Aljehani Areej, Baig Shanat, Kew Tania, Kalla Manish, Sommerfeld Laura C, Murukutla Vaishnavi Ameya, Fabritz Larissa, Steeds Richard P
Institute of Cardiovascular Sciences, University of Birmingham, Birmingham B15 2TT, UK.
Department of Cardiology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth, Birmingham B15 2GW, UK.
Biomedicines. 2024 Jan 31;12(2):328. doi: 10.3390/biomedicines12020328.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited disease characterised by early arrhythmias and structural changes. Still, there are limited echocardiography data on its structural progression. We studied structural progression and its impact on the occurrence of major adverse cardiovascular events (MACE). In this single-centre observational cohort study, structural progression was defined as the development of new major or minor imaging 2010 Task Force Criteria during follow-up. Of 101 patients, a definite diagnosis of ARVC was made in 51 patients, while non-definite 'early' disease was diagnosed in 50 patients. During 4 years of follow-up (IQR: 2-6), 23 (45%) patients with a definite diagnosis developed structural progression while only 1 patient in the non-definite (early) group gained minor imaging Task Force Criteria. Male gender was strongly associated with structural progression (62% of males progressed structurally, while 88% of females remained stable). Patients with structural progression were at higher risk of MACE (64% of patients with MACE had structural progression). Therefore, the rate of structural progression is an essential factor to be considered in ARVC studies.
致心律失常性右室心肌病（ARVC）是一种罕见的遗传性疾病，其特征为早期心律失常和结构改变。然而，关于其结构进展的超声心动图数据有限。我们研究了结构进展及其对主要不良心血管事件（MACE）发生的影响。在这项单中心观察性队列研究中，结构进展被定义为随访期间出现新的主要或次要影像学2010年工作组标准。101例患者中，51例被明确诊断为ARVC，50例被诊断为非明确的“早期”疾病。在4年的随访期间（四分位间距：2 - 6年），51例明确诊断患者中的23例（45%）出现了结构进展，而非明确（早期）组中只有1例患者获得了次要影像学工作组标准。男性与结构进展密切相关（62%的男性出现结构进展，而88%的女性保持稳定）。出现结构进展的患者发生MACE的风险更高（64%发生MACE的患者有结构进展）。因此，结构进展率是ARVC研究中需要考虑的一个重要因素。
