What question did this study set out to answer?

To investigate mortality rates in patients with Ebstein anomaly using data from two countries.

August 13, 2026Journal of the American College of Cardiology22 citationsOpen Access

Mortality in Patients With Ebstein Anomaly

Q: What is the clinical evidence from this study?

Study design: Cohort. Population: Ebstein Anomaly (n=5830). Intervention: Ebstein Anomaly vs. Matched controls from the general population without congenital heart disease. Primary outcome: All-cause mortality (HR 25.0, 95% CI 16.9-37.2, p=<0.001).

FEFilip EckerströmPediatric / Congenital Cardiology MDMikael DellborgPediatric / Congenital Cardiology VHVibeke E. HjortdalPediatric / Congenital Cardiology

Key Result

Ebstein anomaly was associated with a significantly higher risk of mortality compared to the general population, with an overall hazard ratio of 25.0, although survival has improved in the modern era.

Key Points

To investigate mortality rates in patients with Ebstein anomaly using data from two countries.
Identified patients born from 1970 to 2017 diagnosed with EA from Danish and Swedish registries.
Matched patients by birth year and sex with 10 control subjects from the general population.
Computed cumulative mortality and hazard ratios using Kaplan-Meier function and Cox regression model.
Included 530 patients with EA and 5,300 controls with a median follow-up of 11 years.
Patients diagnosed after the year 2000 had lower cumulative mortality (P < 0.001) than those diagnosed earlier.
Patients with severe EA had a 35-year cumulative mortality of 18% and an HR of 36.2 compared to controls.

Study Design

Type

Cohort (n=5,830)

Multicenter

Yes

Structured PICO

Does Ebstein anomaly increase mortality compared to the general population?

Population

530 patients with Ebstein anomaly and 5,300 matched control subjects from the general population, followed for a median of 11 years.

Comparator

5,300 control subjects from the general population without congenital heart disease, matched by country of living, birth year, and sex (10 controls per EA patient).

Outcome

Cumulative mortality and hazard ratio (HR) of mortalityhard clinical

Mortality in patients with Ebstein anomaly remains significantly higher than the general population, even in those with presumed mild disease, although overall survival has improved in the contemporary era.

Main Result

Hazard Ratio: 25 (95% CI 16.9–37.2)

Absolute Event Rate: 15.1% vs 0.68%

p-value: p=<0.001

Limitations

Potential differences in the constitution of birth cohorts with respect to EA severity due to increased detection of milder forms in the modern era.
Registries lack clinical data and granular surgery information, preventing analysis of survival with respect to tricuspid valve morphology and ventricular function.
Cause of death registered may lack precision when autopsy is not performed.
Potential selection bias as mild EA in the historical era might have remained undetected, and survivorship bias in late-diagnosed patients.
Potential overestimation of mortality in late-diagnosed patients because follow-up starts at the time of diagnosis rather than birth.

Abstract

BACKGROUND Low birth prevalence and referral bias constitute significant obstacles to elucidating the natural history of Ebstein anomaly (EA). OBJECTIVES An extensive 2-country register-based collaboration was performed to investigate the mortality in patients with EA. METHODS Patients born from 1970 to 2017 and diagnosed with EA were identified in Danish and Swedish nationwide medical registries. Each patient was matched by birth year and sex with 10 control subjects from the general population. Cumulative mortality and HR of mortality were computed using Kaplan-Meier failure function and Cox proportional regression model. RESULTS The study included 530 patients with EA and 5,300 matched control subjects with a median follow-up of 11 years. In the EA cohort, 43% (228) underwent cardiac surgery. Cumulative mortality was lower for patients diagnosed in the modern era (the year 2000 and later) than for those diagnosed in the prior era (P < 0.001). Patients with isolated lesion displayed lower cumulative mortality than patients with complex lesions did (P < 0.001). Patients with a presumed mild EA anatomy displayed a 35-year cumulative mortality of 11% (vs 4% for the matched control subjects; P < 0.001), yielding an HR for mortality of 6.0 (95% CI: 2.7-13.6), whereas patients with presumed severe EA demonstrated an HR of 36.2 (95% CI: 15.5-84.4) compared with control subjects and a cumulative mortality of 18% 35 years following diagnosis. CONCLUSIONS 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.

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Cite This Study

Eckerström et al. (Thu,) conducted a cohort in Ebstein Anomaly (n=5,830). Ebstein Anomaly vs. Matched controls from the general population without congenital heart disease was evaluated on All-cause mortality (HR 25.0, 95% CI 16.9-37.2, p=<0.001). Ebstein anomaly was associated with a significantly higher risk of mortality compared to the general population, with an overall hazard ratio of 25.0, although survival has improved in the modern era.

synapsesocial.com/papers/6a7de05e1c0f4dfc2773ca66 https://doi.org/https://doi.org/10.1016/j.jacc.2023.04.037

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