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Advanced drug therapy for pulmonary hypertension: epoprostenol (generic, Flolan® and Veletri®), treprostinil (Remodulin® & Tyvaso®), iloprost (Ventavis®) and sildenafil injection (Revatio®)

These services may or may not be covered by all CoOportunity Health plans. Please see your plan documents for your own coverage information. If there is a difference between this general information and your plan documents, your plan will be used to determine your coverage.

Administrative process

Advanced drug therapies for pulmonary hypertension will not be covered without prior authorization from pharmacy administration.


Advanced drug therapy is generally covered for patients with a diagnosis of idiopathic pulmonary arterial hypertension (or primary pulmonary hypertension or WHO Group 1 classification) and when prescribed by a pulmonologist or cardiologist.

Utilization for all other indications will be reviewed for medical necessity.

Combination use requires documentation of medical necessity (including response to single-drug therapy, and rationale for adding a medication).

Use of sildenafil injection requires verification of medical necessity for the parenteral formulation.


Epoprostenol (Flolan, Veletri and generic): is FDA-approved for the long-term intravenous treatment of primary pulmonary hypertension and pulmonary hypertension associated with the scleroderma spectrum of disease in NYHA Class III and Class IV patients who do not respond adequately to conventional therapy.

Treprostinil (Remodulin): is FDA-approved for

  1. Pulmonary Arterial Hypertension in Patients with NYHA Class II-IV Symptoms, to diminish symptoms associated with exercise. It may be administered as a continuous subcutaneous infusion or continuous intravenous infusion; however, because of the risks associated with chronic indwelling central venous catheters, including serious blood stream infections, continuous intravenous infusion should be reserved for patients who are intolerant of the subcutaneous route, or in whom these risks are considered warranted.
  2. Pulmonary Arterial Hypertension in Patients Requiring Transition from Flolan, to diminish the rate of clinical deterioration. The risks and benefits of each drug should be carefully considered prior to transition.

Treprostinil inhaled (Tyvaso): is FDA-approved to increase walk distance in patients with WHO Group I pulmonary arterial hypertension and NYHA Class III symptoms. The effects diminish over the minimum recommended dosing interval of 4 hours; treatment timing can be adjusted for planned activities. While there are long-term data on use of treprostinil by other routes of administration, nearly all controlled clinical experience with inhaled treprostinil has been on a background of bosentan or sildenafil. The controlled clinical experience was limited to 12 weeks in duration.

Iloprost inhaled (Ventavis): is FDA-approved for the treatment of pulmonary arterial hypertension (WHO Group I) in patients with NYHA Class III or IV symptoms. In controlled trials, it improved a composite endpoint consisting of exercise tolerance, symptoms (NYHA Class), and lack of deterioration.

Sildenafil injection (Revatio):  is a phosphodiesterase 5 (PDE5) inhibitor FDA-approved for the treatment of pulmonary arterial hypertension (WHO Group I) to improve exercise ability and delay clinical worsening. Studies establishing effectiveness included predominately patients with NYHA Functional Class II-III symptoms and etiologies of primary pulmonary hypertension or pulmonary hypertension associated with connective tissue disease. The efficacy of Revatio has not been adequately evaluated in patients taking bosentan concurrently.


Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure and subsequent right ventricular failure. It is diagnosed by right heart catheterization measurements and defined by a mean pulmonary artery pressure of ≥25mmHg at rest.

Pulmonary hypertension may occur as a result of idiopathic processes, genetic predisposition or other disease processes such as heart failure and COPD.

Treatment of pulmonary hypertension is directed at correcting underlying causes such as in the case where PH occurs as a result of another disease process. Prostaglandin therapy is considered advanced therapy in that it is not used to correct underlying causes but rather it is used to control pulmonary blood pressure itself.

The World Health Organization (WHO) classifies pulmonary hypertension into five groups based on the etiology of disease (partial listing):5,6

  1. Pulmonary arterial hypertension (PAH)
    1. Idiopathic PAH
    2. Heritable
  2. Pulmonary hypertension owing to left heart disease
  3. Pulmonary hypertension owing to lung diseases
    1. Chronic obstructive pulmonary disease
    2. Interstitial lung disease
  4. Chronic thromboembolic pulmonary hypertension
  5. Pulmonary hypertension with unclear multifactorial mechanisms

If available, codes are listed below for informational purposes only, and do not guarantee member coverage or provider reimbursement. The list may not be all-inclusive.


J1325 – Injection, epoprostenol, 0.5mg
S0155 – Sterile diluents for epoprostenol, 50 mL (Not used with Veletri)
S9347 – Home infusion therapy, uninterrupted, long term, controlled rate intravenous or
subcutaneous infusion therapy (e.g., epoprostenol); administrative services,
professional pharmacy services, care coordination, and all necessary supplies
and equipment (drugs and nursing visits coded separately), per diem
J3285 – Injection, treprostinil, 1 mg
J7686 – Treprostinil, inhalation solution, FDA-approved final product, non-compounded,
administered through DME, unit dose form, 1.74 mg
Q4074 – Iloprost, inhalation solution, FDA-approved final product, non-compounded,
administered through DME, unit dose form, up to 20 mcg (Please note:
AWP/WAC pricing is the same for the 10 mcg and 20 mcg unit dose vials.
Therefore bill/reimburse 1 unit of the code regardless of strength used)
J3490 – Unclassified drugs - Revatio 10 mg/12.5mL solution

NDC Codes

00703-1985-01 – epoprostenol sodium 0.5 mg solution
00703-1995-01 – epoprostenol sodium 1.5 mg solution
00173-0517-00 – Flolan 0.5 mg solution
00173-0519-00 – Flolan 1.5 mg solution
66215-0401-01 – Veletri 1.5 mg solution
66302-0101-01 – Remodulin 1 mg/mL 20 mL solution
66302-0110-01 – Remodulin 10 mg/mL 20 mL solution
66302-0102-01 – Remodulin 2.5 mg/mL 20 mL solution
66302-0105-01 – Remodulin 5 mg/mL 20 mL solution
66302-0206-03 – Tyvaso 0.6 mg/mL single use solution x 4
66302-0206-02 – Tyvaso refill 0.6 mg/mL single use solution x 28
66302-0206-01 – Tyvaso starter 0.6 mg/mL single use solution x 28
66215-0302-00 – Ventavis 10 mcg/mL solution
66215-0302-30 - Ventavis 10 mcg/mL solution x 30
66215-0303-00 - Ventavis 20 mcg/mL solution
66215-0303-30 - Ventavis 20 mcg/mL solution x 30
00069-0338-01 – Revatio 10 mg/12.5 mL solution

CPT Copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.


  1. Flolan PI,
  2. Remodulin PI,
  3. Tyvaso PI,
  4. Ventavis PI,
  5. Revatio PI,
  6. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. McLaughlin VV, et al; American College of Cardiology Foundation Task Force on Expert Consensus Documents; American Heart Association; American College of Chest Physicians; American Thoracic Society, Inc; Pulmonary Hypertension Association. J Am Coll Cardiol. 2009 Apr 28;53(17):1573-619.
  7. Guidelines for the diagnosis and treatment of pulmonary hypertension. Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC); European Respiratory Society (ERS); International Society of Heart and Lung Transplantation (ISHLT), Galiè N, et al. Eur Respir J. 2009 Dec;34(6):1219-63.

This information is for most, but not all, CoOportunity Health plans. Please read your plan documents to see if your plan has limits or will not cover some items. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage.

CoOportunity Health has contracted with HealthPartners Administrators, Inc. to provide claims processing, medical management and certain other administrative services.