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Mortality in Patients With Ebstein Anomaly
Quick Takes
- Even in patients with anticipated mild Ebstein anomaly, mortality is significant, with a 35-year cumulative mortality of 11%.
- Patients with Ebstein anomaly and associated lesions had higher mortality than those with isolated Ebstein anomaly.
- Mortality for patients with Ebstein anomaly diagnosed in the modern era was lower than that of patients diagnosed prior.
Study Questions:
What is the long-term mortality of patients with Ebstein anomaly (EA)?
Methods:
A two-country registry-based cohort study was performed. Patients born from 1970 to 2017 and diagnosed with EA were identified in nationwide Danish and Swedish registries. Each patient was matched with 10 controls from the general population. Cumulative mortality and hazard ratio (HR) of mortality were used using Kaplan-Meier failure function and Cox proportional regression model.
Results:
The study included 530 patients with EA with a median follow-up of 11 years, of which 43% (n = 228) underwent cardiac surgery. Cumulative mortality was lower for patients diagnosed in the year 2000 and later as compared with the prior era (p < 0.001). Patients with isolated lesions displayed lower cumulative mortality than patients with complex lesions (p < 0.001). Patients with mild EA anatomy had a 35-year cumulative mortality of 11%, as compared with 4% for matched controls (p < 0.001). Patients with severe EA had a cumulative mortality of 18% 35 years following diagnosis.
Conclusions:
The authors conclude that 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 current era.
Perspective:
EA is a highly heterogeneous congenital cardiac lesion, with some patients requiring complex intervention in the neonatal period, and some patients living without any intervention for essentially a normal life span with mild disease. Historically, EA requiring intervention in the neonatal period has been associated with an extraordinarily high mortality. This improved significantly with the application of single ventricle palliation including over-sewing the tricuspid valve with a fenestrated patch (Starnes procedure). The management of EA continues to evolve, with the more widespread employment of the cone technique. This study provides useful information regarding long-term survival for patients with EA. Ultimately, it will be important to understand outcomes for specific patient populations including those requiring single ventricle palliation.
Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Interventions, Interventions and Structural Heart Disease
Keywords: Cardiac Surgical Procedures, Ebstein Anomaly, Heart Defects, Congenital, Pediatrics, Tricuspid Valve, Univentricular Heart
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