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

Background Moderate to severe Ebstein's malformation remains a surgical cha llenge. 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 spec trum of Ebstein's malformation, alternative surgical approaches are necessa ry. Methods. From December 1995 to October 1997, 10 patients (median age, 9 yea rs) with moderate or severe Ebstein's malformation and mild to severe tricu spid regurgitation had partial biventricular repair with reduction of right ventricular volume load. All patients were symptomatic in New York Heart A ssociation functional class II (n = 9) or III (n = 1). In addition to bidir ectional cavopulmonary anastomosis and closure of intracardiac defects in a ll patients, 6 underwent tricuspid valve repair using a variety of procedur es, 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 pos toperatively for recurrent regurgitation. At follow-up ranging from 2 to 24 months, all patients are in New York Heart Association class I and have tr ivial tricuspid regurgitation, including the 4 who had no tricuspid valvulo plasty 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 regur gitation will likely improve right ventricular performance, reducing the vo lume load on the ventricle may improve both ventricular (right and left) an d tricuspid valve function. All patients have demonstrated improved exercis e tolerance and right heart function at follow-up ranging to 24 months. Add itional experience will be necessary to evaluate this strategy more complet ely. (C) 1998 by The Society of Thoracic Surgeons.