Notification

Treatment of Pulmonary Artery Hypertension with Intravenous, Subcutaneous, and Inhaled Pharmacologic Agents


Notification Issue Date: 10/03/2019

This version of the policy will become effective 01/01/2020.

The policy was updated to communicate coverage criteria, for sildenafil (Revatio®), intravenous infusion for pulmonary arterial hypertension (PAH) consistent with the US Food and Drug Administration (FDA) labeling.

The following HCPCS codes have been added to this policy to represent sildenafil (Revatio®) IV injection:

C9399 Unclassified drugs or biologicals
J3490 Unclassified drugs

Dosing and frequency requirements were added for all the agents to Attachment A of this policy.



Medical Policy Bulletin


Title:Treatment of Pulmonary Artery Hypertension with Intravenous, Subcutaneous, and Inhaled Pharmacologic Agents

Policy #:08.00.25l

This policy is applicable to the Company’s commercial products only. Policies that are applicable to the Company’s Medicare Advantage products are accessible via a separate Medicare Advantage policy database.


The Company makes decisions on coverage based on Policy Bulletins, benefit plan documents, and the member’s medical history and condition. Benefits may vary based on contract, and individual member benefits must be verified. The Company determines medical necessity only if the benefit exists and no contract exclusions are applicable.

When services can be administered in various settings, the Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member’s medical needs and condition. This decision is based on the member’s current medical condition and any required monitoring or additional services that may coincide with the delivery of this service.

This Medical Policy Bulletin document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy Bulletin will be reviewed regularly and be updated as scientific and medical literature becomes available. For more information on how Medical Policy Bulletins are developed, go to the About This Site section of this Medical Policy Web site.



Policy

Coverage is subject to the terms, conditions, and limitations of the member's contract.

The Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member’s medical needs and condition.

MEDICALLY NECESSARY

EPOPROSTENOL (FLOLAN®, VELETRI®)
Epoprostenol (Flolan®, Veletri®), intravenous infusion, is considered medically necessary and, therefore, covered, when the dosing and frequency requirements listed in Attachment A and all of the following criteria are met:
  • The individual has a confirmed diagnosis of pulmonary arterial hypertension (PAH), in accordance with the World Health Organization (WHO) Group 1 classification.
    • The diagnosis of PAH has been confirmed by a catherization (right heart or Swan-Ganz) or echocardiography.
  • Documentation that the individual's mean pulmonary arterial pressure is greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise/exertion.
  • The individual meets the New York Heart Association (NYHA) Functional Classification requirement of Class III-IV.

TREPROSTINIL SODIUM (REMODULIN®)
Treprostinil sodium (Remodulin®), intravenous or subcutaneous infusion, is considered medically necessary and, therefore, covered when the dosing and frequency requirements listed in Attachment A and all of the following criteria are met:
  • The individual has a confirmed diagnosis of PAH, in accordance with the WHO Group 1 classification.
    • The diagnosis of PAH has been confirmed by a catherization (right heart or Swan-Ganz) or echocardiography.
  • Documentation that the individual's mean pulmonary arterial pressure is greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise/exertion.
  • The individual meets the NYHA Functional Classification requirement of Class II-IV.

In addition, Treprostinil sodium (Remodulin®), intravenous or subcutaneous infusion, is considered medically necessary and, therefore, covered for individuals requiring transition from Flolan® to diminish the rate of clinical deterioration.

*Clinical deterioration is defined as an increase in Flolan® dose, hospitalization due to PAH.

ILOPROST (VENTAVIS®) AND TREPROSTINIL (TYVASO®)
Iloprost (Ventavis®) and Treprostinil (Tyvaso®), inhalation, is considered medically necessary and, therefore, covered when the dosing and frequency requirements listed in Attachment A and all of the following criteria are met:
  • The individual has a confirmed diagnosis of PAH, in accordance with the WHO Group 1 classification.
    • The diagnosis of PAH has been confirmed by a catherization (right heart or Swan-Ganz) or echocardiography.
  • Documentation that the individual's mean pulmonary arterial pressure is greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise/exertion.
  • The individual meets the NYHA Functional Classification requirements of Class III-IV for Iloprost (Ventavis®) or Class III for Treprostinil, (Tyvaso®).

SILDENAFIL (REVATIO®)
Sildenafil (Revatio®), intravenous infusion for continued treatment of individuals who are currently prescribed oral REVATIO® and who are temporarily unable to take oral medication, is considered medically necessary and, therefore, covered, when the dosing and frequency requirements listed in Attachment A and all of the following criteria are met:
  • The individual has a confirmed diagnosis of PAH, in accordance with the World Health Organization (WHO) Group 1 classification.
    • The diagnosis of PAH has been confirmed by a catheterization (right heart or Swan-Ganz) or echocardiography.
  • Documentation that the individual's mean pulmonary arterial pressure is greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise/exertion.
  • The individual meets the New York Heart Association (NYHA) Functional Classification requirement of Class II-III.

