Document Detail

Aortic coarctation associated with alveolar capillary dysplasia and misalignment of the pulmonary veins.
MedLine Citation:
PMID:  17874220     Owner:  NLM     Status:  MEDLINE    
After surgical repair of an aortic coarctation a term infant presented with severe pulmonary hypertension and cyanosis unresponsive to treatment including extracorporeal membrane oxygenation. The atypical clinical course became apparent once the accompanying diagnosis of congenital alveolar capillary dysplasia with misalignment of the pulmonary veins had been established at autopsy. In infants with congenital heart defects and with refractory pulmonary hypertension unexplainable on anatomic findings, a lung biopsy at the time of cardiac repair should be considered to avoid further therapies that would not alter the uniformly fatal course of this rare lung disorder.
Andreas Gamillscheg; Gerfried Zobel; Ekkehard Spuller; Friedrich Reiterer; Albrecht Beitzke
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2007-09-15
Journal Detail:
Title:  Pediatric cardiology     Volume:  29     ISSN:  0172-0643     ISO Abbreviation:  Pediatr Cardiol     Publication Date:  2008 Jan 
Date Detail:
Created Date:  2008-01-18     Completed Date:  2008-05-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8003849     Medline TA:  Pediatr Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  191-4     Citation Subset:  IM    
Department of Pediatrics, Division of Pediatric Cardiology, Medical University of Graz, Auenbruggerplatz 30, Graz 8036, Austria.
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MeSH Terms
Aortic Coarctation / epidemiology*,  surgery
Dilatation, Pathologic
Extracorporeal Membrane Oxygenation
Fatal Outcome
Infant, Newborn
Lymphatic Vessels / pathology
Persistent Fetal Circulation Syndrome / etiology*,  therapy
Pulmonary Alveoli / pathology*
Pulmonary Veins / abnormalities*

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