Keen Susan, Desai Milind Y
Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH, USA.
Future Cardiol. 2025 Jun;21(7):527-537. doi: 10.1080/14796678.2025.2501466. Epub 2025 Jun 3.
Hypertrophic cardiomyopathy is a heterogenous genetic condition caused by myocyte disarray with varying degrees of interstitial fibrosis. Traditional management strategies focused on symptom mitigation and sudden cardiac death prevention among high-risk patients. The only disease-modifying treatments have historically been septal reduction therapy or heart transplantation. However, cardiac myosin inhibitors have emerged as promising novel pharmacotherapies. Emerging evidence indicates that cardiac myosin inhibitors as well as surgical myectomy result in not only symptomatic benefit and reduction in left ventricular outflow tract obstruction, but also positive cardiac remodeling including reduction in left ventricular mass and maximum wall thickness, improved left ventricular diastolic parameters, reduced left atrial volumes, and improved left atrial strain. The long-term durability and clinical significance of these findings require further study. In this article, we will review the existing evidence of favorable reverse remodeling conferred by cardiac myosin inhibitors and surgical myectomy.
肥厚型心肌病是一种异质性遗传疾病,由心肌细胞排列紊乱并伴有不同程度的间质纤维化引起。传统的治疗策略侧重于缓解症状以及预防高危患者的心源性猝死。历史上唯一能改变疾病进程的治疗方法一直是室间隔减容治疗或心脏移植。然而,心肌肌球蛋白抑制剂已成为有前景的新型药物疗法。新出现的证据表明,心肌肌球蛋白抑制剂以及外科室间隔切除术不仅能带来症状改善和左心室流出道梗阻减轻,还能产生积极的心脏重塑,包括左心室质量和最大壁厚降低、左心室舒张参数改善、左心房容积减小以及左心房应变改善。这些发现的长期持续性和临床意义需要进一步研究。在本文中,我们将综述心肌肌球蛋白抑制剂和外科室间隔切除术带来有利逆向重塑的现有证据。