Chua Aileen Paula, Laenens Dorien, Sarrazyn Camille, Lopez-Santi Maria Pilar, Nabeta Takeru, Myagmardorj Rinchyenkhand, Bootsma Marianne, Barge-Schaapveld Daniela Q C M, Bax Jeroen J, Marsan Nina Ajmone
Department of Cardiology, Heart Lung Center, Leiden University Medical Center, Leiden, The Netherlands.
Department of Clinical Genetics, Leiden University Medical Center, Leiden, The Netherlands.
Am J Cardiol. 2025 Apr 15;241:61-68. doi: 10.1016/j.amjcard.2025.01.006. Epub 2025 Jan 11.
Despite arrhythmogenic right ventricular cardiomyopathy (ARVC) being predominantly a right ventricular (RV) disease, concomitant left ventricular (LV) involvement has been recognized. ARVC is diagnosed by the RV-centric 2010 Task Force Criteria(TFC) using routine echocardiography, but previous studies have suggested that strain imaging may be more sensitive to detect RV and LV dysfunction. No data however are available regarding the additional value of combining biventricular strain for risk stratification. This study aims to assess the prognostic value of both LV global longitudinal strain (GLS) and RV free wall strain (FWLS) in patients with ARVC. To accomplish this, 204 patients who met the TFC for the ARVC spectrum were included. Patients (age 41 ± 17 years,55% men) were divided into impaired(n = 33), discordant (RV or LV impaired, n = 70), and normal (n = 101) strain groups based on a value of ≥18% for both ventricles. During a follow-up of 87 [24-136] months, 57 (28%) experienced the composite outcome of all-cause mortality, arrhythmic events, implantable cardioverter defibrillator therapy and heart failure events, and a significant difference in event-free survival was observed (p <0.001) between the 3 groups. In the multivariable analysis, the strain groups remained associated with outcomes (p = 0.014) after adjusting for age, sex, history of syncope and definite ARVC diagnosis. A subanalysis including only definite and borderline diagnosed ARVC confirmed that the strain groups were independently predictive of the endpoint (p = 0.023). In conclusion, biventricular involvement by strain analysis may help risk stratification in ARVC patients, with the worst outcomes of patients with both RV and LV impaired strain.
尽管致心律失常性右室心肌病（ARVC）主要是一种右心室（RV）疾病，但左心室（LV）受累也已得到认可。ARVC通过以右心室为中心的2010年工作组标准（TFC），利用常规超声心动图进行诊断，但先前的研究表明，应变成像在检测右心室和左心室功能障碍方面可能更敏感。然而，关于联合双心室应变进行风险分层的附加价值尚无数据。本研究旨在评估左心室整体纵向应变（GLS）和右心室游离壁应变（FWLS）在ARVC患者中的预后价值。为实现这一目标，纳入了204例符合ARVC谱系TFC的患者。患者（年龄41±17岁，55%为男性）根据双心室≥18%的值分为应变受损组（n = 33）、不一致组（右心室或左心室受损，n = 70）和正常组（n = 101）。在87 [24 - 136]个月的随访期间，57例（28%）经历了全因死亡、心律失常事件、植入式心律转复除颤器治疗和心力衰竭事件的复合结局，并且在3组之间观察到无事件生存率有显著差异（p <0.001）。在多变量分析中，在调整年龄、性别、晕厥病史和明确的ARVC诊断后，应变组仍与结局相关（p = 0.014）。一项仅包括明确和临界诊断的ARVC的亚分析证实，应变组可独立预测终点（p = 0.023）。总之，通过应变分析的双心室受累情况可能有助于ARVC患者的风险分层，右心室和左心室应变均受损的患者预后最差。
