Document Detail

Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect.
MedLine Citation:
PMID:  16798205     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Double-outlet right ventricle encompasses a broad spectrum of anomalies. Heterotaxy syndrome, which is often associated with total anomalous pulmonary venous connection and complete atrioventricular canal defect, has been considered a risk factor for surgical repair of double-outlet right ventricle. METHODS: From January 1992 to May 1999, medical records of 96 patients (50 males, 46 females) who had complex double-outlet right ventricle with heterotaxy and/or complete atrioventricular canal defect were reviewed (median age at initial surgery 3 months). Seventeen patients were neonates requiring surgery. Follow-up ranged from 1 day to 7.4 years (median, 16 months). RESULTS: Sixty-eight patients had heterotaxy syndrome (27 with total anomalous pulmonary venous connection). Eighty-three had complete atrioventricular canal defect, 22 with moderate to severe atrioventricular valve regurgitation at the time of surgical repair. Eight patients had two-ventricle repair, and 88 patients were considered for single-ventricle management (bidirectional Glenn, 37; Fontan, 44). One patient had heart transplantation after bidirectional Glenn. There were 16 deaths including 10 early (<30 days postoperatively). Overall survival (95% confidence interval) estimated by the Kaplan-Meier method was 89% (83% to 96%) at 1 month, 84% (76% to 91%) at 1 year, and 81% (73% to 89%) at 5 years. Multivariate analysis revealed that neonatal presentation requiring surgery (p < 0.0001), moderate to severe atrioventricular valve regurgitation (p = 0.03), and pulmonary venous obstruction (p = 0.02) were risk factors for death. CONCLUSIONS: Atrioventricular valve regurgitation, pulmonary venous obstruction, and neonatal presentation are risk factors for mortality in patients with complex double-outlet right ventricle. Early surgical intervention in symptomatic neonates and infants, including those with pulmonary venous obstruction, may reduce mortality and improve outcome after staged operation.
Koh Takeuchi; Francis X McGowan; Emile A Bacha; John E Mayer; David Zurakowski; Masaki Otaki; Pedro J del Nido
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Publication Detail:
Type:  Evaluation Studies; Journal Article    
Journal Detail:
Title:  The Annals of thoracic surgery     Volume:  82     ISSN:  1552-6259     ISO Abbreviation:  Ann. Thorac. Surg.     Publication Date:  2006 Jul 
Date Detail:
Created Date:  2006-06-26     Completed Date:  2006-08-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  15030100R     Medline TA:  Ann Thorac Surg     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  146-52     Citation Subset:  AIM; IM    
Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
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MeSH Terms
Abnormalities, Multiple / surgery
Child, Preschool
Double Outlet Right Ventricle / surgery*
Follow-Up Studies
Heart Defects, Congenital / surgery
Heart Septal Defects / surgery
Heart Transplantation
Infant, Newborn
Life Tables
Mitral Valve Insufficiency / complications
Postoperative Complications / mortality
Pulmonary Veins / pathology
Retrospective Studies
Risk Factors
Survival Analysis
Treatment Outcome
Tricuspid Valve Insufficiency / complications

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

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