Surgical Outcome and Time of Operation in Ebstein's Anomaly

Jin, Seon Mi; Choi, Eun Young; Kwon, Bo Sang; Lee, Chang Hoon; Ban, Ji Eun; Son, Jae Sung; Lee, Jung Ha; Bae, Eun Jung; Yun, Yong Soo; Noh, Chung Il

Abstract

PURPOSE
Ebstein's anomaly is characterized by ongoing tricuspid regurgitation and right ventricular dysfunction. It is difficult to decide the proper time of surgical correction. The purpose of this study is to evaluate the advantage of early surgical correction of Ebstein's anomaly.
METHODS
The clinical records of the sixteen patients, who had undergone tricuspid valvuloplasty with plication of the atrialized RV and followed-up for more than five years, were reviewed. Surgical outcome was analysed as regards year of surgery, age of the patients, echocardiographic grade of severity, Carpentier type, associated cardiac defects, and type of surgery.
RESULTS
Of sixteen patients, eight patients underwent tricuspid valve repair before 5 years(8 months to 5 years, mean 2.4 years) of age(group A), and eight patients underwent tricuspid valve repair after 5 years(6 to 23 years, mean 12.9 years) of age(group B). Immediately after the operation, tricuspid incompetence regressed significantly in 5 of group A, and 5 of group B, and reduction in heart size was usual. After short and mid- term follow-up period, four of group A showed mild tricuspid valve regurgitation and right ventricular dilatation and relatively well preserved right ventricular function. However, all of group B showed severe right ventricular dilatation with severe tricuspid regurgitation and both ventricular dysfunction. All of group A were in New York Heart Association class I. However, in group B, only three were in class I. Two of group B died of right heart failure 10 and 16 years after the surgical correction, respectively. The year of surgery, mean follow-up periods, associated cardiac defects, and the preoperative echocardiographic grade of the severity were not different significantly between two groups. But there was a difference between two groups in the mean age of the patients, 12.7+/-5.2 years in group A, 21.7+/-5.5 years in group B. The surgical outcome was not closely related to Carpentier types of Ebstein's anomaly.
CONCLUSION
Early repair of tricuspid valve may be advantageous to prevent progressive dilatation of the right side heart and to preserve right ventricular function after surgical correction. But the aging factor was not corrected in our study and it is still necessary to follow-up for a longer period in larger patients group.