Document Detail


Alveolar capillary dysplasia: diagnostic potential for cardiac catheterization.
MedLine Citation:
PMID:  10685275     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: Alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Infants with this condition die despite maximal medical intervention including inhaled nitric oxide therapy and extracorporeal membrane oxygenation. To date, diagnosis of this lethal condition was made by open lung biopsy or during postmortem examination. We examined the possibility that distinct cardiac catheterization findings could be used in the diagnosis of this lethal disorder. STUDY DESIGN: We present three infants with fatal persistent pulmonary hypertension of the newborn refractory to extracorporeal membrane oxygenation and inhaled nitric oxide therapy, two with postmortem autopsy confirmation of alveolar capillary dysplasia. Each infant underwent cardiac catheterization to complete the diagnostic evaluations. RESULTS: Significant right ventricular hypertension and normal pulmonary venous return were demonstrated, but a markedly diminished or absent capillary blush phase was noted in each infant. This finding is distinct from the normal capillary blush seen in infants with persistent pulmonary hypertension of the newborn of other etiologies. CONCLUSION: Cardiac catheterization may provide a useful alternative to tissue examination in the diagnosis of alveolar capillary dysplasia.
Authors:
S R Hintz; J A Vincent; P T Pitlick; J R Fineman; R H Steinhorn; G E Kim; W E Benitz
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of perinatology : official journal of the California Perinatal Association     Volume:  19     ISSN:  0743-8346     ISO Abbreviation:  J Perinatol     Publication Date:  1999 Sep 
Date Detail:
Created Date:  2000-03-08     Completed Date:  2000-03-08     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8501884     Medline TA:  J Perinatol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  441-6     Citation Subset:  IM    
Affiliation:
Division of Neonatal and Developmental Medicine, Stanford University School of Medicine, Palo Alto, CA, USA.
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MeSH Terms
Descriptor/Qualifier:
Angiography
Capillaries / abnormalities
Extracorporeal Membrane Oxygenation
Fatal Outcome
Female
Heart Catheterization*
Humans
Infant, Newborn
Lung / pathology
Male
Nitric Oxide / therapeutic use
Persistent Fetal Circulation Syndrome / diagnosis,  etiology,  physiopathology,  therapy
Pulmonary Alveoli / blood supply*
Pulmonary Artery / radiography
Pulmonary Veins / physiopathology
Ventricular Function, Right
Chemical
Reg. No./Substance:
10102-43-9/Nitric Oxide

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