Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery Presenting in Adulthood: a French Nationwide Retrospective Study

Laura Le Berre (1) , Alban-Elouen Baruteau (2) , Alain Fraisse (3) , Dominique Boulmier (4) , Maria Jimenez , Bruno Gallet , Karine Warin-Fresse (5) , Jacques Mansourati , Patrice Guerin (5)

1 CHU - BREST - Hôpital de la Cavale Blanche - CHRU Brest
2 CCML - Centre Chirurgical Marie Lannelongue
3 TIMONE - Hôpital de la Timone [CHU - APHM]
4 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]
5 ITX - ITX - unité de recherche de l'institut du thorax

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease usually diagnosed during the first months of life. Without surgical treatment, ALCAPA carries a high mortality risk, and disease presentation in adulthood is rare. We describe the diagnosis and management of patients presenting with ALCAPA in adulthood. This multicenter French nationwide retrospective study included adult patients diagnosed from 1980 to 2014. Eleven adult patients (mean age: 38 ± 17 years) were analyzed. All patients were symptomatic, presenting with chest pain, palpitations, heart failure, or syncope. Electrocardiogram was abnormal in 8 (73%) patients. Echocardiogram showed a mildly depressed left ventricular ejection fraction of 50 ± 13%, kinetic abnormalities in 5 (45%) patients, and significant mitral regurgitation in 8 (73%) patients. Coronary angiography was performed in 10 (91%) patients and confirmed the diagnosis. Computerized tomography scan, magnetic resonance imaging, and myocardial scintigraphy were performed when deemed necessary. Ten patients underwent reconstructive surgery, but 1 patient was not operated because of age. Four patients experienced postoperative complications including cardiogenic shock, heart failure, renal failure, or additional surgery. After a median follow-up of 2.5 years, all 10 operated patients were alive and asymptomatic, and the nonoperated patient had died at the age of 70 from syncope related to ventricular tachycardia. ALCAPA may be diagnosed in adults. Although complications may occur postoperatively, long-term outcome is favorable in adult patients undergoing surgical correction. Surgery should be discussed as first-line therapy in adults with ALCAPA.

Mots clés: Bland-White-Garland syndrome; ALCAPA; myocardial infarction; congenital heart disease

Domaines: Chirurgie; Cardiologie et système cardiovasculaire

Laura Le Berre, Alban-Elouen Baruteau, Alain Fraisse, Dominique Boulmier, Maria Jimenez, et al.. Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery Presenting in Adulthood: a French Nationwide Retrospective Study. Seminars in Thoracic and Cardiovascular Surgery, 2017, 29 (4), pp.486-490. ⟨10.1053/j.semtcvs.2017.08.018⟩. ⟨hal-01735777⟩