Document Detail

Regression of pulmonary arteriovenous malformations following heart transplantation.
MedLine Citation:
PMID:  11079267     Owner:  NLM     Status:  MEDLINE    
Pulmonary arteriovenous malformations (PAVMs) can occur following caval to pulmonary artery connection, Glenn and/or Fontan procedure, leading to severe cyanosis and exercise intolerance. It is unknown whether these abnormalities regress or persist following heart transplantation (HTx). Twenty patients with failed Fontan or Glenn procedures were screened for PAVMs prior to HTx by contrast echocardiography, selective pulmonary angiography, and pulmonary venous desaturation. Age at transplant, diagnosis, previous operations, time from Glenn to transplant, systemic oxygenation, hemoglobin level, and ventricular function were determined. The clinical course after HTx was characterized in three patients with significant PAVMs. Indications for HTx were exercise intolerance and severe cyanosis in one patient, and cyanosis and ventricular dysfunction in two. Pre-HTx, mean systemic saturation was 67%; mean pulmonary venous wedge saturation was 81%. Post-HTx, oxygen saturations were normal (> 96%) at 14, 40, and 180 days. Contrast echocardiography, performed 1 month to 3.3 yrs after HTx, showed no intrapulmonary shunting in two patients and minimal shunting in one. One patient suffered an embolic stroke from right-to-left shunting post-HTx. All patients are alive and well 35, 71, and 73 months post-HTx. In patients with single ventricle physiology, PAVMs are not an absolute contraindication to HTx. Heart-lung transplant may not be required for these patients.
J M Lamour; D T Hsu; M R Kichuk; M E Galantowicz; J M Quaegebeur; L J Addonizio
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric transplantation     Volume:  4     ISSN:  1397-3142     ISO Abbreviation:  Pediatr Transplant     Publication Date:  2000 Nov 
Date Detail:
Created Date:  2001-03-06     Completed Date:  2001-05-31     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9802574     Medline TA:  Pediatr Transplant     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  280-4     Citation Subset:  IM    
Department of Pediatrics, College of Physicians and Surgeons, Columbia University, New York, New York, USA.
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MeSH Terms
Arteriovenous Malformations / complications*
Exercise Tolerance
Fontan Procedure
Heart Defects, Congenital / surgery
Heart Transplantation* / methods
Oxygen / blood
Pulmonary Circulation*
Treatment Outcome
Reg. No./Substance:

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