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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the的后期增强量在识别有心脏猝死风险的肥厚型心肌病患者中胜过当前的指南推荐标准。

背景技术对于识别出肥厚型心肌病（HCM）的患者，其心脏猝死（SCD）的风险可以证明在一级预防中植入心脏复律除颤器（ICD）仍然具有挑战性。在这种情况下，欧洲和美国指南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）g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诊断确诊和/或风险分层的HCM患者进行了多中心回顾性分析。根据HCM Risk-SCD评分和美国心脏病学会/美国心脏协会（ACCF / AHA）算法评估ICD的资格。定量LGE的量（LGE％），并分类为0％，0.1-10％，10.1〜19.9％和≥20％。主要终点是SCD，流产的SCD，持续性室性心动过速（VT）或适当的ICD排出的复合物。结果共有493例患者可供分析（男性58％，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进行的风险评估之间的一致性相对较弱。在3.4年（IQR 1.5-6.8年）的中位随访期间，有23名患者经历了一次事件（12例SCD，6例适当的ICD出院和5例持续性VT）。调整HCM Risk-SCD和ACCF / AHA标准后，LGE的量是结果的唯一独立预测因子（校正后的HR：1.08； 95％CI：1.04-1.12； p <0.001）。与ACCF / AHA（C统计0.61; 95％CI：0.49-0.72; p用于比较<0.001）和HCM相比，LGE的量具有更大的判别力（C统计0.84; 95％CI：0.76-0.91） Risk-SCD（C统计量0.68; 95％CI：0.59-0.78; p = 0.006）。LGE能够提高ACCF / AHA和HCM Risk-SCD标准的判别力，净重分类分别提高了0.36（p = 0.021）和0.43（p = 0.011）。结论在确定SCD风险增加的HCM患者中，LGE的数量似乎优于HCM Risk-SCD评分和ACCF / AHA算法，并重新分类了相关比例的患者。0.59-0.78；用于比较的p = 0.006）。LGE能够提高ACCF / AHA和HCM Risk-SCD标准的判别力，净重分类分别提高了0.36（p = 0.021）和0.43（p = 0.011）。结论在确定具有SCD风险的HCM患者中，LGE的数量似乎优于HCM Risk-SCD评分和ACCF / AHA算法，并重新分类了相关比例的患者。0.59-0.78；用于比较的p = 0.006）。LGE能够提高ACCF / AHA和HCM Risk-SCD标准的判别力，净重分类分别提高了0.36（p = 0.021）和0.43（p = 0.011）。结论在确定SCD风险增加的HCM患者中，LGE的数量似乎优于HCM Risk-SCD评分和ACCF / AHA算法，并重新分类了相关比例的患者。