Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading.

BACKGROUND: Moderate to severe Ebstein's malformation remains a surgical challenge. Although the various approaches that have been used are appropriate and successful in many patients, there are many for which these approaches are suboptimal. To improve the prognosis for patients across the full spectrum of Ebstein's malformation, alternative surgical approaches are necessary.

METHODS: From December 1995 to October 1997, 10 patients (median age, 9 years) with moderate or severe Ebstein's malformation and mild to severe tricuspid regurgitation had partial biventricular repair with reduction of right ventricular volume load. All patients were symptomatic in New York Heart Association functional class II (n = 9) or III (n = 1). In addition to bidirectional cavopulmonary anastomosis and closure of intracardiac defects in all patients, 6 underwent tricuspid valve repair using a variety of procedures, most often simple horizontal annuloplasty.

RESULTS: There were no deaths. Early reoperation was required in 1 patient (atrial septostomy on the day after operation for right ventricular failure) and another required revision of the tricuspid valve repair 10 months postoperatively for recurrent regurgitation. At follow-up ranging from 2 to 24 months, all patients are in New York Heart Association class I and have trivial tricuspid regurgitation, including the 4 who had no tricuspid valvuloplasty performed.

CONCLUSIONS: We have presented an alternative approach to the management of severe Ebstein's malformation that focuses on both the tricuspid valve and the right ventricle. Just as tricuspid valve repair and reduction of regurgitation will likely improve right ventricular performance, reducing the volume load on the ventricle may improve both ventricular (right and left) and tricuspid valve function. All patients have demonstrated improved exercise tolerance and right heart function at follow-up ranging to 24 months. Additional experience will be necessary to evaluate this strategy more completely.