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Pharmacologic Treatment of Pulmonary Arterial Hypertension

Section: Injections
Effective Date: May 10, 2019
Revised Date: May 10, 2019

Description

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. 

Criteria

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: 

  • Mean pulmonary artery pressure (mPAP) greater than or equal to 25 mmHg; and 
  • Pulmonary wedge pressure (PWP)* less than or equal to 15 mmHg; and 
  • Pulmonary vascular resistance (PVR) greater than 250 dynes-sec/cm or equal to 3 Wood units. 

* 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). 

Note:

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. 

Procedure Codes 

93451 J1325 J3285 J3490 J7686
K0455 K0730 Q4074 S0155

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 Guidelines
Treatment with epoprostenol requires three (3) steps: initial dose-ranging, catheter insertion and portable pump attachment, and catheter and pump maintenance. 

  • Initial dose-ranging study, which is typically performed as an inpatient. The pulmonary capillary wedge pressure is monitored, and the infusion rate of the drug is increased until dose-limiting pharmacologic effect such as nausea, vomiting, or headache is elicited. Some practitioners may consider the initial dose-ranging study optional. 
  • Insertion of central venous catheter and attachment to portable infusion pump. Since rebound pulmonary hypertension may recur if the drug is abruptly withdrawn, the drug labeling advises that all patients should have access to a backup infusion pump and intravenous infusion set. 
  • Ongoing maintenance of portable infusion pump and treatment of complications related to the pump. Complications include catheter thrombosis, sepsis, and pump malfunction. The medication must be kept cold at all times during administration with ice packs to cool the pump cassette. 

Procedure Codes 

93503 J1325 K0455 S0155 S9347

Treprostinil (Remodulin) continuous subcutaneous (SQ) or IV infusion

Treprostinil (Remodulin) may be considered medically necessary for the treatment of EITHER of the following: 

  • PAH (WHO Group 1) with documented WHO-FC II-IV symptoms; or 
  • PAH (WHO Group 1) patients requiring transition from epoprostenol sodium (e.g. Flolan). 

Procedure Codes 

J3285 K0455

Treprostinil (Tyvaso) for oral inhalation

Treprostinil (Tyvaso) may be considered medically necessary for the treatment of EITHER of the following: 

  • PAH (WHO Group I) with documented WHO-FC III symptoms; or 
  • PAH (WHO Group I) with documented WHO-FC IV symptoms if parenteral therapy not feasible or tolerated. 

Treatment with Treprostinil (Tyvaso) requires the use of a specialized dispensing device. 

Procedure Codes 

J7686

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. 

Procedure Codes 

K0730 Q4074

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. 

Procedure Codes 

J3490

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: 

  • Medications are from different therapeutic classes; and
  • Each medication may be considered medically necessary for the treatment of PAH.

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. 

Procedure Codes 

J1325 J3285 J3490 J7686 K0455
K0730 Q4074 S0155

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: 

  • Pulmonary hypertension (PH) associated with left heart diseases; or 
  • PH associated with lung diseases and/ or hypoxemia (including chronic obstructive pulmonary disease); or 
  • PH due to chronic thrombotic and/or embolic disease; or 
  • Miscellaneous group (i.e., sarcoidosis, histiocytosis X, and lymphangiomatosis). 

Procedure Codes 

J1325 J3285 J3490 J7686 K0455
K0730 Q4074 S0155

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.

Professional Statements and Societal Positions

The World Health Organization (WHO) classifies pulmonary hypertension (PH) into five (5) categories based on the disease etiology. These diagnostic classifications include:

  • Group I pulmonary arterial hypertension
  • Group II pulmonary hypertension with left heart disease
  • Group III pulmonary hypertension associated with lung disease and/or hypoxemia
  • Group IV pulmonary hypertension due to chronic thromboembolic disease
  • Group V pulmonary hypertension with unclear multifactorial mechanisms.

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):

  • Class I – No limitation of clinical activity; ordinary physical activity does not cause dyspnea or fatigue.
  • Class II – Slight limitation in physical activity; ordinary physical activity produces dyspnea, fatigue, chest pain, or near-syncope; no symptoms at rest.
  • Class II I–  Marked limitation of physical activity; less than ordinary physical activity produces dyspnea, fatigue, chest pain, or near-syncope; no symptoms at rest.
  • Class IV – Unable to perform any physical activity without symptoms; dyspnea and/or fatigue present at rest; discomfort increased by any physical activity.

New York Heart Association (NYHA) Functional Classifications:

  • Class I – Patients with no limitation of activities; they suffer no symptoms from ordinary activities
  • Class II – Patients with slight, mild limitation of activity; they are comfortable with rest or mild exertion.
  • Class III–  Patients with marked limitation of activity; they are comfortable only at rest.
  • Class IV – Patients who should be at complete rest, confined to bed or chair; any physical activity brings on discomfort and symptoms occur at rest.

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:

  • For patients with PAH who are treatment-naive, have WHO functional class II or class III symptoms and have failed or are not candidates for CCB therapy, monotherapy with an approved medication is recommended (eg, endothelin receptor antagonist, phosphodiesterase type 5 inhibitor, riociguat).
  • For patients with PAH in WHO functional class III who have evidence of disease progression or poor clinical prognosis despite treatment with 1 or 2 classes of oral agents, consideration of the addition of a parenteral or inhaled prostanoid is recommended.
  • For patients with PAH who are treatment-naive and have WHO functional class IV symptoms, initial monotherapy with a parental prostanoid agent is recommended. If patients are unable or unwilling to manage parenteral therapy, combination treatment with an inhaled prostanoid and an endothelin receptor antagonist is recommended.

Diagnosis Codes

Covered Diagnosis Codes for Procedure Codes J1325, J3285, J7686, or Q4074

I27.0 I27.20 I27.21 I27.29 I27.89

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Disclaimer

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.

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