Outcomes and Risk Factors for Morbidity and Mortality in Ebstein’s Anomaly: An Evaluation Based on the German National Register for Congenital Heart Defects

Background: Ebstein’s anomaly (EA) is a rare congenital anomaly of the tricuspid valve (TV) and the right ventricle with considerable variety in disease severity and clinical presentation. The study aim was therefore to evaluate long-term outcomes and risk factors for morbidity and mortality of EA patients in the German National Register of Congenital Heart Defects (NRCHD).

Methods: All patients with EA treated conservatively or surgically with at least one clinical presentation reported to the NRCHD between March 1973 and June 2021 were eligible for retrospective analysis. The incidence of complete atrioventricular block (CAVB), heart failure, tricuspid valve regurgitation (TVR), reoperation, heart transplantation, and all-cause mortality were assessed.

Results: Of 398 patients, conservatively treated patients (n = 194/49%) had less TV regurgitation and heart failure symptoms (p < 0.001) than patients with at least one corrective surgery (n = 204, 51%). Postoperative survival at 10, 20, and 30 years was 97%, 93%, and 80%. Initial corrective surgery was complicated by CAVB in 9% (n = 17), the risk of which increased with older age (p = 0.001) and with TV replacement compared with reconstruction (p = 0.029), in addition to being an independent predictor for all-cause mortality (p < 0.001). CAVB (p = 0.008) and TV regurgitation (p < 0.001) were less frequent with cone than monocusp reconstruction. Postoperative NYHA class >I was more likely with older age (p < 0.001). Eighty-one (39.7%) patients underwent multiple TV surgeries, which occurred most frequently in those who first operated before adolescence (p = 0.025).

Conclusion: This large registry-based study of EA shows that surgical treatment results in low early and late mortality. Patients who were operated upon before adolescence were at the highest risk of reoperation, while older age at the time of the first surgery increased the risk of CAVB. Choosing an optimal time window for surgery and use of the cone technique may further improve outcomes.

Publication History

Article published online:
11 February 2025