Document Detail

Functional single ventricle with extracardiac total anomalous pulmonary venous connection.
MedLine Citation:
PMID:  19375345     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: The purpose of this study is to evaluate the surgical repair of functional single ventricle and extracardiac total anomalous pulmonary venous connection (TAPVC). METHODS: Between January 1998 and December 2007, 26 consecutive patients underwent surgical repair of extracardiac TAPVC. Their characteristics were as follows: median age, 34 (range 0-744) days; median weight 3.2 (range 2.0-9.6) kg; supracardiac TAPVC, 11 patients; infracardiac, 5; mixed, 10; right atrial isomerism, 24; pulmonary atresia, 16; and obstructed TAPVC, 17. Concomitant procedures included systemic-to-pulmonary shunt in 9 patients, pulmonary artery banding in 5, ventricle-to-pulmonary artery shunt in 1, Norwood procedure in 1, bidirectional Glenn in 9, and Fontan procedure in 1. RESULTS: The overall survival after the repair of TAPVC was 58% (95% confidence interval [CI], 39-77%) and 54% (95% CI, 34-73%) at 1 and 5 years, respectively. Of the 14 survivors (supracardiac, 9; infracardiac, 4; and mixed, 1), 12 underwent Fontan completion; 1, bidirectional Glenn; and 1 is awaiting bidirectional Glenn. Anastomotic stenosis did not occur, but recurrent pulmonary venous ostial stenosis (PVS) was observed in nine patients. Freedom from recurrent PVS was 56% (95% CI, 34-78%) at both 1 and 5 years. Reoperation for recurrent PVS was performed in six patients; of these patients, two underwent Fontan completion, but three with bilateral and multiple PVS declined. By Cox multivariate regression analysis, mixed TAPVC (p=0.001, hazard ratio, 13.4; 95% CI, 2.8-64.4) was a risk factor for mortality, and atrioventricular valve regurgitation, which required surgical intervention at the palliative stage (p=0.024, hazard ratio, 23.4; 95% CI, 1.5-363.4) was a risk factor for recurrent PVS. CONCLUSIONS: The mid-term results of the surgical repair of functional single ventricle with supracardiac or infracardiac TAPVC are acceptable. The surgical treatment of patients with mixed TAPVC and with severe atrioventricular valve regurgitation is not promising, but can be improved.
Tomohiro Nakata; Yoshifumi Fujimoto; Keiichi Hirose; Masaki Osaki; Yuko Tosaka; Yujiro Ide; Maiko Tachi; Kisaburo Sakamoto
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Publication Detail:
Type:  Evaluation Studies; Journal Article     Date:  2009-04-16
Journal Detail:
Title:  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery     Volume:  36     ISSN:  1873-734X     ISO Abbreviation:  Eur J Cardiothorac Surg     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-06-22     Completed Date:  2009-10-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8804069     Medline TA:  Eur J Cardiothorac Surg     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  49-56; discussion 56     Citation Subset:  IM    
Department of Cardiovascular Surgery, Shizuoka Children's Hospital, Shizuoka, Japan.
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MeSH Terms
Abnormalities, Multiple / surgery*
Child, Preschool
Fontan Procedure
Heart Ventricles / abnormalities,  surgery
Infant, Newborn
Pulmonary Veins / abnormalities*,  surgery
Pulmonary Veno-Occlusive Disease / etiology,  surgery
Reoperation / methods
Retrospective Studies
Survival Analysis

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

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