BACKGROUND Identifying the patients with hypertrophic cardiomyopathy (HCM) in whom the risk of sudden cardiac death (SCD) justifies the implantation of a cardioverter-defibrillator (ICD) in primary prevention remains challenging. Different risk stratification and criteria are used by the European and American guidelines in this setting. We sought to evaluate the role of cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) in improving these risk stratification strategies. METHODS We conducted a multicentric retrospective analysis of HCM patients who underwent CMR for diagnostic confirmation and/or risk stratification. Eligibility for ICD was assessed according to the HCM Risk-SCD score and the American College of Cardiology Foundation/American Heart Association (ACCF/AHA) algorithm. The amount of LGE was quantified (LGE%) and categorized as 0%, 0.1-10%, 10.1-19.9% and ≥ 20%. The primary endpoint was a composite of SCD, aborted SCD, sustained ventricular tachycardia (VT), or appropriate ICD discharge. RESULTS A total of 493 patients were available for analysis (58% male, median age 46 years). LGE was present in 79% of patients, with a median LGE% of 2.9% (IQR 0.4-8.4%). The concordance between risk assessment by the HCM Risk-SCD, ACCF/AHA and LGE was relatively weak. During a median follow-up of 3.4 years (IQR 1.5-6.8 years), 23 patients experienced an event (12 SCDs, 6 appropriate ICD discharges and 5 sustained VTs). The amount of LGE was the only independent predictor of outcome (adjusted HR: 1.08; 95% CI: 1.04-1.12; p <  0.001) after adjustment for the HCM Risk-SCD and ACCF/AHA criteria. The amount of LGE showed greater discriminative power (C-statistic 0.84; 95% CI: 0.76-0.91) than the ACCF/AHA (C-statistic 0.61; 95% CI: 0.49-0.72; p for comparison < 0.001) and the HCM Risk-SCD (C-statistic 0.68; 95% CI: 0.59-0.78; p for comparison = 0.006). LGE was able to increase the discriminative power of the ACCF/AHA and HCM Risk-SCD criteria, with net reclassification improvements of 0.36 (p = 0.021) and 0.43 (p = 0.011), respectively. CONCLUSIONS The amount of LGE seems to outperform the HCM Risk-SCD score and the ACCF/AHA algorithm in the identification of HCM patients at increased risk of SCD and reclassifies a relevant proportion of patients.

中文翻译：

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在识别有心源性猝死风险的肥厚型心肌病患者时，晚期钆增强量优于当前指南推荐的标准。


背景 确定哪些肥厚型心肌病 (HCM) 患者存在心源性猝死 (SCD) 风险并证明在一级预防中植入心律转复除颤器 (ICD) 仍然具有挑战性。欧洲和美国的指南在这种情况下使用了不同的风险分层和标准。我们试图评估心血管磁共振（CMR）晚期钆增强（LGE）在改善这些风险分层策略中的作用。方法 我们对接受 CMR 进行诊断确认和/或风险分层的 HCM 患者进行了多中心回顾性分析。 ICD 的资格根据 HCM Risk-SCD 评分和美国心脏病学会基金会/美国心脏协会 (ACCF/AHA) 算法进行评估。 LGE的量被量化(LGE%)并分类为0%、0.1-10%、10.1-19.9%和≥20%。主要终点是 SCD、中止 SCD、持续性室性心动过速 (VT) 或适当的 ICD 放电的复合终点。结果 共有 493 名患者可供分析（58% 为男性，中位年龄 46 岁）。 79% 的患者存在 LGE，中位 LGE% 为 2.9%（IQR 0.4-8.4%）。 HCM Risk-SCD、ACCF/AHA 和 LGE 的风险评估一致性相对较弱。在中位随访 3.4 年（IQR 1.5-6.8 年）期间，23 名患者经历了事件（12 例 SCD、6 例适当的 ICD 放电和 5 例持续性 VT）。根据 HCM 风险-SCD 和 ACCF/AHA 标准进行调整后，LGE 量是唯一独立的结果预测因子（调整后 HR：1.08；95% CI：1.04-1.12；p < 0.001）。 LGE 的量显示出比 ACCF/AHA（C 统计量 0.61；95% CI：0.49-0）更大的判别力（C 统计量 0.84；95% CI：0.76-0.91）。72;比较 p < 0.001）和 HCM 风险-SCD（C 统计量 0.68；95% CI：0.59-0.78；比较 p = 0.006）。 LGE 能够提高 ACCF/AHA 和 HCM Risk-SCD 标准的判别力，净重分类改进分别为 0.36 (p = 0.021) 和 0.43 (p = 0.011)。结论 在识别 SCD 风险增加的 HCM 患者并重新分类相关比例的患者方面，LGE 的数量似乎优于 HCM Risk-SCD 评分和 ACCF/AHA 算法。