| Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle. | |
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MedLine Citation:
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PMID: 20152252 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The purposes of this study were to characterize the morphologic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle (DI-DORV), a rare congenital heart disease, and to compare these findings to those in patients with double-inlet left ventricle (DILV). The clinical records, imaging studies, and heart specimens of 22 consecutive patients with DI-DORV were reviewed. A comparison group of 44 patients with DILV was matched by age at latest follow-up. Among the 22 patients with DI-DORV, 14 had clinical data, 5 had clinical and autopsy data, and 3 had only autopsy data. Abdominal and atrial situs were normal in all, and heart position was levocardia in 91%. The morphology of the atrioventricular valves was variable, with 15 patients showing neither a typical tricuspid nor mitral valve. In the remaining 7 patients, 1 or both atrioventricular valves resembled a tricuspid or a mitral valve. Myocardial architecture was characterized by atypical muscle bundles of varying degrees of hypertrophy and orientation. Most patients had bilateral conus, and 82% had either aortic or pulmonary outflow tract obstructions. The rate of overall mortality or heart transplantation was 36%. Significant differences between DI-DORV and DILV included the type of ventricular loop, the type of conus, and a higher mortality rate in patients with DI-DORV. In conclusion, DI-DORV is a distinct type of functional single ventricle congenital heart disease with variable atrioventricular valve morphology and myocardial architecture. Accurate diagnosis using noninvasive imaging techniques is of paramount importance for optimal management. |
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Authors:
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Susan F Saleeb; Amy Juraszek; Tal Geva |
Publication Detail:
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Type: Comparative Study; Journal Article |
Journal Detail:
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Title: The American journal of cardiology Volume: 105 ISSN: 1879-1913 ISO Abbreviation: Am. J. Cardiol. Publication Date: 2010 Feb |
Date Detail:
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Created Date: 2010-02-15 Completed Date: 2010-03-19 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0207277 Medline TA: Am J Cardiol Country: United States |
Other Details:
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Languages: eng Pagination: 542-9 Citation Subset: AIM; IM |
Copyright Information:
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Copyright 2010 Elsevier Inc. All rights reserved. |
Affiliation:
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Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Autopsy Boston / epidemiology Cardiac Surgical Procedures / methods Coronary Angiography Double Outlet Right Ventricle / diagnosis*, mortality, surgery Female Follow-Up Studies Heart Transplantation / mortality Heart Ventricles / abnormalities*, surgery Humans Infant Infant, Newborn Magnetic Resonance Imaging Male Mitral Valve / abnormalities*, radiography, surgery, ultrasonography Retrospective Studies Survival Rate Treatment Failure Treatment Outcome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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