肥厚性心肌病的多模式治疗。

Shiavax J Rao, Shaikh B Iqbal, Arjun S Kanwal, Wilbert S Aronow, Srihari S Naidu
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引用次数: 2

摘要

肥厚性心肌病(HCM)是一种常染色体显性遗传病,定义为左心室壁厚度大于15mm,没有其他条件可以解释肥厚程度。伴有左心室流出道梗阻的梗阻性HCM由心室内收缩压梯度大于或等于30mmhg来定义。在过去的几十年里,HCM患者的侵入性和药物治疗选择不断扩大,最近的指南呼吁采用侵入性和非侵入性治疗策略。有几种侵入性治疗方法,包括已证实的治疗方法,如酒精室间隔消融和室间隔肌切除术。新的侵入性治疗,如MitraClip,射频间隔消融和SESAME手术最近得到了推广。药理学治疗也发生了巨大的变化,包括传统的药物治疗,如-受体阻滞剂、钙通道阻滞剂和双酰胺。Mavacamten,一种新型心肌肌球蛋白抑制剂,可能会显著改变治疗方法。其他肌球蛋白抑制剂和调节剂也正在开发中,并在大型临床试验中进行测试。考虑到HCM患者显著的表型变异性,临床管理可能具有挑战性,通常需要结合侵入性和非侵入性选择的个性化方法。
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Multi-modality management of hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant inherited condition defined by left ventricular wall thickness greater than 15 mm in the absence of other conditions that could explain that degree of hypertrophy. Obstructive HCM associated with left ventricular outflow tract obstruction is defined by an intraventricular systolic pressure gradient greater than or equal to 30 mm Hg. Over the past couple of decades, there has been an expansion of both invasive and pharmacotherapeutic options for patients with HCM, with recent guidelines calling for a melody of invasive and non-invasive treatment strategies. There are several invasive therapies including proven therapies such as alcohol septal ablation and septal myectomy. Novel invasive therapies such as MitraClip, radiofrequency septal ablation and SESAME procedure have more recently been promoted. Pharmacological therapy has also dramatically evolved and includes conventional medications such as beta-blockers, calcium channel blockers, and disopyramide. Mavacamten, a novel cardiac myosin inhibitor, may significantly change management. Other myosin inhibitors and modulators are also being developed and tested in large clinical trials. Given significant phenotypical variability in patients with HCM, clinical management can be challenging, and often requires an individualized approach with a combination of invasive and non-invasive options.

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来源期刊
Hospital practice (1995)
Hospital practice (1995) Medicine-Medicine (all)
CiteScore
2.80
自引率
0.00%
发文量
54
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