Listing of Pharmaceutical Benefits (NHL) - Schedule 4 part 1

Symptomatic obstructive hypertrophic cardiomyopathy

First continuing treatment (until at least 6 months on optimal dose is achieved)

Patient must have previously received PBS-subsidised treatment with this drug for this condition under the initial treatment restriction; AND

Patient must be undergoing concomitant treatment with at least one of: (i) a beta-blocker (ii) non-dihydropyridine calcium channel blocker, unless at least one of the following is present: (a) a contraindication to beta-blocker and/or non-dihydropyridine calcium channel blocker therapy as listed in the TGA approved Product Information; (b) an intolerance to beta-blocker and/or non-dihydropyridine calcium channel blocker therapy; AND

Patient must have a current left ventricular ejection fraction (LVEF) of no less than 50%; AND

Patient must be titrating mavacamten treatment until optimal dose is achieved; or

Patient must be continuing mavacamten treatment to reach at least 6 months on the optimal dose prior to assessing the response; AND

Must be treated by a cardiologist; or

Must be treated by a consultant physician with experience in the management of hypertrophic cardiomyopathy.

The assessment of response must be conducted after at least 6 months on optimal dose to determine the patient's eligibility for maintenance treatment. Where an assessment is not undertaken, the patient will not be eligible for ongoing treatment. This treatment phase listing intends to provide up to 36 weeks of treatment in 3 treatment courses.

For the purposes of this restriction, an adequate response to treatment is defined as: an improvement in at least one of the following: (i) symptoms, (ii) quality of life, (iii) exercise capacity, (iv) peak left ventricular outflow tract (LVOT) gradient.