Güler Arda, Erata Yunus Emre, Demirtola Ayşe İrem, Türkmen İrem, Atmaca Sezgin, Şahin Hasan, Aydın Sinem, Almasri Muayad, Tekin Meltem, Coşkun Gizemnur, Uygur Begüm, Cansever Aysel Turkvatan, Güler Gamze Babur
University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Department of Cardiology, Istanbul, Turkey.
University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Department of Cardiology, Istanbul, Turkey.
Int J Cardiol. 2025 Feb 15;421:132913. doi: 10.1016/j.ijcard.2024.132913. Epub 2024 Dec 18.
Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular outflow tract obstruction (LVOTO), leading to symptoms and adverse outcomes. Disopyramide, with its negative inotropic effects, is commonly used to reduce LVOTO in obstructive HCM (HOCM). This study evaluates the impact of disopyramide on functional capacity in HOCM patients.
Symptomatic HOCM patients evaluated between October 2021 and May 2024 were divided into two groups: those receiving disopyramide and those treated with beta-blockers or calcium channel blockers due to disopyramide unavailability. A treatment response was defined as at least a 1-stage improvement in NYHA class. Clinical and laboratory data, including NT-proBNP levels and LVOT gradient, were compared between groups.
A total of 127 patients were included (median age 54.0 years, 58.2% male). 79% of patients were in NYHA class 2. After follow-up, 62% of the disopyramide group (the mean follow-up duration was 15.2 months, and the mean daily dose was 395 mg) showed at least a 1-stage improvement in functional capacity, compared to 26% in the control group. Disopyramide use and presence of extent LGE were independent predictors of functional improvement. Despite functional gains, there was no difference in overall clinical outcomes between the groups. Disopyramide-related side effects were minimal, and no patients discontinued due to QT prolongation.
Disopyramide significantly improved functional capacity in HOCM patients, with 62% achieving a 1-stage NYHA improvement compared to 26% in the control group. These results highlight the need for better accessibility to disopyramide in regions where it is difficult to obtain.
肥厚型心肌病（HCM）的特征是左心室流出道梗阻（LVOTO），可导致症状及不良后果。丙吡胺具有负性肌力作用，常用于减轻梗阻性肥厚型心肌病（HOCM）的LVOTO。本研究评估丙吡胺对HOCM患者功能能力的影响。
2021年10月至2024年5月期间评估的有症状HOCM患者被分为两组：接受丙吡胺治疗的患者以及因无法获得丙吡胺而接受β受体阻滞剂或钙通道阻滞剂治疗的患者。治疗反应定义为纽约心脏协会（NYHA）心功能分级至少改善1级。对两组之间的临床和实验室数据进行比较，包括N末端B型利钠肽原（NT-proBNP）水平和LVOT梯度。
共纳入127例患者（中位年龄54.0岁，男性占58.2%）。79%的患者为NYHA 2级。随访后，丙吡胺组62%的患者（平均随访时长为15.2个月，平均日剂量为395 mg）功能能力至少改善1级，而对照组为26%。使用丙吡胺和存在广泛心肌延迟强化（LGE）是功能改善的独立预测因素。尽管功能有所改善，但两组之间的总体临床结局并无差异。丙吡胺相关的副作用极小，且没有患者因QT间期延长而停药。
丙吡胺显著改善了HOCM患者的功能能力，62%的患者NYHA分级改善1级，而对照组为26%。这些结果凸显了在难以获取丙吡胺的地区提高其可及性的必要性。