EXPERIMENTAL/INVESTIGATIONAL

All other uses for epoprostenol (Flolan®) (Veletri®); treprostinil sodium (Remodulin®); iloprost (Ventavis®); treprostinil (Tyvaso®), and sildenafil (Revatio®); are considered experimental/investigational and, therefore, not covered unless the indication is supported as an accepted off-label use, as defined in the medical policy on off-label coverage for prescription drugs and biologics.

Although the US Food and Drug Administration (FDA) has approved a fully implantable intravenous infusion pump system used to deliver pharmacological agents (prostanoids) for the treatment of PAH, the Company has determined that the safety and/or effectiveness of this device cannot be established by review of the available published peer-reviewed literature. Therefore, a fully implantable intravenous infusion pump system used to deliver pharmacological agents (prostanoids) for the treatment of PAH is considered experimental/investigational by the Company and not covered.

NOT ELIGIBLE FOR SEPARATE REIMBURSEMENT

The Company covers the use of intravenous (IV) fluids for the preparation (e.g., dilution, reconstitution) of pharmaceuticals, biologics, and other substances for IV administration; however, such use is considered to be integral to the administration of the pharmaceutical, biologic, or other substance and is, therefore, not eligible for separate reimbursement. Participating providers may not bill members for this service.

DOSING AND FREQUENCY REQUIREMENTS

Refer to Attachment A for dosing and frequency requirements for epoprostenol (Flolan®) (Veletri®); treprostinil sodium (Remodulin®); iloprost (Ventavis®); treprostinil (Tyvaso®); and sildenafil (Revatio®).

The Company reserves the right to modify the Dosing and Frequency Requirements listed in this Policy to ensure consistency with the most recently published recommendations for the use of epoprostenol (Flolan®) (Veletri®); treprostinil sodium (Remodulin®); iloprost (Ventavis®); treprostinil (Tyvaso®); and sildenafil (Revatio®). Changes to these guidelines are based on a consensus of information obtained from resources such as, but not limited to: the US Food and Drug Administration (FDA); Company-recognized authoritative pharmacology compendia; or published peer-reviewed clinical research. The professional provider must supply supporting documentation (i.e., published peer-reviewed literature) in order to request coverage for an amount of epoprostenol (Flolan®) (Veletri®); treprostinil sodium (Remodulin®); iloprost (Ventavis®); treprostinil (Tyvaso®); and sildenafil (Revatio®) outside of the Dosing and Frequency Requirements listed in this Policy. For a list of Company-recognized pharmacology compendia, view our policy on off-label coverage for prescription drugs and biologics.

Accurate member information is necessary for the Company to approve the requested dose and frequency of this drug. If the member’s dose, frequency, or regimen changes (based on factors such as changes in member weight or incomplete therapeutic response), the provider must submit those changes to the Company for a new approval based on those changes as part of the precertification process. The Company reserves the right to conduct post-payment review and audit procedures for any claims submitted for epoprostenol (Flolan®) (Veletri®); treprostinil sodium (Remodulin®); iloprost (Ventavis®); treprostinil (Tyvaso®); and sildenafil (Revatio®).

REQUIRED DOCUMENTATION

The individual's medical record must reflect the medical necessity for the care provided. These medical records may include, but are not limited to: records from the professional provider's office, hospital, nursing home, home health agencies, therapies, and test reports.

The Company may conduct reviews and audits of services to our members, regardless of the participation status of the provider. All documentation is to be available to the Company upon request. Failure to produce the requested information may result in a denial for the drug.
Guidelines

The World Health Organization (WHO) Group 1 Classification

Pulmonary arterial hypertension (PAH) represents Group 1 within the Pulmonary Hypertension World Health Organization (WHO) clinical classification system and is one of five such groups. The groups are divided based on etiology.
  • Idiopathic (IPAH): a rare form of PAH characterized by elevated pulmonary artery pressure with no apparent cause.
  • Heritable (HPAH): a form of PAH caused by predisposing genes or from a familial context.
    • Bone morphogenetic protein receptor type 2 (BMPR2)
    • Activin receptor-like kinase 1 gene (ALK1), endoglin (with or without haemorrhagic telangiectasia)
    • Unknown
  • Drug- and toxin-induced
  • Associated with (APAH)
    • Connective tissue diseases
    • Human immunodeficiency virus (HIV) infection
    • Portal hypertension
    • Congenital heart disease (CHD)
    • Schistosomiasis
    • Chronic haemolytic anemia
  • Persistent pulmonary hypertension of the newborn (PPHN)
Unique class 1 (Group 1)
  • Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)

The New York Heart Association (NYHA) Functional Classifications are summarized as follows:

Class I: Individuals with no limitation of physical activity and no symptoms. Ordinary physical activity does not cause undue fatigue, palpitations, or dyspnea (shortness of breath).

