Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN 55407, USA.
Cardiovasc Pathol. 2010 Nov-Dec;19(6):329-35. doi: 10.1016/j.carpath.2010.03.002. Epub 2010 Apr 8.
Hypertrophic cardiomyopathy is regarded as the most common cause of sudden cardiac death in young people (including trained athletes). Introduction of implantable cardioverter-defibrillators to the hypertrophic cardiomyopathy patient population represents a new paradigm for clinical practice and perhaps the most significant advance in the management of this disease to date. Implantable defibrillators offer the only proven protection against sudden death by virtue of effectively terminating ventricular tachycardia/fibrillation and, in the process, altering the natural history of hypertrophic cardiomyopathy and providing the potential opportunity of normal or near-normal longevity for many patients. However, targeting the most appropriate candidates for prophylactic device therapy can be complex, compounded by the unpredictability of the underlying arrhythmogenic substrate, absence of a single dominant and quantitative risk marker in this heterogeneous disease, and the historical difficulty in assembling sufficiently powered prospective and randomized trials in large patient populations. Nevertheless, the current risk factor algorithm, when combined with a measure of individual physician judgment, is an effective strategy for identifying high-risk patients. Indeed, prevention of sudden death has now become an integral, albeit challenging, component of overall hypertrophic cardiomyopathy management.
肥厚型心肌病被认为是年轻人（包括训练有素的运动员）心脏性猝死的最常见原因。植入式心脏复律除颤器的引入代表了临床实践的一个新范例，也许是迄今为止肥厚型心肌病治疗中最重要的进展。植入式除颤器通过有效终止室性心动过速/颤动提供了唯一可证明的预防猝死的保护，从而改变了肥厚型心肌病的自然病程，并为许多患者提供了正常或接近正常寿命的潜在机会。然而，针对最合适的预防性器械治疗候选者可能很复杂，这是由潜在的心律失常底物的不可预测性、这种异质性疾病缺乏单一的主导和定量风险标志物以及在大型患者群体中进行充分的前瞻性和随机试验的历史困难所造成的。然而，目前的风险因素算法，结合个体医生判断的衡量标准，是识别高危患者的有效策略。事实上，预防猝死现在已成为肥厚型心肌病整体管理的一个不可或缺的（尽管具有挑战性）组成部分。
