CAMZYOS is a drug used to treat obstructive hypertrophic cardiomyopathy. Its main function is to reduce the number of myosin heads that can effectively interact with actin, thereby reducing left ventricular outflow tract (LVOT) obstruction and left ventricular filling pressure by reducing sarcomere hyperactivity.

Dosage and Administration of CAMZYOS

1. Initiation, maintenance and interruption of treatment

(1) Confirm that females of childbearing potential are not pregnant and are using effective contraceptive measures.

(2) Concomitant administration of weak CYP2C19 or moderate CYP3A4 inhibitors.

(3) The recommended starting dose is 5 mg, taken orally once daily, regardless of food; the permitted subsequent doses are 2.5 mg, 5 mg, 10 mg or 15 mg taken orally once daily; the maximum recommended dose is 15 mg, taken orally once daily.

(4) Patients may develop heart failure while taking malvaketamine, and regular assessment of left ventricular ejection fraction (LVEF) and left ventricular outflow tract (LVOT) gradient is required to administer the drug with caution, achieve an appropriate target Valsalva LVOT gradient, maintain LVEF ≥ 50% and avoid symptoms of heart failure.

(5) Daily dosing requires several weeks to achieve steady-state drug levels and therapeutic effect, and genetic variations in metabolism and drug interactions can lead to large differences in exposure.

(6) When initiating or titrating malvaketamine, first consider left ventricular ejection fraction (LVEF), then consider Valsalva LVOT gradient and patient clinical status to guide appropriate malvaketamine dosing.

(7) If left ventricular ejection fraction (LVEF) is < 50% while taking malvaketamine, interrupt treatment, follow the interruption, restart or discontinuation of malvaketamine, if interrupted at 2.5 mg, either restart at 2.5 mg or permanently discontinue.

The delayed dose may be increased if there is a concurrent illness that could impair cardiac systolic function (such as a serious infection) or arrhythmias (such as atrial or other uncontrolled tachyarrhythmias); consider interrupting mavaketide therapy in patients with concomitant illnesses.

2. Missed or Delayed Doses

If a dose is missed, it should be taken as soon as possible and the next scheduled dose should be taken at the usual time the next day. The exact time of daytime dosing is not important, but two doses should not be taken on the same day. Swallow the capsule whole; do not break, open, or chew it.

3. Concomitant Use of Weak CYP2C19 or Moderate CYP3A4 Inhibitors

1. For patients on stable therapy with a weak CYP2C19 inhibitor or a moderate CYP3A4 inhibitor, the recommended starting dose for mavaketide is 5 mg once daily.

2. Reduce the dose of CAMZYOS by one level (i.e., 15 mg → 10 mg; 10 mg → 5 mg; 5 mg → 2.5 mg) in patients initiating a weak CYP2C19 inhibitor or a moderate CYP3A4 inhibitor. Schedule clinical and echocardiographic evaluations 4 weeks after initiation of the inhibitor, and do not increase the dose of CAMZYOS until 12 weeks after initiation of the inhibitor.

3. Avoid concomitant use of weak CYP2C19 and moderate CYP3A4 inhibitors in patients stable on 2.5 mg of CAMZYOS, as a lower once-daily dose is not available.