肥厚型心肌病与心源性猝死

肥厚型心肌病是最常见的遗传性心血管疾病，心源性猝死是其最恶劣的并发症，可以在各个年龄段发生。该病为35岁以下青年人和运动员发生心源性猝死的最主要原因。植入式心脏除颤器在预防猝死的发生方面取得了令人瞩目的成果，可以及时终止致命的恶性心律失常，挽救猝死高风险患者的生命。所以，判断哪些患者是心源性猝死的高危人群、哪些患者需进行ICD植入、如何进行危险分层至关重要。现有的危险评估模型仍有很大的局限性，需要更大规模的前瞻性研究及完整的数据采集以对其进行补充完善。

Sudden cardiac death in hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy （HCM） is one of the most common inherited cardiovascular diseases,sudden cardiac death （SCD） is the most devastating complication of it and may occur at any age.The prevention of SCD is a major aspiration for the HCM population. This disease is the most important reason for SCD in young people and athletes under the age of 35, caused significant damage to families and society. Over the past decade, studies documenting the frequency of Implantable Cardioverter Defibrillator （ICD） interventions terminating potentially lethal tachyarrhythmias, largely in adult patients undergoing implantation for high-risk status, have reported the lifesaving potential of this contemporary therapy. For the foreseeable future＋ the ICD is likely to remain the mainstay in the prevention of SCD in HCM. So, there is a clinical need to identify accurately individual patients at high risk.But we should be made aware of the limited discriminatory power of the current algorithms. Larger prospective studies with complete data acquisition from all participants will be necessary.