Dados do Trabalho

Título

Lifetime burden of morbidity in Ebstein anomaly patients: A two-country, population-based study including 823 patients

Introdução e/ou Fundamentos

Introduction: The lifetime burden of morbidity in patients with Ebstein anomaly (EA) is to a large extent undescribed. We conducted an extensive two-country register-based collaboration aiming to describe the burden of morbidity in patients with EA.

Métodos

Methods: Patients born in the period 1930-2017 and diagnosed with EA were identified in Danish and Swedish nationwide medical registries and subsequently merged. Each EA patient was matched by birth year and gender with ten controls without congenital heart disease from the general population. Cox proportional hazard regression model was used to compute risk (hazard ratio (HR)) of morbidity (arrhythmia, heart failure, and stroke). Follow-up started at birth. Patients and controls became at risk at birth.

Resultados e Conclusões

Results: We included 823 patients and 8,230 matched controls; median follow-up was 13.2 years (interquartile range: 3.5-20.9). In the total EA cohort, 44% (n=366) underwent operative management, predominantly EA-related surgery (81%) and other congenital cardiac surgery (15%). Approximately half of the cohort had an isolated EA (n=442) and 13% (n=108) had a concomitant atrial septal defect (ASD) only. Patients with isolated EA had a 13-fold increased risk of arrhythmia and a 7-fold increased risk of heart failure compared with the general population [HRarrhythmia 13.1 (95% CI: 10.5-16.4) and HRheart failure 6.9 (95% CI: 4.9-9.8)]. The risk of arrhythmia, heart failure, and stroke among patients with EA and concomitant ASD only was 46.6 (95% CI: 27.6-78.8), 19.6 (95% CI:10.5-36.8), and 8.3 (95% CI: 4.0-17.4), respectively. Patients with non-operated isolated EA had a 9-fold increased risk of arrhythmia and a 5-fold increased risk of heart failure compared with the general population [HRarrhythmia 9.4 (95% CI: 6.9-12.7) and HRheart failure 5.2 (95% CI: 3.2-8.3)]. Corresponding estimates for operated patients with isolated EA was 25.0 (95% CI: 17.7-35.3) and 10.1 (95% CI: 6.0-16.9], respectively. Conclusion: Patients with EA carries a substantial burden of arrhythmia, heart failure, and stroke compared with the general population. Even patients with isolated EA without need for operative management demonstrated high risk of arrhythmia and heart failure, necessitating meticulous follow-up in specialized adult congenital heart disease clinics to detect and possibly prevent or limit morbidity at an early stage, irrespective of treatment pathway and associated cardiac malformations.

Palavras Chave

Congenital,Ebstein anomaly