The long-term impact of various techniques for tricuspid repair in Ebstein's anomaly.
OBJECTIVE
We describe a repertoire of repair techniques according to type of Ebstein's anomaly to correct tricuspid valve (TV) incompetence, and report long-term ventricular function and functional outcomes.


METHODS
Sixty-eight patients (mean age, 26.9 ± 7.3 years) with Ebstein's anomaly (type A, n = 21; type B, n = 23; type C, n = 15; type D, n = 9) underwent correction of TV incompetence under normothermic cardiopulmonary bypass. The atrialized ventricle, TV, and subvalvar apparatus were inspected to analyze the precise morphology and determine which leaflet was the most mobile. Various repair strategies (anterior and/or posterior annulorrhapy, Sebening stitich, double-orifice valve technique, with bidirectional Glenn anastomosis if necessary) were used according to the presenting morphology and applied according to the type of Ebstein's anomaly. In all, the atrialized right ventricle (RV) was incorporated into the contractile RV by partial closure of the natural annulus using the most mobile leaflet for valve competence.


RESULTS
The mean duration of follow-up was 13.25 ± 1.3 years (median, 9.34 years; range, 1-24 years). The mean New York Heart Association class improved from 3.4 to 1.3 (P < .001). The mean severity of TV incompetence was reduced from 3.2 to 1.3 (P < .001). Exercise tolerance tests demonstrated improved maximal oxygen uptake from a mean of 15 ± 7.8 ng/kg/min preoperatively to a mean of 24.9 ± 2.0 ng/kg/min postoperatively (P < .02). Displacement tissue Doppler imaging was used to evaluate overall cardiac performance of the RV and left ventricle and interventricular septum. The mean basal, middle, and apical ventricular strain improved significantly from preoperative values of 18.08%, 15.6%, and 13.9%, respectively to postoperative values of 25.7% (P < .011), 23.7% (P < .001), and 19.36% (P < .05), respectively. Freedom from reoperation was 100% at 1 year, 98.3% at 5 years, and 92.9% at 20 years. Early mortality was 2.9%, and late mortality was 5.8%. The overall survival rate was 97.6% at 30 days, 92.7% at 5 years, and 91.26% at 20 years.


CONCLUSIONS
The various repair techniques, all of which preserve the atrialized chamber and are used individually according to morphology, provide satisfactory long-term ventricular function and functional outcome even in severe types of Ebstein's anomaly.
Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany.
Roland
R
Hetzer
Department of Congenital Heart Disease/Pediatric Cardiology, Deutsches Herzzentrum Berlin, Berlin, Germany.
Paul
P
Hacke
Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany.
Mariano
M
Javier
Department of Congenital Heart Disease/Pediatric Cardiology, Deutsches Herzzentrum Berlin, Berlin, Germany.
Oliver
O
Miera
Department of Congenital Heart Disease/Pediatric Cardiology, Deutsches Herzzentrum Berlin, Berlin, Germany.
Katharina
K
Schmitt
Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany.
Yuguo
Y
Weng
Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany. Electronic address: eva.delmowalter@gmail.com.
Eva Maria
EM
Delmo Walter
D000293:Adolescent; D000328:Adult; D000368:Aged; D000714:Anastomosis, Surgical; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D058385:Cardiac Valve Annuloplasty; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004437:Ebstein Anomaly / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D020127:Recovery of Function; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013536:Suture Techniques; D013997:Time Factors; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D014262:Tricuspid Valve Insufficiency / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right; D055815:Young Adult
Ebstein's anomaly; atrialized chamber; tricuspid valve; ventricular function