This program applies to Blue Partner, Commercial, GenPlus, NetResults A series, SourceRx and Health Insurance Marketplace formularies.

Tafamidis Prior Authorization with Quantity Limit

TARGET AGENT

Vyndaqel™ (tafamidis meglumine)

Vyndamax™ (tafamidis)

CRITERIA FOR APPROVAL

Initial Evaluation

Target Agents will be approved when ALL of the following are met:

1. ONE of the following:
   1. The patient has a diagnosis of cardiomyopathy of wild type or hereditary transthyretin-mediated amyloidosis as confirmed by radionuclide bone scintigraphy with technetium-labeled bisphosphonates or cardiac biopsy

OR

1. 2. The patient has another FDA approved indication for the requested agent  

AND

2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g. cardiologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis

AND

3. ONE of the following:
   1. The patient is NOT currently being treated with another agent in this program

OR

1. 2. The patient is currently being treated with another agent in this program AND will discontinue prior to starting the requested agent

AND

4. The patient does NOT have any FDA labeled contraindications to the requested agent

AND

5. ONE of the following:
   1. The requested quantity (dose) does not exceed the program quantity limit

OR

1. 2. ALL of the following:
      1. The requested quantity (dose) is greater than the program quantity limit

AND

1. 1. 2. The requested quantity (dose) does not exceed the maximum FDA labeled dose for the requested indication

AND

1. 1. 3. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit

Length of Approval:  12 months

Renewal Evaluation

Target Agents will be approved when ALL of the following are met:

1. The patient has been previously approved for the requested agent through the Prime Therapeutics Prior Authorization process

AND

2. The patient has had clinical benefit with the requested agent

AND

3. The prescriber is a specialist in the area of the patient’s diagnosis (e.g. cardiologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis

AND

4. ONE of the following:
   1. The patient is NOT currently being treated with another agent in this program

OR

1. 2. The patient is currently being treated with another agent in this program AND will discontinue prior to starting the requested agent

AND

5. The patient does NOT have any FDA labeled contraindications to the requested agent

AND

6. ONE of the following:

1. The requested quantity (dose) does not exceed the program quantity limit

OR

2. ALL of the following:

1. The requested quantity (dose) is greater than the program quantity limit

AND

2. The requested quantity (dose) does not exceed the maximum FDA labeled dose for the requested indication

AND

3. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit

Length of Approval:  12 months

FDA APPROVED INDICATIONS AND DOSAGE¹

CLINICAL RATIONALE

Transthyretin amyloid cardiomyopathy is a disease characterized by the accumulation of amyloid fibrils composed of misfolded transthyretin protein in the heart.  Misfolded monomers or oligomers of transthyretin are deposited in the myocardium, leading to cardiomyopathy and symptoms of heart failure.  Infiltration of the conduction system can lead to bundle-branch block, atrioventricular block, sinoatrial disease, and atrial fibrillation.  Transthyretin amyloid cardiomyopathy is a late-onset disease; symptoms are predominately manifested in male patients 60 years of age or older.  The condition can be inherited as an autosomal dominant trait caused by pathogenic mutations in the transthyretin gene TTR (ATTRm) or by the deposition of wild-type transthyretin protein (ATTRwt).  There are more than 120 pathogenic mutations in TTR that result in a variable phenotypic presentation.  The prevalence of ATTRwt is uncertain, some studies have reported a prevalence of 13% among patients with heart failure with a preserved ejection fraction, 16% among patients undergoing transcatheter aortic-valve replacement for severe aortic stenosis, and 5% among patients with presumed hypertrophic cardiomyopathy.  Treatments have previously been limited to supportive care.  Median survival in untreated patients is reported to be 2.5 years after diagnosis for ATTRm caused by the TTR Val122Ile mutation and 3.6 years for ATTRwt.  Death in most patients is from cardiac causes, including sudden death and heart failure.² Diagnosis of cardiac amyloidosis (CA) is performed through radionuclide bone scintigraphy with technetium-labeled bisphosphonates or cardiac biopsy.^3,4

Tafamidis is a selective stabilizer of TTR.  Tafamidis binds to TTR at the thyroxine binding sites, stabilizing the tetramer and slowing dissociation into monomers, the rate-limiting step in the amyloidogenic process.  Efficacy was demonstrated in a multicenter, international, randomized, double-blind, placebo-controlled study in 441 patients with wild type or hereditary ATTR-CM.  Patients were randomized in a 1:2:2 ration to receive Vyndaqel 20 mg, Vyndaqel 80 mg, or placebo once daily for 30 months, in addition to standard of care (e.g. diuretics).  Treatment assignment was stratified by the presence or absence of a variant TTR genotype as well as baseline disease severity (NYHA Class).  The primary analysis points were all-cause mortality and frequency of cardiovascular-related hospitalizations.  The analysis demonstrated a significant reduction in all-cause mortality and frequency of cardiovascular-related hospitalizations in the pooled Vyndaqel group.¹ 

References

1. Vyndaqel and Vyndamax Prescribing.  Pfizer, Inc. 05/2019.
2. Maurer MS, Schwartz JH, Gundapeneni BG, et al.  Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy.  NEJM 2018; 379: 1007-16.
3. Gillmore JD, Maurer MS, Falk RH, et al.  Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis.  Circulation. 2016;133:2404-2412.
4. Donnelly, JP, Hanna M.  Cardiac amyloidosis: An update on diagnosis and treatment.  Cleveland Clinic Journal of Medicine.  2017;84(3):12-26. 

This pharmacy policy is not an authorization, certification, explanation of benefits or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All pharmacy policies are based on (i) information in FDA approved package inserts (and black box warning, alerts, or other information disseminated by the FDA as applicable); (ii) research of current medical and pharmacy literature; and/or (iii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.
 
The purpose of pharmacy policies are to provide a guide to coverage. Pharmacy policies are not intended to dictate to physicians how to practice medicine. Physicians should exercise their medical judgment in providing the care they feel is most appropriate for their patients.
 
Neither this policy, nor the successful adjudication of a pharmacy claim, is guarantee of payment.

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