Heart failure & cardiomyopathiesCohort study
Effect of mavacamten on sudden cardiac death risk scores in obstructive hypertrophic cardiomyopathy
- retrospective
- consecutive patients
- four French referral centres for cardiomyopathies
Authors' conclusion
Mavacamten therapy was associated with marked haemodynamic and symptomatic improvements and a reduction in the ESC-based SCD risk in obstructive HCM. While these findings need to be confirmed and were not paralleled by a reduction in arrhythmic events, they suggest that myosin inhibition may influence arrhythmic risk stratification. Prospective studies with longer follow-up are required to determine whether such favourable remodelling translates into true arrhythmic protection.
Study context
- Population
- Consecutive patients with symptomatic obstructive hypertrophic cardiomyopathy (oHCM) treated with mavacamten between October 2023 and June 2025included among 186 eligible patients
- Participants
- 161 patients
- Exposure
- European Society of Cardiology (ESC) HCM Risk-SCD score and clinical parameters at baseline vs after 6 months of mavacamten
- mavacamtenn = 161
Main finding
As reported in the abstract
high
6%
intermediate
9%
low
85%
Observational data: associations, not effects.
Further findings
NYHA class III · 6 months
- NYHA class III, baseline to 6 months
- 35 to 2%
- P < .001
Resting LV outflow tract (LVOT) gradient · 6 months
- baseline to 6 months
- 45 ± 31 to 12 ± 9 mmHg
- P < .001
Provoked gradient · 6 months
- baseline to 6 months
- 80 ± 32 to 25 ± 19 mmHg
- P < .001
NT-proBNP levels · 6 months
- significant improvement
Median expected 5-year sudden cardiac death (SCD) risk · 6 months
- decreased from
- 2.1% (1.7-3) to 1.4% (1.1-1.8)
- P < .001
5-year SCD risk score in high-risk patients · 6 months
- decreased from
- 7.7% (7.4-8.4) to 4.6% (4.5-6.0)
- P = .001
Changes in modifiable ESC Risk-SCD parameters · 6 months
- rest/Valsalva LVOT gradients
- −33/−57 mmHg
- left atrial diameter
- −1.2 mm
- septal thickness
- −1.5 mm
- all P < .001
- ESC Risk-SCD reduction entirely attributable to modifiable parameters
Mean HCM Risk Score reduction · 6 months
- mean reduction
- 0.7%
- heterogeneous inter-patient response
- homogeneous efficacy across centres (P = .206)
Proportion with high rhythmic risk considering late gadolinium enhancement (LGE) · 6 months
- fell from
- 16% to 15%
Safety
SCD, secondary prevention ICD, NSVT · median follow-up of 19 months
- new non-sustained ventricular tachycardia episodes
- two
- no patient experienced SCD or required secondary prevention implantable cardioverter-defibrillator
Original publication
Summarised from the published abstract; every number is checked against it