Document Detail

Tricuspid atresia: association with persistent truncus arteriosus.
MedLine Citation:
PMID:  1877458     Owner:  NLM     Status:  MEDLINE    
Clinical, echo-Doppler, and pathologic data of a rare case of tricuspid atresia in association with truncus arteriosus are presented. There are only six patients (including the patient reported here) with this anomaly in whom detailed pathologic and/or clinical descriptions are available in the literature. An additional six cases were mentioned in the literature but without any details. A prevalence rate of 1.4% among tricuspid atresia cases is estimated. All infants presented with symptoms of cyanosis and/or congestive heart failure within a few days to 2 months after birth and died shortly thereafter. Cardiomegaly and increased pulmonary vascular markings on the chest x-ray film and left axis deviation with left ventricular hypertrophy on the ECG are usually present. Echocardiographic, catheterization, and angiocardiographic studies are helpful in documenting anatomic and physiologic features of this anomaly. Pathologically, the muscular type of tricuspid atresia, type I or II truncus arteriosus, a large subtruncal VSD, and a hypoplastic right ventricle are present. It is concluded that coexistence of tricuspid atresia and truncus arteriosus is rare; clinical, laboratory, and pathologic features are distinctive; the clinical presentation early in life with rapid demise is common; and prompt palliative surgery immediately after recognition of this anomaly should be undertaken.
P S Rao; J M Levy; E Nikicicz; E F Gilbert-Barness
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  American heart journal     Volume:  122     ISSN:  0002-8703     ISO Abbreviation:  Am. Heart J.     Publication Date:  1991 Sep 
Date Detail:
Created Date:  1991-09-26     Completed Date:  1991-09-26     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0370465     Medline TA:  Am Heart J     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  829-35     Citation Subset:  AIM; IM    
Department of Pediatrics, University of Wisconsin Medical School, Madison.
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MeSH Terms
Infant, Newborn
Myocardium / pathology
Tricuspid Valve / abnormalities*
Truncus Arteriosus, Persistent / epidemiology*,  pathology,  ultrasonography

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