Document Detail

Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome.
MedLine Citation:
PMID:  17059929     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Primary repair of tetralogy of Fallot with absent pulmonary valve syndrome has been associated with significant mortality, particularly for neonates in respiratory distress. Controversy persists regarding the method of establishing right ventricle-pulmonary artery continuity. METHODS: Anatomic and demographic parameters were evaluated for patients undergoing repair of tetralogy of Fallot with absent pulmonary valve syndrome from 1990 to 2005, as were perioperative and late postoperative parameters (airway complications, reoperation or catheter-based intervention, and mortality). RESULTS: Twenty-three patients underwent repair. Median age was 15 days (range 2-1154 days). Patients were followed up for 5.3 +/- 3.9 years. Seventeen (85%) required preoperative ventilatory assistance. One patient died within 24 hours; 1 patient died 8 months postoperatively. Four patients received valved homografts, and the remainder had valveless connections. All patients underwent reduction pulmonary arterioplasty and mobilization, unifocalization (in 3), and ventricular septal defect closure. Valveless connection recipients had a transannular hood. No patient underwent a Lecompte maneuver. Four patients underwent reoperation for conversion to valveless connection (n = 1), reduction arterioplasty (n = 1), and repair of pulmonary stenosis (n = 2). Three patients required catheter-based intervention, with balloon angioplasty (n = 3) and stent placement (n = 1); 2 now demonstrate equal quantitative lung perfusion. No patient has had significant debility from airway compromise. All patients demonstrate free pulmonary insufficiency and good biventricular function. CONCLUSIONS: We report excellent overall survival (89%) and low postoperative morbidity for neonates and infants undergoing primary repair of tetralogy of Fallot with absent pulmonary valve syndrome. Our recent experience supports the use of a valveless right ventricle-pulmonary artery connection, which, combined with catheter-based intervention, reduces the likelihood of reoperation necessitated by homograft placement.
Jonathan M Chen; Julie S Glickstein; Renee Margossian; Michelle L Mercando; William E Hellenbrand; Ralph S Mosca; Jan M Quaegebeur
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of thoracic and cardiovascular surgery     Volume:  132     ISSN:  1097-685X     ISO Abbreviation:  J. Thorac. Cardiovasc. Surg.     Publication Date:  2006 Nov 
Date Detail:
Created Date:  2006-10-24     Completed Date:  2006-11-27     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376343     Medline TA:  J Thorac Cardiovasc Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1099-104     Citation Subset:  AIM; IM    
Department of Cardiothoracic Surgery, Weill Medical School at Cornell University, New York, NY 10021, USA.
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MeSH Terms
Child, Preschool
Infant, Newborn
Pulmonary Valve / abnormalities*,  surgery*
Recovery of Function
Survival Analysis
Tetralogy of Fallot / surgery*
Treatment Outcome

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