https://doi.org/10.1007/s00392-025-02625-4
1Elektrophysiologie Bremen Kardiologie Bremen, Deutschland; 2Klinik für Kinderherzmedizin und EMAH Bereich Kinderkardiologie Hamburg, Deutschland; 3Department of Radiology, Brigham and Women's Hospital and Harvard Medical School Non-Invasive Cardiovascular Imaging Program Boston, USA; 4Kompetenznetz Angeborene Herzfehler e. V. Berlin, Deutschland; 5Kinderherzmedizin und EMAH, Universitätsklinikum Hamburg-Eppendorf Bereich Erwachsene mit angeborenem Herzfehler Hamburg, Deutschland
Background
Ebstein’s anomaly (EA) is a rare congenital heart disease characterized by apical displacement of the hinge points of the tricuspid valve (TV) and abnormal structure of the right ventricle (RV). The study objective was to assess long-term outcomes and risk factors for morbidity and mortality for EA patients in the German National Register for 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), re-operation, heart transplantation, and all-cause mortality were assessed.
Results
Of 398 patients (230/58% female) included, 204 (51%) underwent at least one TV surgery at a median age of 14 years (IQI 7-25). Initial corrective surgery (n=193) was complicated by post-operative CAVB in 17 (9%) patients. The risk of CAVB increased with older age (p=0.001) and TV replacement compared to reconstruction (p=0.029). Post-operative survival (all-mortality death or heart transplant) at 10, 20 and 30 years was 96%, 93% and 78% after TV reconstruction and 100%, 91% and 91% after replacement (p=0.500). CAVB was an independent predictor of all-cause mortality (p<0.001). Eighty-one (39.7%) patients underwent multiple TV surgeries, which occurred most frequently in those first operated before adolescence (p=0.025). Post-operative NYHA class >I was more likely with older age (p<0.001) and post-operative cyanosis (p<0.001). The risk of post-operative TVR grade >I increased with time after surgery (p<0.001). Cone reconstruction reduced post-operative CAVB (p=0.008) and post-operative TVR compared to monocusp reconstruction (p<0.001). The 194 conservatively treated patients had less TVR and heart failure symptoms (p<0.001).
Conclusions
The to-date largest registry-based study of EA patients in Germany covering more than four decades confirms that conservative or surgical treatment results in low early and late mortality. Patients operated before adolescence were at the highest risk of re-operation, while older age at the time of the first surgery increased the risk of CAVB and post-operative heart failure. Better timing of the first TV surgery and use of the cone reconstruction technique may further improve outcomes.