Anomalous left coronary artery from the pulmonary artery (ALCAPA) diagnosed in adulthood: Varied clinical presentation, therapeutic approach and outcome

• Published in: International journal of cardiology 2018-06, Vol.261, p.49-53
• Main Authors: Boutsikou, M., Shore, D., Li, W., Rubens, M., Pijuan, A., Gatzoulis, M.A., Babu-Narayan, S.V.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Adults; Aged; ALCAPA; Bland White Garland Syndrome - diagnostic imaging; Bland White Garland Syndrome - surgery; Coronary Vessel Anomalies - diagnostic imaging; Coronary Vessel Anomalies - surgery; Delayed Diagnosis - trends; Female; Follow-Up Studies; Humans; Ischaemia; Male; Middle Aged; Mitral regurgitation; Myocardial Revascularization - methods; Myocardial Revascularization - trends; Pulmonary Artery - abnormalities; Pulmonary Artery - diagnostic imaging; Pulmonary Artery - surgery; Retrospective Studies; Treatment Outcome; Young Adult
• ISSN: 0167-5273; 1874-1754
• DOI: 10.1016/j.ijcard.2018.02.082

The diagnosis of ALCAPA syndrome is sporadic in adulthood, of the limited cases in the literature most are incidental or without symptoms. There is a broad spectrum of clinical manifestations of ALCAPA syndrome however, including sudden cardiac death. We present herewith a series of 12 consecutive patients with ALCAPA, all diagnosed in adulthood (between 18 and 73 years of age). Five patients developed symptoms (breathlessness) after the fourth decade of life, 3 were undiagnosed despite a history of previous mitral valve repair, one presented with heart failure, one with resuscitated cardiac arrest, whereas two patients were asymptomatic. We review in this paper, the clinical history, diagnostic approach and therapeutic choices of ALCAPA syndrome. ALCAPA syndrome is not confined to childhood, late diagnosis in adulthood has a varied clinical presentation. ALCAPA syndrome should be particularly considered as a potential, albeit uncommon cause of mitral regurgitation and/or dilated cardiomyopathy. •ALCAPA syndrome may present in late adulthood with a varied clinical presentation.•Angina and shortness of breath are among the most common symptoms in adults with ALCAPA syndrome.•Mitral regurgitation and LV dilatation are common presentations of the syndrome and are frequently overlooked.•Multimodality imaging is crucial for the initial diagnosis and follow up.•Patients with inducible ischaemia should be offered surgical repair as it provides symptomatic and prognostic benefit.