Fondazione Toscana G. Monasterio, Pisa, Italy.
Cardio-thoraco-vascular Department, University of Trieste, Trieste, Italy.
J Am Coll Cardiol. 2020 Jun 9;75(22):2753-2765. doi: 10.1016/j.jacc.2020.04.023.
Cardiac magnetic resonance (CMR) is widely used to assess tissue and functional abnormalities in arrhythmogenic right ventricular cardiomyopathy (ARVC). Recently, a ARVC risk score was proposed to predict the 5-year risk of malignant ventricular arrhythmias in patients with ARVC. However, CMR features such as fibrosis, fat infiltration, and left ventricular (LV) involvement were not considered.
The authors sought to evaluate the prognostic role of CMR phenotype in patients with definite ARVC and to evaluate the effectiveness of the novel 5-year ARVC risk score to predict cardiac events in different CMR presentations.
A total of 140 patients with definite ARVC were enrolled (mean age 42 ± 17 years, 97 males) in this multicenter prospective registry. As per study design, CMR was performed in all the patients at enrollment. The novel 5-year ARVC risk score was retrospectively calculated using the patient's characteristics at the time of enrollment. During a median follow-up of 5 years (2 to 8 years), the combined endpoint of sudden cardiac death, appropriate implantable cardioverter-defibrillator intervention, and aborted cardiac arrest was considered.
CMR was completely negative in 14 patients (10%), isolated right ventricular (RV) involvement was found in 58 (41%), biventricular in 52 (37%), and LV dominant in 16 (12%). During the follow-up, 48 patients (34%) had major events, but none occurred in patients with negative CMR. At Kaplan-Meier analysis, patients with LV involvement (LV dominant and biventricular) had a worse prognosis than those with lone RV (p < 0.0001). At multivariate analysis, the LV involvement, a LV-dominant phenotype, and the 5-year ARVC risk score were independent predictors of major events. The estimated 5-year risk was able to predict the observed risk in patients with lone RV but underestimated the risk in those with LV involvement.
Different CMR presentations of ARVC are associated with different prognoses. The 5-year ARVC risk score is valid for the estimation of risk in patients with lone-RV presentation but underestimated the risk when LV is involved.
心脏磁共振（CMR）广泛用于评估心律失常性右心室心肌病（ARVC）的组织和功能异常。最近，提出了一种 ARVC 风险评分来预测 ARVC 患者 5 年内恶性室性心律失常的风险。然而，CMR 特征如纤维化、脂肪浸润和左心室（LV）受累并未被考虑在内。
作者旨在评估 CMR 表型在明确 ARVC 患者中的预后作用，并评估新型 5 年 ARVC 风险评分在不同 CMR 表现中预测心脏事件的有效性。
本多中心前瞻性研究纳入了 140 名明确 ARVC 患者（平均年龄 42 ± 17 岁，97 名男性）。根据研究设计，所有患者在入组时均行 CMR 检查。使用患者入组时的特征，回顾性计算新型 5 年 ARVC 风险评分。在中位随访 5 年（2 至 8 年）期间，考虑到心源性猝死、适当植入式心脏复律除颤器干预和心脏骤停的联合终点。
14 名患者（10%）的 CMR 完全阴性，58 名患者（41%）为孤立性右心室（RV）受累，52 名患者（37%）为双心室受累，16 名患者（12%）为 LV 为主。在随访期间，48 名患者（34%）发生了主要事件，但 CMR 阴性的患者均未发生。在 Kaplan-Meier 分析中，LV 受累（LV 为主和双心室）患者的预后较单独 RV 受累患者差（p < 0.0001）。多变量分析显示，LV 受累、LV 为主的表型和 5 年 ARVC 风险评分是主要事件的独立预测因素。估计的 5 年风险能够预测单独 RV 患者的实际风险，但低估了 LV 受累患者的风险。
ARVC 的不同 CMR 表现与不同的预后相关。5 年 ARVC 风险评分可用于预测单独 RV 患者的风险，但低估了 LV 受累患者的风险。
