Description
Beacon Pharmaceuticals PLC
Indications
Camzoy is indicated for the treatment of adults with symptomatic New York Heart Association (NYHA) class II-III obstructive hypertrophic cardiomyopathy (HCM) to improve functional capacity and symptoms.
Pharmacology
Mavacamten is an allosteric and reversible inhibitor selective for cardiac myosin. Mavacamten modulates the number of myosin heads that can enter “on actin” (power-generating) states, thus reducing the probability of force-producing (systolic) and residual (diastolic) cross-bridge formation. Excess myosin actin cross-bridge formation and dysregulation of the super-relaxed state are mechanistic hallmarks of HCM. Mavacamten
shifts the overall myosin population towards an energy-sparing, recruitable, super-relaxed state. In HCM patients, myosin inhibition with mavacamten reduces dynamic LVOT obstruction and improves cardiac filling pressures.
shifts the overall myosin population towards an energy-sparing, recruitable, super-relaxed state. In HCM patients, myosin inhibition with mavacamten reduces dynamic LVOT obstruction and improves cardiac filling pressures.
Dosage & Administration
The recommended starting dose is 5 mg orally once daily without regard to food; allowable subsequent doses with titration are 2.5 mg, 5 mg, 10 mg, or 15 mg orally once daily. The maximum recommended dose is 15 mg orally once daily.
Patients may develop heart failure while taking Mavacamten. Regular LVEF and Valsalva left ventricular outflow tract (LVOT) gradient assessment is required for careful titration to achieve an appropriate target Valsalva LVOT gradient, while maintaining LVEF ≥50% and avoiding heart failure symptoms.
Daily dosing takes weeks to reach steady-state drug levels and therapeutic effects, and genetic variation in metabolism and drug interactions can cause large differences in exposure.
When initiating or titrating Mavacamten, first consider LVEF then consider the Valsalva LVOT gradient and patient clinical status to guide appropriate Mavacamten dosing. Follow the algorithms for Initiation and Maintenance for appropriate Mavacamten dosing and monitoring schedules.
If LVEF <50% while taking Mavacamten, interrupt treatment. Follow the algorithm for Interruption for guidance on interrupting, restarting, or discontinuing Mavacamten. If interrupted at 2.5 mg, either restart at 2.5 mg or discontinue permanently.
Pediatric Use: The safety and effectiveness of Mavacamten have not been established in pediatric patients.
Patients may develop heart failure while taking Mavacamten. Regular LVEF and Valsalva left ventricular outflow tract (LVOT) gradient assessment is required for careful titration to achieve an appropriate target Valsalva LVOT gradient, while maintaining LVEF ≥50% and avoiding heart failure symptoms.
Daily dosing takes weeks to reach steady-state drug levels and therapeutic effects, and genetic variation in metabolism and drug interactions can cause large differences in exposure.
When initiating or titrating Mavacamten, first consider LVEF then consider the Valsalva LVOT gradient and patient clinical status to guide appropriate Mavacamten dosing. Follow the algorithms for Initiation and Maintenance for appropriate Mavacamten dosing and monitoring schedules.
If LVEF <50% while taking Mavacamten, interrupt treatment. Follow the algorithm for Interruption for guidance on interrupting, restarting, or discontinuing Mavacamten. If interrupted at 2.5 mg, either restart at 2.5 mg or discontinue permanently.
Pediatric Use: The safety and effectiveness of Mavacamten have not been established in pediatric patients.
Reviews
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