Survival, tachyarrhythmias and adverse cardiovascular outcomes in adult patients with Ebstein anomaly

Chun-Wei Lu; Mei-Hwan Wu; Shuenn-Nan Chiu; Jou-Kou Wang; Wei-Chieh Tseng; Chun-An Chen; Ming-Tai Lin

doi:10.1016/j.jfma.2025.05.038

Survival, tachyarrhythmias and adverse cardiovascular outcomes in adult patients with Ebstein anomaly

J Formos Med Assoc. 2025 Jun 3:S0929-6646(25)00268-2. doi: 10.1016/j.jfma.2025.05.038. Online ahead of print.

Authors

Chun-Wei Lu¹, Mei-Hwan Wu², Shuenn-Nan Chiu¹, Jou-Kou Wang¹, Wei-Chieh Tseng¹, Chun-An Chen¹, Ming-Tai Lin¹

Affiliations

¹ Department of Pediatrics, National Taiwan University Hospital and Medical College, National Taiwan University, Taipei, Taiwan.
² Department of Pediatrics, National Taiwan University Hospital and Medical College, National Taiwan University, Taipei, Taiwan. Electronic address: wumh@ntu.edu.tw.

PMID: 40467398
DOI: 10.1016/j.jfma.2025.05.038

Abstract

Background: The clinical spectrum of adults with Ebstein anomaly (EA) is variable, influenced by interventions and aging.

Objectives: To assess long-term outcomes in adults with EA who survived into adulthood.

Methods: This retrospective study analyzed 106 adults with EA treated between 1980 and 2022.

Results: Of the 106 adults, 14 received definitive intervention in childhood and 34 in adulthood (2 underwent heart transplantation). Transplant-free survival by age 60 was 0.885 ± 0.061. Preexcitation was present in 32 patients (30.2 %), with 29 developing tachyarrhythmias, 11 of whom experienced them in adulthood. Ablation was successful in 23 patients (16 during adulthood). Excluding those who underwent childhood ablation, the incidence of adult-onset supraventricular tachycardia (SVT) was 15.6 % by age 40 and 20 % by age 50. Among patients with preexcitation, the incidence of adult-onset SVT reached 83.6 % by age 50. Adult-onset atrial tachyarrhythmias became more common with age, with incidences of 21.7 %, 37.5 %, and 58.9 % by ages 50, 60, and 70, respectively. These were not associated with preexcitation. Notably, none of the seven patients who underwent successful ablation in childhood developed atrial tachyarrhythmias. Adverse cardiovascular outcomes occurred in 9.1 %, 17.7 %, and 41.2 % of patients aged 18-39, 40-59, and 60+, respectively, with atrial tachyarrhythmias as the most significant predictor.

Conclusions: Adults with EA face a substantial risk of tachyarrhythmias and adverse cardiovascular outcomes, both of which increase with age. By age 50, the incidence of atrial tachyarrhythmias surpasses that of SVT and strongly predicts adverse cardiovascular outcomes.

Keywords: Adult congenital heart disease; Ebstein anomaly; Long-term outcomes.