The aim of the study was to assess the long-term outcome of patients with ALCAPA.

Retrospective single-center analysis of patients who underwent surgery for ALCAPA from 1980 to 2012. Surgical techniques, demographics, echocardiographic parameters and outcomes were collected. Patients were divided into group I<2years at diagnosis, group II:>2years).

Forty-eight patients (28 females) were included, median age 6 months (min 4 months, max 65 years), median weight 6kg (min 1.9kg): 36 in group I and 12 in II. HF was patent in 39 patients (mean age 4 years), 9 were asymptomatic (mean age 10 years), Qwave on ECG was present in 87% of cases. Mean LVSF=24.2% (group I=26.6% vs II=37.7%, and 16.6% in patients<6 months of age), LVEDD and LVESD Z-scores were respectively +2 and +5 in groups I and II; 73% had MR: severe in 6%, moderate in 46%, mild in 21%. Left coronary artery ostium located in the left posterior sinus in 31cases, right posterior sinus in 12 and in right pulmonary branch in 4. Direct coronary artery reimplantation was performed in 71%, Takeuchi technique in 6%, Meyer technique in 20% and LCA ligation in 3%. Mean age at surgery was 29 months, mean weight 9kg. Postoperative mechanical circulatory support was required in 3 cases, who had more severe HF, lower LVSF and longer bypass duration. Mean FU was 81 months (6 to 312 months). Freedom from reoperation was 100% at 1 year, 91% at 10 years and 88% at 20 years. LVSF increased by 20% in the early postoperative course and 36% at late FU. MR improved significantly in most of the cases. Overall mortality was 33% (15 in group I died before day 30, none in group II), decreasing over time from 55% to 11%, and was lower in patients who had direct reimplantation. Q wave disappeared in 82% of the cases; 93% of survivors were asymptomatic at latest evaluation.

ALCAPA patients have good long-term survival and outcome. Age>2 years at diagnosis and direct implantation are factors of favorable prognosis.