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Morbidity Burden in Patients With Ebstein Anomaly: The Natural History
Filip Eckerström1,2,3, Vibeke Elisabeth Hjortdal3,4, Peter Eriksson1,2
1Adult Congenital Heart Disease Unit, Department of Medicine Sahlgrenska University Hospital Gothenburg Sweden.
Journal of the American Heart Association
|November 4, 2024
Summary
Ebstein anomaly (EA) patients face significant lifelong cardiovascular issues, including high rates of arrhythmia and heart failure. This highlights the need for proactive management of EA
Area of Science:
- Cardiology
- Medical Registries
- Epidemiology
Background:
- The lifetime morbidity burden associated with Ebstein anomaly (EA) is not well-established.
- Understanding long-term outcomes is crucial for managing this congenital heart condition.
Purpose of the Study:
- To quantify the lifetime cardiovascular morbidity burden in patients with Ebstein anomaly.
- To compare morbidity in isolated EA versus EA with atrial septal defects.
Main Methods:
- A 2-country register-based study identified 794 patients with EA (born 1930-2017) and 7940 matched controls.
- Cox regression and competing risk models analyzed morbidity, with a median follow-up of 33 years.
Main Results:
- Patients with EA experienced high cumulative incidences of supraventricular arrhythmia (~70%) and ventricular preexcitation (~19%).
- Complex EA anatomy correlated with the highest cardiovascular morbidity.
- Atrial septal defects significantly impacted arrhythmia and heart failure burden.
Conclusions:
- Ebstein anomaly, with or without atrial septal defects, carries a substantial lifelong cardiovascular morbidity burden.
- Patients with EA have a vulnerable long-term prognosis requiring vigilant monitoring and management.
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