The 2019 arrhythmogenic right ventricular cardiomyopathy (ARVC) risk model has proved insufficient in the capability of predicting ventricular arrhythmia (VA) risk in non-classical arrhythmogenic cardiomyopathy (ACM). Furthermore, the prognostic value of ringlike late gadolinium enhancement (LGE) of the left ventricle in non-classical ACM remains unknown. We aimed to assess the incremental value of ringlike LGE over the 2019 ARVC risk model in predicting sustained VA in patients with non-classical ACM. In this retrospective study, consecutive patients with non-classical ACM who underwent CMR from January 2011 to January 2022 were included. The pattern of LGE was categorized as no, non-ringlike, and ringlike LGE. The primary outcome was defined as the occurrence of sustained VA. Univariable and multivariable Cox regression analysis was used to evaluate the impact of LGE patterns on sustained VA and area under curve (AUC) was calculated for the incremental value of ringlike LGE. A total of 73 patients were collected in the final cohort (mean age, 39.3 ± 14.4 years, 51 male), of whom 10 (13.7%) had no LGE, 33 (45.2%) had non-ringlike LGE, and 30 (41.1%) had ringlike LGE. There was no statistically significant difference in the 5-year risk score among the three groups (P = 0.190). During a median follow-up of 34 (13–56) months, 34 (46.6%) patients experienced sustained VA, including 1 (10.0%), 13 (39.4%) and 20 (66.7%) of patients with no, non-ringlike and ringlike LGE, respectively. After multivariable adjustment, ringlike LGE remained independently associated with the presence of sustained VA (adjusted hazard ratio: 6.91, 95% confidence intervals: 1.89–54.60; P = 0.036). Adding ringlike LGE to the 2019 ARVC risk model showed significantly incremental prognostic value for sustained VA (AUC: 0.80 vs. 0.67; P = 0.024). Ringlike LGE provides independent and incremental prognostic value over the 2019 ARVC risk model in patients with non-classical ACM.

中文翻译：

---

环状晚期钆增强为非经典致心律失常性心肌病提供了增量预后价值

2019年致心律失常性右室心肌病（ARVC）风险模型已被证明在预测非经典致心律失常性心肌病（ACM）的室性心律失常（VA）风险方面能力不足。此外，左心室环状晚期钆增强（LGE）在非经典 ACM 中的预后价值仍不清楚。我们的目的是评估环状 LGE 相对于 2019 ARVC 风险模型在预测非经典 ACM 患者持续 VA 方面的增量价值。在这项回顾性研究中，纳入了 2011 年 1 月至 2022 年 1 月期间连续接受 CMR 的非经典 ACM 患者。LGE 的模式分为无、非环状和环状 LGE。主要结局定义为持续 VA 的发生。使用单变量和多变量 Cox 回归分析来评估 LGE 模式对持续 VA 的影响，并计算环形 LGE 增量值的曲线下面积 (AUC)。最终队列中总共收集了 73 名患者（平均年龄，39.3 ± 14.4 岁，51 名男性），其中 10 名（13.7%）没有 LGE，33 名（45.2%）有非环状 LGE，30 名（41.1%）有非环状 LGE。 %) 具有环状 LGE。三组间5年风险评分差异无统计学意义（P=0.190）。在中位随访 34 (13-56) 个月期间，34 名 (46.6%) 患者经历了持续 VA，其中 1 名 (10.0%)、13 名 (39.4%) 和 20 名 (66.7%) 患者没有、非分别为环状和环状LGE。经过多变量调整后，环状 LGE 仍然与持续 VA 的存在独立相关（调整后的风险比：6.91，95% 置信区间：1.89–54.60；P = 0.036）。将环状 LGE 添加到 2019 ARVC 风险模型中显示出持续 VA 的预后价值显着增加（AUC：0.80 与 0.67；P = 0.024）。Ringlike LGE 为非经典 ACM 患者提供了比 2019 ARVC 风险模型独立且增量的预后价值。