Tyvaso — Blue Cross Blue Shield of Oklahoma

Initial criteria

• ONE of the following:
• A. Continuation of therapy: prescriber states the patient has been treated with the requested agent (not samples) within past 90 days AND at risk if therapy is changed, AND patient has an FDA labeled indication for the requested agent and route of administration.
• B. Chronic thromboembolic pulmonary hypertension (CTEPH, WHO Group 4): requested agent is Adempas; diagnosis confirmed by ventilation-perfusion scan and selective pulmonary angiography; mean pulmonary artery pressure >20 mmHg; pulmonary capillary wedge pressure ≤15 mmHg; pulmonary vascular resistance >2 Wood units; ONE of: patient not a candidate for surgery OR has had pulmonary endarterectomy and has persistent/recurrent disease; patient will NOT use requested agent with a PDE5 inhibitor (e.g., tadalafil, sildenafil).
• C. Pulmonary arterial hypertension (PAH, WHO Group 1): diagnosis confirmed by right heart catheterization; mean pulmonary artery pressure >20 mmHg; pulmonary capillary wedge pressure ≤15 mmHg; pulmonary vascular resistance >2 Wood units; if Winrevair, patient is not pregnant or planning pregnancy; if PDE5 inhibitor or Adempas type agent, not used in combination with another PDE5 inhibitor; ONE of the following therapy structures:
•   • Monotherapy with a non‑Winrevair agent; OR
•   • Dual therapy: one agent from two classes (ERA, PDE5i, prostanoid) OR ERA + sGC (Adempas) after unacceptable response to ERA + PDE5i; OR
•   • Triple therapy: ERA + PDE5i + prostanoid for high‑risk or WHO IV; OR ERA + PDE5i + Winrevair/prostanoid after inadequate response to ≥2 PAH classes; OR
•   • Quadruple therapy: patient high risk or WHO IV; 4 agents from different classes; inadequate response to ≥3 PAH classes; AND ONE of: prostanoid started, OR intolerance/contraindication/hypersensitivity to all prostanoids.
• D. Pulmonary hypertension with interstitial lung disease (PH‑ILD, WHO Group 3): requested agent Tyvaso or Yutrepia; diagnosis confirmed by right heart catheterization; mean pulmonary artery pressure >20 mmHg; pulmonary capillary wedge pressure ≤ 15 mmHg; pulmonary vascular resistance > 2 Wood units; PH‑ILD assessed using risk factors, trajectory, imaging, etc.; extensive parenchymal changes on CT; BOTH patient currently treated with and will continue standard ILD therapy (e.g., Ofev).
• E. Raynaud’s phenomenon: requested agent Adcirca or Revatio; not concurrently taking oral erectile‑dysfunction agent (Cialis, Levitra, Viagra) or guanylate‑cyclase stimulator (Adempas); ONE of: tried dihydropyridine calcium‑channel blocker, OR intolerance/hypersensitivity, OR FDA contraindication to all such agents.
• F. Other FDA labeled indication for requested agent and route of administration.
• If FDA labeled indication: patient’s age within labeling OR supported off‑label use for age.
• If request is for branded form (Revatio tablet, Adcirca, Tadliq, Tracleer, Letairis, Revatio oral suspension, Liqrev): ONE of the following—use in stage‑4 metastatic cancer per criteria; stable on brand; inadequate response or adverse event with corresponding generic; intolerance/hypersensitivity; contraindication; expected ineffectiveness or barrier to adherence; medical necessity; prior different drug in class discontinued due to lack of efficacy; or documented support for brand use. Chart notes required as indicated.
• Prescriber is a specialist (e.g., cardiologist, pulmonologist) or has consulted one.
• Patient does NOT have FDA labeled contraindications to requested agent.

Approval duration

12 months