title: The management of the older adult patient with anomalous left coronary artery from the pulmonary artery syndrome: a presentation of two cases and review of the literature.
abstract: ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital abnormality that involves an anomalous insertion of the left coronary artery into the pulmonary artery. Ninety percent of patients present in the first year of life with signs and symptoms of heart failure or sudden cardiac death secondary to chronic myocardial ischemia. There have been an increasing number of reports of ALCAPA patients surviving to adulthood. There seems, however, to be a tendency to die suddenly in the third decade of life. Adult survivors are either asymptomatic or present with mitral regurgitation, cardiomyopathy, myocardial ischemia, or malignant arrhythmias. The management of the older patient presenting with symptoms resulting from ischemia and progressive left ventricular dysfunction remains a challenge. Treatment is largely based on guidelines for adult congenital heart disease management and an extrapolation of evidence from heart failure practice. Currently, surgical reimplantation of the anomalous coronary onto the aorta is the mainstay of treatment. The management of heart failure, sudden cardiac death, and ventricular arrhythmia present problems that are not addressed by reimplantation of the anomalous vessel alone. In this report, we present two cases with different modes of presentation and discuss treatment options.
authors: Adult Congenital Heart Disease Unit, The Prince Charles Hospital, Brisbane, Qld, Australia.
authors: Jing Xian
authors: JX
authors: Quah
authors: Lou
authors: L
authors: Hofmeyr
authors: Haris
authors: H
authors: Haqqani
authors: Andrew
authors: A
authors: Clarke
authors: Atifur
authors: A
authors: Rahman
authors: Peter
authors: P
authors: Pohlner
authors: Dorothy
authors: D
authors: Radford
authors: Mugur
authors: M
authors: Nicolae
mesh_terms: D000328:Adult; D000367:Age Factors; D063748:Bland White Garland Syndrome / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006348:Cardiac Surgical Procedures; D003097:Collateral Circulation; D017023:Coronary Angiography / Q000379:methods; D003326:Coronary Circulation; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D004452:Echocardiography; D004554:Electric Countershock / Q000295:instrumentation; D005260:Female; D006349:Heart Valve Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008875:Middle Aged; D020127:Recovery of Function; D012092:Replantation; D013318:Stroke Volume; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D016277:Ventricular Function, Left
keywords: ALCAPA; Cardiomyopathy; Coronary Artery Anomaly; Implantable Cardioverter Defibrillator; Myocardial Ischemia