Background.– ALCAPA syndrome is a rare congenital heart disease, mainly diagnosed during the first months of life with a high early mortality in symptomatic children in the absence of surgery. Adult form is extremely rare and no recommendation for its management is available. We undertook this study to analyze initial presentation, management and long-term outcome of adult patients with ALCAPA.

Methods.– We analyzed retrospectively the data of patients hospitalized for a first diagnosis or management of ALCAPA from the charts of cardiology departments of seven French hospitals.

Results.– Twelve adults (30.2 ± 21.4 years) were included. Symptoms consisted of chest pain (58.3%), supra-ventricular arrhythmia (42.9%) and heart failure (33.3%). 60% of the ECG were abnormals (ischemic signs). Echocardiogram showed a mean left ventricular ejection fraction of 51.5 ± 12.8%. Mitral regurgitation was present in 83.3%. Coronary angiography was performed in 83.3% of patients and always showed ALCAPA. CT-scan, myocardial scintigraphy and MRI were performed for diagnosis or follow-up (FU) respectively in 45.5%, 41.7% and 27.3% of the cases. Reconstructive surgery was performed in 11 patients and a mitral valve reconstruction in one patient. Immediate post-operative complications were: cardiogenic shock (two patients), non-significant narrowing of the transferred coronary artery ostium that was treated medically (one patient) and a significant stenosis of the pulmonary artery trunk and of the left coronary artery required surgery (one patient). The only patient who refused surgery died two years later. Because of pulmonary hypertension due to a severe ischemic heart disease, one patient had a coil in the left coronary artery before undergoing a coronary artery bypass grafting. All operated patients are alive with a FU of 21.3 ± 21.2 years.

Conclusion.– Diagnosis of ALCAPA in adult patients is very rare and is confirmed mainly by coronary angiography in presence of atypical symptoms. Considering its complicated natural course and the good surgical results, surgery should probably be proposed systematically even in asymptomatic adults, in case of accidental discovery.