Camzyos (mavacamten) Form

Mavacamten (Camzyos®) is a cardiac myosin inhibitor. FDA Approved Indication(s) Camzyos 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. Policy/Criteria Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria.
It is the policy of health plans affiliated with Centene Corporation® that Camzyos is medically necessary when the following criteria are met:
I. Initial Approval Criteria
A. Obstructive Hypertrophic Cardiomyopathy (must meet all):

1. Diagnosis of obstructive HCM;
   2. Member exhibits NYHA Class II to III symptoms, including but not limited to: effort- related dyspnea or chest pain, or syncope or near syncope attributed to left ventricular outflow tract obstruction;
2. Prescribed by or in consultation with a cardiologist;
   
   4. Age ≥ 18 years;
   5. Member has left ventricular hypertrophy with maximal left ventricular wall thickness of one of the following (a or b): a. ≥ 15 mm; b. ≥ 13 mm to < 15 mm if member has familial hypertrophic cardiomyopathy or in conjunction with a positive genetic test;
3. Member has a left ventricular ejection fraction (LVEF) ≥ 55%;
   7. Member has a peak left ventricular outflow tract (LVOT) gradient ≥ 50 mmHg at rest or with provocation;

4. Failure of two of the following at up to maximally indicated doses, unless clinically significant adverse effects are experienced or all are contraindicated (a, b, and c): a. Non-vasodilating beta-blocker (e.g., atenolol, metoprolol, bisoprolol, propranolol); b. Non-dihydropyridine calcium channel blocker (e.g., verapamil, diltiazem); Page 1 of 7

   CLINICAL POLICY Mavacamten c. Add-on disopyramide therapy after failure of beta-blocker or calcium channel blocker monotherapy;

5. Dose does not exceed 15 mg per day. Approval duration: 6 months
   B. Other diagnoses/indications (must meet 1 or 2):
6. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or
7. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid.
   II. Continued Therapy A. Obstructive Hypertrophic Cardiomyopathy (must meet all):
8. Member meets one of the following (a or b): a. Currently receiving medication via Centene benefit or member has previously met initial approval criteria; b. Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations (refer to state specific addendums for CC.PHARM.03A and CC.PHARM.03B);
9. Member is responding positively to therapy as evidenced by improvement in obstructive HCM symptoms;
10. Member has not undergone a septal reduction procedure within the last 6 months;
    4. If request is for a dose increase, new dose does not exceed 15 mg per day. Approval duration: 12 months
       B. Other diagnoses/indications (must meet 1 or 2):

11. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or Page 2 of 7

    CLINICAL POLICY Mavacamten b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

12. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid.
    III. Diagnoses/Indications for which coverage is NOT authorized:
    A. Non-FDA approved indications, which are not addressed in this policy, unless there is sufficient documentation of efficacy and safety according to the off label use policies – CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid, or evidence of coverage documents. IV. Appendices/General Information Appendix A: Abbreviation/Acronym Key ER: extended release FDA: Food and Drug Administration HCM: hypertrophic cardiomyopathy IR: immediate release LVEF: left ventricular ejection fraction LVOT: left ventricular outflow tract NYHA: New York Heart Association REMS: Risk Evaluation and Mitigation Strategy Appendix B: Therapeutic Alternatives
    This table provides a listing of preferred alternative therapy recommended in the approval criteria. The drugs listed here may not be a formulary agent for all relevant lines of business and may require prior authorization.
    Dosing Regimen Drug Name atenolol metoprolol bisoprolol propranolol nadolol verapamil diltiazem Dose Limit/ Maximum Dose 200 mg/day 50-100 mg PO QD 400 mg/day 50-100 mg PO QD 20 mg/day 5-20 mg PO QD 80-320 mg PO QD or divided into 2-4 doses/day 320 mg/day 240 mg/day 40-80 mg PO QD 480 mg/day 80-120 mg PO TID IR: 360 mg/day Immediate-release (IR): 30 mg PO QID ER: 360-540 mg/day Extended-release (ER): 120-180 mg PO QD 600 mg/day 200-250 mg PO BID disopyramide Therapeutic alternatives are listed as Brand name® (generic) when the drug is available by brand name only and generic (Brand name®) when the drug is available by both brand and generic. Appendix C: Contraindications/Boxed Warnings • Contraindication(s): concomitant use of moderate to strong CYP2C19 inhibitors/inducers or strong CYP3A4 inhibitors of moderate to strong CYP3A4 inducers • Boxed warning(s): risk of heart failure due to systolic dysfunction
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    CLINICAL POLICY Mavacamten o Echocardiogram assessments of LVEF are required prior to and during treatment with Camzyos; initiation of Camzyos in patients with LVEF < 55% is not recommended; interrupt Camzyos if LVEF is < 50% at any visit or if the patient experiences heart failure symptoms or worsening clinical status; because of the risk of heart failure due to systolic dysfunction, Camzyos is available only through a restricted program under a Risk Evaluation and Mitigation Strategy (REMS) called Camzyos REMS Program V. Dosage and Administration Indication Obstructive HCM Dosing Regimen Initiation: 5 mg PO QD x 4 weeks
    Maximum Dose 15 mg/day Week 4:
    • If Valsalva LVOT gradient is < 20 mmHg, down-titrate to 2.5 mg PO QD • If Valsalva LVOT gradient is ≥ 20 mmHg, maintain 5 mg daily dose Week 8: • If Valsalva LVOT gradient is ≥ 20 mmHg, maintain current dose x 4 weeks and then begin Maintenance therapy at Week 12 • If Valsalva LVOT gradient is < 20 mmHg and previous dose was 2.5 mg daily: withhold drug and return at Week 12 o At Week 12, restart on 2.5 mg daily dose if LVEF ≥ 50% and recheck clinical status and echocardiogram in 4 weeks o Maintain same dose x 8 weeks, consistent with Maintenance dosing, unless LVEF is < 50% • If Valsalva LVOT gradient is < 20 mmHg and previous dose was 5 mg daily: down- titrate to 2.5 mg PO QD x 4 weeks and then begin Maintenance therapy Maintenance: • If LVEF is < 50%: interrupt Camzyos treatment (see instructions for dose interruption below) • If LVEF is 50-55%, regardless of Valsalva LVOT gradient OR LVEF is > 55% and Valsalva LVOT gradient is < 30 mmHg: maintain on the same dose and follow-up 12 weeks later • If LVEF ≥ 55% and Valsalva LVOT gradient ≥ 30 mmHg: Up-titration to next Page 4 of 7

    CLINICAL POLICY Mavacamten Indication Dosing Regimen Maximum Dose higher daily (mg) dose level (2.5  5; 5  10; 10  15); recheck clinical status and echocardiogram in 4 weeks and maintain the same dose for the next 8 weeks unless LVEF is < 50%; further up-titration is allowed after 12 weeks of treatment on the same dose level Dose interruption at any clinic visit if LVEF is < 50%: • After dose interruption, recheck echocardiogram parameters every 4 weeks until LVEF ≥ 50%; once LVEF ≥ 50%: o Restart treatment at next lower daily (mg) dose level (5  2.5; 10  5; 15  10; if interrupted at 2.5 mg, restart at 2.5 mg) o Recheck clinical status and echocardiogram in 4 weeks and maintain the same dose for the next 8 weeks unless LVEF < 50%; o Next follow instructions above for Maintenance dosing • Permanently discontinue Camzyos treatment if LVEF is < 50% twice on 2.5 mg daily dose. VI. Product Availability
    Capsules: 2.5 mg, 5 mg, 10 mg, 15 mg VII.