Document Detail

Progressive pulmonary venous obstruction and pulmonary interstitial fibrosis associated with tetralogy of Fallot.
MedLine Citation:
PMID:  7997423     Owner:  NLM     Status:  MEDLINE    
Stenosis of individual pulmonary veins is a rare condition associated with a very high mortality. The present report is the first known case of progressive pulmonary venous obstruction associated with the tetralogy of Fallot. This clinical and anatomical study suggests that initial dynamic and possibly reversible occlusion occurs at the junction of the pulmonary vein and left atrium. Anatomical lesions in pulmonary veins then develop, possibly secondary to functional stenosis. The usually fatal outcome of these cases is due to the development of fixed pulmonary venous occlusion and possibly pulmonary interstitial fibrosis. The rapid progression of pulmonary venous stenosis strongly suggests that discrete veno-atrial stenosis should be surgically corrected as early as possible to prevent the irreversible diffuse pulmonary venous obstruction associated with an invariably fatal prognosis.
M E McConnell; A D Pacifico; W H Johnson; E Mroczek
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric cardiology     Volume:  15     ISSN:  0172-0643     ISO Abbreviation:  Pediatr Cardiol     Publication Date:    1994 Mar-Apr
Date Detail:
Created Date:  1995-01-13     Completed Date:  1995-01-13     Revised Date:  2008-02-20    
Medline Journal Info:
Nlm Unique ID:  8003849     Medline TA:  Pediatr Cardiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  95-9     Citation Subset:  IM    
Department of Pediatrics, Pathology, and Surgery, University of Alabama in Birmingham.
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MeSH Terms
Ductus Arteriosus, Patent / complications*,  surgery,  ultrasonography
Fatal Outcome
Infant, Low Birth Weight
Infant, Newborn
Infant, Premature
Infant, Premature, Diseases / physiopathology*,  surgery,  ultrasonography
Pulmonary Fibrosis / etiology*,  pathology
Pulmonary Veno-Occlusive Disease / etiology*,  pathology,  radiography,  surgery
Tetralogy of Fallot / complications*,  surgery,  ultrasonography

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

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