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Treatment strategies of pediatric Ebstein malformation

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Author:
No author available
Journal Title:
Chinese Journal of Pediatric Surgery
Issue:
8
DOI:
10.3760/cma.j.issn.0253-3006.2016.08.008
Key Word:
爱泼斯坦异常;心脏缺损,先天性;心脏外科手术;Ebstein'anomaly;Heart defects,congenital;Cardiac surgical procedures

Abstract: Objective To evaluate the outcomes of cone technique in pediatric Ebstein malformation.Methods A total of 115 patients with Ebstein malformation between January 2006 and December 2014 were recruited and analyzed.Their mean age was (4.9-± 4.3) (0.75-16.1) years and mean weight (18.4 ± 12.2) (5.5-59.2) kg.Among 104 cases of cone technique,39 cases underwent additional bidirectional cavo-pulmonary shunt (BCPS) and 9 cases uni-ventricle procedures.And two cases had valvular transplantation.Results There were 2 hospital deaths (1.7%).For 113 cases,the mean follow-up period was 32.7 (4-88) months.Postoperative color echocardiograph showed mild tricuspid regurgitation (TR) (n =31) and moderate TR (n =8) in 39 cases with BCPS;mild TR (n =35),moderate TR (n =21) and severe TR (n =8) 64 cases without BCPS.Conclusions Cone technique may be the most important treatment for Ebstein malformation.The outcomes are satisfactory.The cone technique with BCPS or one-and-half ventricle repair is more efficacious.The treatment strategies are determined by age,structure of valvular regurgitation and right ventricle function.

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