Pulmonary arterial hypertension (PAH) is a chronic, progressive condition characterized by abnormally high pulmonary vascular pressure. PAH is a rare and debilitating disease associated with abnormal proliferation of smooth muscle cells in the pulmonary arterial system, causing progressive right ventricular dilation
and low cardiac output.
Coverage is subject to the specific terms of the member's benefit plan.
Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.
A diagnosis of PAH must be confirmed by a right heart catheterization. Documentation confirming the results of the right heart catheterization diagnosing PAH must be available upon request and prior to receiving any of the advanced therapy drugs listed in this policy.
A right heart catheterization may be considered medically necessary to confirm the diagnosis of PAH as evidenced by ALL of the following hemodynamic parameters:
* Pulmonary wedge pressure (PWP) may also be referred to as pulmonary arterial wedge pressure (PAWP), pulmonary capillary wedge pressure (PCWP), pulmonary venous wedge pressure (PVWP), pulmonary artery occlusion pressure (PAOP), or left ventricular end-diastolic pressure (LVEDP).
Individual consideration may be warranted by health care professionals, weighing the benefits compared to potential risks associated with use of these agents in the pediatric population when otherwise not indicated or recommended in compendia.
Epoprostenol sodium (Flolan, Veletri) continuous intravenous (IV) infusion
Epoprostenol sodium may be considered medically necessary for the treatment of PAH World Health Organization Group 1 (PAH [WHO Group I]) with documented WHO Functional Class (FC) III-IV symptoms.
Treatment with epoprostenol requires three (3) steps: initial dose-ranging, catheter insertion and portable pump attachment, and catheter and pump maintenance.
Treprostinil (Remodulin) continuous subcutaneous (SQ) or IV infusion
Treprostinil (Remodulin) may be considered medically necessary for the treatment of EITHER of the following:
Treprostinil (Tyvaso) for oral inhalation
Treprostinil (Tyvaso) may be considered medically necessary for the treatment of EITHER of the following:
Treatment with Treprostinil (Tyvaso) requires the use of a specialized dispensing device.
Iloprost (Ventavis) for inhalation via nebulizer
Iloprost (Ventavis) may be considered medically necessary for the treatment of PAH (WHO Group I) with documented WHO-FC III-IV symptoms.
Do not initiate therapy in individuals with systolic blood pressure less than 85 mmHg.
Treatment with Iloprost requires the use of a specialized dispensing device.
Sildenafil Citrate (Revatio) IV bolus injection
Sildenafil Citrate (Revatio) may be considered medically necessary for the treatment of PAH (WHO Group I) with documented WHO-FC II-III symptoms.
Combination therapy may be considered medically necessary for the treatment of PAH (WHO Group I) with documented WHO-FC II-IV symptoms when BOTH of the following conditions are met:
Use of epoprostenol, treprostinil, iloprost or sildenafil citrate is considered not medically necessary as a treatment for individuals who demonstrate a favorable acute hemodynamic response to vasodilators (vaso-responder) at cardiac catheterization who are deemed appropriate by the treating physician for a trial of calcium channel blocker treatment.
The use of epoprostenol, treprostinil, iloprost, sildenafil citrate is considered experimental/investigational and therefore non-covered due to lack of scientific based evidence for the treatment of non-PAH conditions, WHO Groups II-V, including but not limited to:
NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.
The World Health Organization (WHO) classifies pulmonary hypertension (PH) into five (5) categories based on the disease etiology. These diagnostic classifications include:
Functional assessment of patients with Pulmonary Arterial Hypertension (PAH) is based on the severity of the disease, which is classified according to the clinical symptoms on a scale of I to IV. Categorization is based on the WHO classification system, which has been modified for PAH from the New York Heart Association (NYHA) functional classifications of patients with cardiac disease.
WHO Functional Classification for Pulmonary Arterial Hypertension (PAH):
New York Heart Association (NYHA) Functional Classifications:
American College of Chest Physicians
In 2014, the American College of Chest Physicians published guidelines on pharmacologic therapy for PAH in adults.29 Relevant recommendations include:
Covered Diagnosis Codes for Procedure Codes J1325, J3285, J7686, or Q4074
Medical policies do not constitute medical advice, nor are they intended to govern the practice of medicine. They are intended to reflect BCBSND’s reimbursement and coverage guidelines. Coverage for services may vary for individual members, based on the terms of the benefit contract.