Back to normal: super-response to mavacamten in a patient with obstructive hypertrophic cardiomyopathy.

Tobia Albertini, Alain M Bernheim, Annina A Studer Bruengger
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Abstract

Hypertrophic cardiomyopathy (HCM) is a primary myocardial disorder characterized by left ventricular hypertrophy, often leading to left ventricular outflow tract (LVOT) obstruction and secondary mitral regurgitation (MR) due to systolic anterior motion (SAM) of the mitral valve leaflets. Mavacamten, a cardiac myosin inhibitor, is a modern treatment option aimed at reducing myocardial contractility, SAM and LVOT obstruction. We present the case of a patient with HCM responding not only with a marked reduction in LVOT obstruction and mitral regurgitation, but also with normalization of left ventricular (LV) mass.

恢复正常:阻塞性肥厚性心肌病患者对马伐卡坦的超反应。
肥厚性心肌病(HCM)是一种以左心室肥厚为特征的原发性心肌疾病,常导致左心室流出道(LVOT)阻塞和二尖瓣小叶收缩前运动(SAM)引起的继发性二尖瓣反流(MR)。Mavacamten是一种心肌肌球蛋白抑制剂,是一种旨在降低心肌收缩力、SAM和LVOT阻塞的现代治疗选择。我们报告了一例HCM患者的反应,不仅是LVOT阻塞和二尖瓣反流的显著减少,而且左心室(LV)质量的正常化。
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