Real-world effectiveness, response patterns and clinical implementation of Mavacamten in obstructive hypertrophic cardiomyopathy: Insights from the SWISS-MAVA cohort.

Authors

Niccolò Maurizi, Dennis Jensen, Annina S Vischer, Simon F Stämpfli, Pierre Monney, Christiane Gruner

Published in

International journal of cardiology. Pages 134689. Jul 25, 2026. Epub Jul 25, 2026.

Abstract

Mavacamten has demonstrated efficacy and safety in randomized trials for the treatment of symptomatic obstructive hypertrophic cardiomyopathy (oHCM), but detailed real-world data in clinically complex populations remain limited.
We evaluated the effectiveness, response patterns, safety and implementation challenges of mavacamten in a multicenter cohort (SWISS-MAVA) of adult patients with oHCM treated without CYP2C19 genotyping. The primary outcome was reduction of provocable (Valsalva) left ventricular outflow tract gradient (LVOTGval) ≤ 30 mmHg, or LVOTGval≤50 mmHg with ≥1 NYHA class improvement at last follow-up.
Eighty-two patients were included (age 59.5 ± 13.4 years, 63% male) and followed for 52.4 ± 21.4 weeks. Mavacamten reduced LVOTGval from 82[62 to 112] to 20[6 to 25] mmHg, NT-proBNP from 537[273-1279] to 142[77-303] ng/L, E/e' from 14.5 ± 6.6 to 10.8 ± 4.5 and left atrial volume indexed from 44.7 ± 12.5 to 37.2 ± 11.2 mL/m² (p < 0.001). Mean left ventricular ejection fraction (LVEF) decreased (66.7 ± 5.4% to 62.6 ± 5.8%, (p < 0.001)) but remained preserved overall. The primary endpoint was achieved in 74/82 patients (90%), with 62/82 (76%) responding within 6 months. Twelve patients (15%) showed a super-hemodynamic response requiring protocol-mandated dose reduction, prolonging titration by 3.5 ± 2 months. Transient LVEF<50% occurred in 5 patients (6%; 5.9/100 patient-years), all reversible after treatment interruption; in 4/5 cases, a potential cofactor was identified. New-onset atrial fibrillation occurred in 6 patients (7.3%; 7.1/100 patient-years). Weight loss was observed in some obese/overweight responders, with transitions to lower BMI categories.
These data support the effectiveness and safety of mavacamten in real-world practice while highlighting clinically relevant heterogeneity in response patterns, titration burden, and adverse-event susceptibility.

PMID:
42501898
Bibliographic data and abstract were imported from PubMed on 26 Jul 2026.

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