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Pulmonary arteriovenous malformations in children after the Kawashima operation.
MedLine Citation:
PMID:  16242422     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Pulmonary arteriovenous malformations are a major cause of progressive late cyanosis in patients treated with cavopulmonary anastomoses. Previous experience suggests that exclusion of the hepatic venous effluent from the pulmonary circulation may cause the development of pulmonary arteriovenous malformations after the Kawashima operation in children with interrupted inferior vena cava with azygous continuation. METHODS: From January 1990 to November 2004, 21 children (median age, 2 years) with heterotaxy syndrome and interrupted inferior vena cava with azygous continuation underwent Kawashima operation. The average preoperative arterial oxygen saturation was 76% +/- 7% (range, 64% to 90%). RESULTS: Follow-up was complete in all survivors except 1 at a median duration of 4.5 years. Pulmonary arteriovenous malformations were diagnosed at a median of 5 years after Kawashima operation in 11 patients (58%). Completion Fontan operation has been performed in 15 (79%). Five children who underwent a completion Fontan procedure 1 to 1.5 years after Kawashima operation did not have pulmonary arteriovenous malformations. There were 2 late deaths due to chronic congestive heart failure 4 months and 7 years after Kawashima operation. Overall survival at 10 years is 90%. Univariate and multivariate analysis demonstrated presence of bilateral superior vena cavae (p = 0.002) and interval longer than 2 years between Kawashima operation and completion Fontan operation (p = 0.04) as predictors of developing pulmonary arteriovenous malformations. CONCLUSIONS: In most patients with heterotaxy and interrupted inferior vena cava with azygous continuation, clinical evidence of pulmonary arteriovenous malformations will develop after Kawashima operation if they are followed up long enough. Early redirection of the hepatic venous effluent to the pulmonary arterial circulation may prevent or lead to regression of pulmonary arteriovenous malformations, with low mortality and morbidity.
Authors:
John W Brown; Mark Ruzmetov; Palaniswamy Vijay; Mark D Rodefeld; Mark W Turrentine
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Annals of thoracic surgery     Volume:  80     ISSN:  1552-6259     ISO Abbreviation:  Ann. Thorac. Surg.     Publication Date:  2005 Nov 
Date Detail:
Created Date:  2005-10-24     Completed Date:  2006-11-02     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  15030100R     Medline TA:  Ann Thorac Surg     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  1592-6     Citation Subset:  AIM; IM    
Affiliation:
Indiana University School of Medicine, James Whitcomb Riley Hospital for Children, Indianapolis, Indiana, USA. jobrown@iupui.edu
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MeSH Terms
Descriptor/Qualifier:
Arteriovenous Malformations / diagnosis,  etiology*
Child
Child, Preschool
Heart Bypass, Right / adverse effects*
Humans
Infant
Postoperative Complications
Pulmonary Artery / abnormalities*
Vena Cava, Inferior / abnormalities*

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


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