Back to Search Start Over

Safety And Efficacy Of Mavacamten In Adults Wth Hypertrophic Cardiomyopathy With Obstruction. Insights From A Tertiary Center.

Safety And Efficacy Of Mavacamten In Adults Wth Hypertrophic Cardiomyopathy With Obstruction. Insights From A Tertiary Center.

Authors :
Konstantinidis, Ippokratis
Bihag, Zade
Friligkou, Eleni
Burke-Martindale, Carolyn
Smith, Emily
Patel, Nirav
Weissler-Snir, Adaya
Jaiswal, Abhishek
Source :
Journal of Cardiac Failure; Jan2025, Vol. 31 Issue 1, p253-253, 1p
Publication Year :
2025

Abstract

In controlled studies, Mavacamten improved exercise capacity with minimal adverse effects in patients with symptomatic hypertrophic cardiomyopathy with obstruction (HOCM). We describe our experience from a cohort of adults treated with mavacamten in a hypertrophic cardiomyopathy center in the United States. We reviewed 26 consecutive adult patients with HOCM and peak left ventricular outflow gradients (LVOT) of ≥ 50 mmHg. We compared baseline characteristics and echocardiographic parameters, including ejection fraction (EF) and LVOT gradients at rest and during Valsalva. LVOT gradients at baseline and 4, 8, and 12 weeks were compared using Friedman's test, and EFs were compared using repeated measures ANOVA in similar timeframes. The mean age was 61.8 years; 53% were female, 88% were white, 61% were obese (average BMI 32 kg/m<superscript>2</superscript>), and 77% had hypertension. MYPBC3 and MYH7 pathogenic variants were identified in four and one patient, respectively. Most (84%) were treated with metoprolol succinate with an average dose of 79 mg, and 7% with calcium channel blockers before mavacamten. The starting dose of mavacamten per protocol was 5 mg daily. Compared to the baseline median LVOT gradient measured at rest and during Valsalva, there was a statistically significant reduction of 35.6 mmHg and 43 mmHg at four weeks, 30.8 mmHg and 37.2 mmHg at eight weeks and 27 mmHg and 32.2 mmHg at 12 weeks, respectively (p rest =0.00001, p valsalva =0.0001). Of note, 13 patients required dose reduction due to rapid decline in the LVOT gradients as per protocol, and two out of those 13 required temporary discontinuation due to persistence of the low LVOT gradients. One patient had persistently high gradients despite being maintained on a max dose of 15 mg daily. Compared to the baseline, there was a significant decrease in mean LVEF by 5.34% at four weeks, 5.45% at eight weeks, and 2.84% at 12 weeks (p=0.0002). No patients experienced significant adverse effects, including a reduction in EF to less than 50%, symptomatic heart failure, arrhythmia, syncope, or death. Twenty patients reported improvement in NYHA class by ≥1 class. Our clinical experience shows that treatment with mavacamten was highly effective at reducing LVOT gradients and alleviating symptoms without increased adverse effects. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
10719164
Volume :
31
Issue :
1
Database :
Supplemental Index
Journal :
Journal of Cardiac Failure
Publication Type :
Academic Journal
Accession number :
182054679
Full Text :
https://doi.org/10.1016/j.cardfail.2024.10.186