Document Detail


Left coronary artery arteriovenous malformation presenting as a diastolic murmur with exercise intolerance in a child with a suspected familial vascular malformation syndrome.
MedLine Citation:
PMID:  18377467     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: Intracardiac arteriovenous malformations are rare and may be associated with sudden death in adults. This case report describes an intracardiac left coronary arteriovenous malformation in a 7-year-old boy with a suspected familial cutaneous vascular malformation syndrome. The patient presented with a diastolic murmur, exercise intolerance, chest pain, and a left ventricular mass.
METHODS: The left ventricular mass was initially identified by echocardiography. Subsequently, a computed tomography scan revealed the vascular nature of the lesion. We hypothesized that the lesion represented either an arteriovenous malformation (AVM) or a hemangioma. These lesions are thought to cause coronary steal and myocardial dysfunction. Skin biopsies of the patient's cutaneous lesions revealed capillary hyperplasia, which was not consistent with either hemangioma or AVM. Thus, a surgical biopsy and partial resection of the mass was performed.
RESULTS: The surgical pathology of the cardiac mass was consistent with an AVM. Within 6 months following partial resection of the mass, the patient unexpectedly developed a left ventricular pseudoaneurysm at the resection site and required re-operation. Although a portion of the mass remains, both the patient's chest pain and exercise tolerance have improved subjectively.
CONCLUSION: Patients with cutaneous vascular malformations and diastolic murmurs, as well as cardiac symptoms, should undergo echocardiography or alternative imaging modalities to screen for treatable pathological myocardial vascular malformations.
Authors:
Valerie A Schroeder; Eun Y Lee; William I Douglas; Carole M Cottrill
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Congenital heart disease     Volume:  2     ISSN:  1747-0803     ISO Abbreviation:  Congenit Heart Dis     Publication Date:    2007 May-Jun
Date Detail:
Created Date:  2008-04-01     Completed Date:  2008-06-19     Revised Date:  2011-05-05    
Medline Journal Info:
Nlm Unique ID:  101256510     Medline TA:  Congenit Heart Dis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  203-7     Citation Subset:  IM    
Affiliation:
Division of Pediatric Cardiology, University of Kentucky, Lexington, KY 40536, USA. vschroeder1@hotmail.com
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MeSH Terms
Descriptor/Qualifier:
Aneurysm, False / surgery
Arteriovenous Malformations / complications*,  diagnosis,  pathology,  surgery
Chest Pain / etiology
Child
Coronary Vessel Anomalies / complications*,  diagnosis,  pathology,  surgery
Diastole
Echocardiography
Exercise Tolerance*
Heart Aneurysm / surgery
Heart Murmurs / etiology*,  physiopathology
Heart Ventricles
Humans
Male
Postoperative Complications
Reoperation
Skin / blood supply*
Tomography, X-Ray Computed
Vascular Malformations / genetics*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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