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  • 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

    Author(s)
    Quah, JX
    Hofmeyr, L
    Haqqani, H
    Clarke, A
    Rahman, A
    Pohlner, P
    Radford, D
    Nicolae, M
    Griffith University Author(s)
    Rahman, Atifur
    Year published
    2014
    Metadata
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    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, ...
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    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.
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    Journal Title
    Congenital Heart Disease
    Volume
    9
    Issue
    6
    DOI
    https://doi.org/10.1111/chd.12125
    Subject
    Cardiovascular medicine and haematology
    ALCAPA
    Cardiomyopathy
    Coronary Artery Anomaly
    Implantable Cardioverter Defibrillator
    Myocardial Ischemia
    Publication URI
    http://hdl.handle.net/10072/399278
    Collection
    • Journal articles

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