Surgery of the Ebstein's anomaly: early and late outcomes

Ebstein's anomaly of the tricuspid valve is a complex malformation. Several operations have been undertaken with varying results. The severity of the morphology of the lesion and ventricular function determine the spectrum of surgical techniques that are employed with varying results.

Between 1980 and 2005, 45 patients with Ebstein's anomaly underwent surgical repair. Age at operation ranged from 3 to 26 years (median 15.5, mean 18.0 years). In 41 patients (91%), tricuspid valvuloplasty was done. The other four patients had valve replacement with bio-prosthesis. Eight patients required ventricular unloading by creating bidirectional Glenn procedure in addition to a valve repair. Seven needed re-operations: one repeat valve repair, two valve replacements and three takedown of Glen shunt, and one Fontan operation.

In-hospital deaths occurred in two patients (4.4%) and late mortality in four patients (9%). 95% of 39 survivors were followed for 5 months to 19.5 years (median follow-up, 5.0 years; mean follow-up, 6.9 years). The actuarial survival rate (Kaplan-Meier) was 96.5%± 2.4% at 1 year, 84.4%± 3.5% at 10 years and 83.3%± 5.6% at 19 years. At follow-up 90% were in functional class I or II with substantial improvement compared with their preoperative status. Doppler echocardiographic studies demonstrated good tricuspid valve function in most patients.

Valve repair or replacement tailored to the anatomical substrate of the anomaly yielded good long-term results with substantial improvement in functional status. Bidirectional Glenn anastomosis combined with a valve repair improved ventricular function and improved both the early and late outcomes.