Aljehani Areej, Win Kyaw Zaw, Baig Shanat, Kalla Manish, Ensam Bode, 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, Birmingham B15 2GW, UK.
J Cardiovasc Dev Dis. 2024 Dec 3;11(12):388. doi: 10.3390/jcdd11120388.
Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetic disorder associated with an elevated risk of life-threatening arrhythmias and progressive ventricular impairment. Risk stratification is essential to prevent major adverse cardiac events (MACE). Our study aimed to investigate the incremental value of strain measured by two-dimensional speckle-tracking echocardiography in predicting MACE in ARVC patients compared to conventional echocardiographic parameters. Methods and Results This was a retrospective, single-centre cohort study of 83 patients with ARVC (51% males, median age 37 years (IQR: 23, 53)) under the care of the Inherited Cardiac Conditions clinic at University Hospital Birmingham. MACE was defined as one of the following: sustained ventricular tachycardia (Sus VT), ventricular fibrillation (VF), appropriate implantable cardio-defibrillator (ICD) therapy [shock/anti-tachycardia pacing (ATP)], heart failure (defined as decompensated heart failure, cardiac index by heart catheter, HF medication, and symptoms), cardiac transplantation, or cardiac death. Echocardiography images were analysed by a single observer for right ventricle (RV) and left ventricular (LV) global longitudinal strain (GLS). Multivariable Cox regression was performed in combination with RV fractional area change and tricuspid annular plane systolic excursion. During three years of follow-up, 12% of patients suffered a MACE. ARVC patients with MACE had significantly reduced RV GLS (-13 ± 6% vs. -23 ± 6%, < 0.001) and RV free wall longitudinal strain (-15 ± 5% vs. -25 ± 7%, < 0.001) compared to those without MACE. Conclusions Right ventricular free wall longitudinal strain (RVFWLS) may be a more sensitive predictor of MACE than conventional echocardiographic parameters of RV function. Moreover, RV-free wall longitudinal strain may have superior predictive value compared to RV GLS.
致心律失常性右室心肌病（ARVC）是一种罕见的遗传性疾病，与危及生命的心律失常和进行性心室功能损害风险升高相关。风险分层对于预防主要不良心脏事件（MACE）至关重要。我们的研究旨在探讨与传统超声心动图参数相比，二维斑点追踪超声心动图测量的应变在预测ARVC患者MACE中的增量价值。
这是一项回顾性、单中心队列研究，纳入了伯明翰大学医院遗传性心脏病诊所诊治的83例ARVC患者（51%为男性，中位年龄37岁（四分位间距：23，53））。MACE定义为以下之一：持续性室性心动过速（Sus VT）、心室颤动（VF）、合适的植入式心脏除颤器（ICD）治疗[电击/抗心动过速起搏（ATP）]、心力衰竭（定义为失代偿性心力衰竭、心导管检查测得的心脏指数、心力衰竭药物治疗及症状）、心脏移植或心源性死亡。由一名观察者分析超声心动图图像，测量右心室（RV）和左心室（LV）整体纵向应变（GLS）。结合RV面积变化分数和三尖瓣环平面收缩期位移进行多变量Cox回归分析。在三年的随访期间，12%的患者发生了MACE。与未发生MACE的ARVC患者相比，发生MACE的患者RV GLS显著降低（-13±6% vs. -23±6%，P<0.001），RV游离壁纵向应变也显著降低（-15±5% vs. -25±7%，P<0.001）。
右心室游离壁纵向应变（RVFWLS）可能是比传统RV功能超声心动图参数更敏感的MACE预测指标。此外，与RV GLS相比，RV游离壁纵向应变可能具有更高的预测价值。
