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Congenit Heart Dis. 2014 Nov-Dec;9(6):E185-94. doi: 10.1111/chd.12125. Epub 2013 Aug 18.

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.

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  • 1Adult Congenital Heart Disease Unit, The Prince Charles Hospital, Brisbane, Qld, Australia.


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.

© 2013 Wiley Periodicals, Inc.


ALCAPA; Cardiomyopathy; Coronary Artery Anomaly; Implantable Cardioverter Defibrillator; Myocardial Ischemia

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