Selective right ventricular unloading and novel technical concepts in Ebstein's anomaly.
BACKGROUND
Favorable outcomes in Ebstein's anomaly are predicated on tricuspid valve competence and right ventricular function. Successful valve repair should be aggressively pursued to avoid the morbidity of prosthetic tricuspid valve replacement. We report our experience with valve-sparing intracardiac repair, emphasizing novel concepts and techniques of valve repair supplemented by selective bidirectional Glenn (BDG).


METHODS
Between June 1993 and December 2008, 57 nonneonatal patients underwent Ebstein's anomaly repairs. The median age at operation was 8.1 years. All were symptomatic in New York Heart Association (NYHA) functional class II (n = 38), III (n = 17), or IV (n = 1). Preoperatively, 26 had mild or moderate cyanosis at rest. We used a number of valve reconstructive techniques that differed substantially from those currently described. BDG was performed in 31 patients (55%) who met specific criteria.


RESULTS
No early or late deaths occurred. At the initial repair, 3 patients received a prosthetic valve. Four patients required reoperation for severe tricuspid regurgitation. Repeat repairs were successful in 2 patients. At follow-up (range, 3 months to 6 years), all patients were acyanotic and in NYHA class I. Tricuspid regurgitation was mild or less in 49 (86%) and moderate in 6 (11%). Freedom from a prosthesis was 91% (52 of 57).


CONCLUSIONS
Following a protocol using BDG for ventricular unloading in selected patients with Ebstein's anomaly can achieve a durable valve-sparing repair using the techniques described. Excellent functional midterm outcomes can be obtained with a selective one and a half ventricle approach to Ebstein's anomaly.
Malhotra
Sunil P
SP
Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Stanford, California, USA. spmalhotra@peds.ufl.edu
Petrossian
Ed
E
Reddy
V Mohan
VM
Qiu
Mary
M
Maeda
Katsushide
K
Suleman
Sam
S
MacDonald
Malcolm
M
Reinhartz
Olaf
O
Hanley
Frank L
FL
D000293:Adolescent; D000328:Adult; D006348:Cardiac Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome; D016278:Ventricular Function, Right / Q000502:physiology*; D055815:Young Adult