Early And Mid-term Outcomes Of Ebstein’s Anomaly Patients With Correction Surgery

Objective:The incidence of Ebstein’s anomaly is extremely low,and except for the Mayo Clinic,no cardiac center has reported on a sufficient number of patients.The aim of our study was to report the early and mid-term outcomes of Ebstein’s anomaly patients with correction surgery.Methods:Form July 2006 to April 2016,Ebstein’s anomaly correction surgery was performed in 346 patients.And 322 patients underwent follow-up(93.1%).We reviewed patients’ records and contacted patients via outpatient service and over the telephone.Results:The mean follow-up time was 44.8±33.4 months.The mean operative age was 23.4± 18.1 years(range from 0.5 year to 66 years),with 183(56.8%)patients being female.Tricuspid valvuloplasty(TVP)was performed in 292(90.7%)patients,tricuspid valve replacement(TVR)was performed in 26(8.1%)patients,and isolated Glenn procedure was performed in 4(1.2%)patients.The early mortality rate was 1.9%,only one patients died of cardiac arrest during the follow-up period and the overall survival rate was 97.6%at 5 and 10 years.For TVP patients,the rate of residual and recurrent tricuspid regurgitation(TR)in patients with prosthetic ring was lower than that in patients without prosthetic ring(1.6%vs.4.9%,12.7%vs.21.9%,respectively),however,there was no significant difference between two groups.The rate of residual and recurrent TR in patients with autologous pericardium had no significant difference with that in patients without autologous pericardium(1.4%vs.5.0%,20.3%vs.19.7%,respectively).After propensity score matching,the rates of mortality and New York Heart Association(NYHA)class>Ⅲ were lower in TVP patients than those in TVR patients without statistical significance.Carpentier(C+D)rate in patients with Glenn procedure was significantly higher than that in patients without Glenn procedure(85.7%vs.11.5%,P<0.001),however,the early mortality and overall mortality in patients with Glenn procedure had no significant difference with those in patients without Glenn procedure(2.9%vs.1.7%,2.9%vs.2.1%,respectively).Nine patients with type B Wolff-Parkinson-White syndrome underwent one-stage surgery,and arrhythmias disappeared.Seven patients suffered from episodes of left ventricular outflow tract obstruction(LVOTO)during surgery.The rate of LVOTO in patients with modified Danielson procedure were significantly higher than that in patients with modified Carpentier patients(2.0%vs.10.3%,P=0.038).Conservative treatment was unsuccessful in 3 patients with LVOTO,and 2 patients were treated with reoperation of the atrialized right ventricle.Conclusion:1)For Ebstein’s anomaly patients,optimal early and mid-term outcomes could be obtained through Ebstein’s anomaly correction surgery,and the TVP should be the first choice.2)For patients with TVP,prosthetic ring implantation had no significant effect on the rate of residual and recurrent TR;and Ebstein’s anomaly patients with severely dysplastic leaflets could have similar resut with other patients by using autologous pericardium.3)For patients with TVR,similar result could be obtained as TVP.4)Severe Ebstein’s anomaly patients could obtain similar outcome with mild Ebstein’s anomaly patients by performing bidirectional Glenn procedure.5)Optimal outcomes can be obtained in patents with type B WPW syndrome who undergo a one-stage operation.6)The occurrence of severe LVOTO during correction surgery for Ebstein’s anomaly is rarely low,the probable cause may be improper operation of the atrialized right ventricle,and good result could be obtained with reoperation of the atrialized right ventricle.