Aaserud Linda T, Rootwelt-Norberg Christine, Olsen Paul A S, Five Christian K, Castrini Anna I, Aabel Eivind W, Haugaa Kristina H, Lie Øyvind H
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway; Department of Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway; ProCardio Center for Innovation, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway; ProCardio Center for Innovation, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Unit for Cardiac and Cardiovascular Genetics, Department of Medical Genetics, Oslo University Hospital, Ullevaal, Oslo, Norway.
JACC Cardiovasc Imaging. 2025 Aug;18(8):853-863. doi: 10.1016/j.jcmg.2025.03.018. Epub 2025 Jul 9.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inheritable heart disease, whereas exercise-induced arrhythmogenic cardiomyopathy (EiAC) is a proposed acquired similar phenotype in athletes. The differences in disease progression between these entities are not well understood.
This study aims to assess structural, functional, and arrhythmic disease progression in EiAC compared with ARVC.
This longitudinal cohort study included EiAC patients who were competitive endurance athletes (>24 MET-hours/week for >6 consecutive years) referred due to ventricular arrhythmias (VA), without inherited or genetic factors or other evident causes, and genotype-positive ARVC patients with a definite diagnosis and their genotype-positive family members for comparison. Disease progression was assessed by repeated echocardiographic examinations and incident VA during long-term follow-up.
The authors included 125 ARVC patients (61 women, aged 38 ± 17 years) and 41 EiAC patients (6 women, aged 45 ± 13 years) and followed them for 96 months (Q1-Q3: 73-132 months) and 82 months (Q1-Q3: 50-118 months), respectively. The authors analyzed 730 echocardiographic examinations (538 ARVC, 192 EiAC). Right ventricular (RV) structure and function remained stable in EiAC patients, whereas those in ARVC patients deteriorated during follow-up. The 5-year and 10-year cumulative incidences of VA were similar between EiAC and ARVC patients.
RV structure and function deteriorated in ARVC patients but remained stable in EiAC patients during follow-up. The incidence of VA was high in both populations. These results indicate that EiAC patients should be followed closely over time regardless of structural and functional progression.
致心律失常性右室心肌病（ARVC）是一种遗传性心脏病，而运动诱发的致心律失常性心肌病（EiAC）是运动员中一种后天获得的类似表型。目前对这两种疾病在疾病进展方面的差异了解不足。
本研究旨在评估与ARVC相比，EiAC的结构、功能和心律失常疾病进展情况。
这项纵向队列研究纳入了因室性心律失常（VA）转诊的EiAC患者，这些患者是竞技耐力运动员（连续6年以上每周>24代谢当量小时），无遗传或基因因素或其他明显病因，以及明确诊断的基因型阳性ARVC患者及其基因型阳性家庭成员作为对照。通过长期随访期间重复的超声心动图检查和新发VA来评估疾病进展。
作者纳入了125例ARVC患者（61名女性，年龄38±17岁）和41例EiAC患者（6名女性，年龄45±13岁），分别对他们进行了96个月（第一四分位数-第三四分位数：73-132个月）和82个月（第一四分位数-第三四分位数：50-118个月）的随访。作者分析了730次超声心动图检查（538次ARVC，192次EiAC）。EiAC患者的右心室（RV）结构和功能保持稳定，而ARVC患者的在随访期间恶化。EiAC和ARVC患者的VA 5年和10年累积发生率相似。
随访期间，ARVC患者的RV结构和功能恶化，但EiAC患者保持稳定。这两个人群的VA发生率都很高。这些结果表明，无论结构和功能进展如何，都应随时间对EiAC患者进行密切随访。
