postoperative circulatory support. Mean follow-up was 15 months [54-360 months]. Fractional shortening of the left ventricle has increased by almost 20% (p = 0.03) in the immediate postoperative and over 40% at 6 months (p = 0.01).This long-term increase was more pronounced in patients younger than 6 months (56%, p <0.001). 73% of patients had preoperative mitral insufficiency, 6% had severe mitral insufficiency. The mitral insufficiency has declined over time. Mortality was mainly 15 early deaths, exclusively in patients less than 2 years old and one late death due to arrhythmias. CPB average time was shown to be a mortality factor (p <0.01). Mortality showed a decrease of 60% in 30 years with 12% of deaths in the last 10 years. Direct reimplantation technique showed the best results. At follow-up, 93% of patients were NYHA I and 82% had a regression of necrosis signs on the ECG.
Conclusion: The diagnosis by ultrasound leading to early surgery has dramatically improved the prognosis. The discovery in adulthood because of the risk of sudden death warrant surgical correction. The restoration of a "two coronary" system by direct aortic reimplantation remains the treatment of choice.