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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.
MedLine Citation:
PMID:  23953779     Owner:  NLM     Status:  Publisher    
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.
Jing Xian Quah; Lou Hofmeyr; Haris Haqqani; Andrew Clarke; Atifur Rahman; Peter Pohlner; Dorothy Radford; Mugur Nicolae
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-8-18
Journal Detail:
Title:  Congenital heart disease     Volume:  -     ISSN:  1747-0803     ISO Abbreviation:  Congenit Heart Dis     Publication Date:  2013 Aug 
Date Detail:
Created Date:  2013-8-19     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101256510     Medline TA:  Congenit Heart Dis     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2013 Wiley Periodicals, Inc.
Adult Congenital Heart Disease Unit, The Prince Charles Hospital, Brisbane, Qld, Australia.
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