Cohort study
Mortality in Patients With Ebstein Anomaly
- Matched cohort study
- 2-country register-based
- nationwide registries
- matched by birth year and sex
Authors' conclusion
Mortality in patients with EA is high irrespective of presence of concomitant congenital cardiac malformations and time of diagnosis compared with the general population, but overall mortality has improved in the contemporary era.
Study context
- Population
- Patients born 1970–2017 with Ebstein anomaly, Danish and Swedish nationwide registriesmedian follow-up 11 years
- Participants
- 530 vs 5,300
- Exposure
- Ebstein anomaly vs matched general-population controls
- Ebstein anomaly (EA)n = 530
- Controln = 5,300reference
- Outcome
- Mortality (cumulative mortality and HR)
Main finding
As reported in the abstract
Mortality by presumed EA severity · 35 years
Each group vs controlHazard ratio (95% CI)P value
Mild EAP for 35-year cumulative mortality
6.0(2.7–13.6)
P < 0.001Severe EA
36.2(15.5–84.4)
Observational data: associations, not effects.
Further findings
- Cardiac surgery in EA cohort
- 43% · 228 of 530
Cumulative mortality by era of diagnosis
- Lower in modern era (2000 and later) than prior era
- P < 0.001
Cumulative mortality, isolated vs complex lesions
- Lower with isolated lesion than complex lesions
- P < 0.001
Original publication
Mortality in Patients With Ebstein Anomaly. J Am Coll Cardiol. Published 27 June 2023. doi:10.1016/j.jacc.2023.04.037
Summarised from the published abstract; every number is checked against it