Document Detail


Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle.
MedLine Citation:
PMID:  20152252     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Susan F Saleeb; Amy Juraszek; Tal Geva
Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  The American journal of cardiology     Volume:  105     ISSN:  1879-1913     ISO Abbreviation:  Am. J. Cardiol.     Publication Date:  2010 Feb 
Date Detail:
Created Date:  2010-02-15     Completed Date:  2010-03-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0207277     Medline TA:  Am J Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  542-9     Citation Subset:  AIM; IM    
Copyright Information:
Copyright 2010 Elsevier Inc. All rights reserved.
Affiliation:
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, USA.
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MeSH Terms
Descriptor/Qualifier:
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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