Class II: Individuals with slight limitation of physical activity and mild symptoms. Comfortable at rest. Ordinary physical activity results in fatigue, palpitations, dyspnea, or anginal pain.

Class III: Individuals with marked limitation of physical activity due to symptoms. Comfortable at rest, but less than ordinary activity causes fatigue, palpitations, dyspnea, or anginal pain.

Class IV: Individuals with severe limitations experience symptoms of heart failure or the anginal syndrome that may be present even while at rest. If any physical activity is undertaken, discomfort is increased.

BENEFIT APPLICATION

Subject to the terms and conditions of the applicable benefit contract, epoprostenol (Flolan®) (Veletri®); treprostinil sodium (Remodulin®); iloprost (Ventavis®); treprostinil (Tyvaso®); and sildenafil (Revatio®) are covered under the medical benefit of the Company’s products when the medical necessity criteria listed in this medical policy are met.

A medication or class of medications may be a product or group contract exclusion. Individual benefits must be verified.

US FOOD AND DRUG ADMINISTRATION (FDA) STATUS

All pharmacologic agents listed in this medical policy have US Food and Drug Administration approval for the treatment of PAH.

Description

DESCRIPTION AND ETIOLOGY OF PULMONARY ARTERIAL HYPERTENSION (PAH)

Pulmonary arterial hypertension (PAH) (also referred to as pulmonary hypertension [PH] or primary or secondary pulmonary hypertension [PPH, SPH]) is a progressive disorder characterized by abnormally high blood pressure (hypertension) in the pulmonary artery, which carries blood from the heart to the lungs. Hypertension occurs when most of the small arteries throughout the lungs narrow in diameter, increasing resistance to blood flow through the lungs. To overcome the increased resistance, pressure intensifies in the pulmonary artery and in the right ventricle that pumps blood into the pulmonary artery. The right side of the heart is forced to work harder against this increased pressure, and, over time, this heightened work load causes the right side of the heart to become enlarged and can progress to right-sided heart failure (cor pulmonale). The National Institutes of Health (NIH) registry working group has defined PAH as a mean pulmonary artery pressure >25 mm Hg at rest or 30 mm Hg with exercise and no known or proven etiology. There are two main methods used to measure pulmonary artery (PA) pressures: trans-thoracic echocardiogram (TTE) and right heart catheterization.

Signs and symptoms of the presence of PAH occur when increased pressure cannot fully overcome the elevated resistance, resulting in insufficient blood flow to the body and its organs. The most frequently reported symptom of PAH is dyspnea of exertion (DOE), and the person may report periods of fainting. As the condition worsens, other symptoms, such as dizziness, swelling (edema) of the ankles or legs, overall muscle weakness, chest pain, cyanosis of the skin and/or lips, and heart palpitations, are reported.

THE WORLD HEALTH ORGANIZATION (WHO) CLASSIFICATION OF PULMONARY HYPERTENSION (PH)

PAH is one subset of the larger disease category of pulmonary hypertension (PH). PH is a general term used to describe a chronic, progressive condition characterized by high pulmonary vascular pressure in the lungs. The World Health Organization (WHO) developed a 5-group classification system for PH based upon disease etiology. The individuals in WHO Group 1 are considered to have PAH, whereas those in the remaining Group 2--Group 5 are considered to have PH. However, when the groups are discussed collectively, the term PH is used.

OVERALL TREATMENT OF PAH

The treatment of PAH is highly individualized. The goals of PAH treatment consist of symptom improvement, enhanced functional capacity, reversal or prevention of disease worsening, and prevention of hospitalization. Therapy to control PAH consists of diet, exercise, and pharmacologic treatment. Oxygen and medications such as anticoagulants, diuretics, and digoxin are used initially to treat the underlying cause of PAH. In more advanced disease, medications such as calcium channel blockers, intravenous (IV) or inhaled prostanoids (e.g., epoprostenol), endothelin receptor antagonists (e.g., bosentan), or phosphodiesterase 5 inhibitors (e.g. sildenafil) are used as monotherapy or in combination.

AGENTS ADMINISTERED VIA INTRAVENOUS ROUTE

EPOPROSTENOL (FLOLAN®)
Epoprostenol (Flolan®) is a naturally occurring prostaglandin with potent vasodilatory activity and inhibitory activity of platelet aggregation. It is indicated for the treatment of pulmonary arterial hypertension (WHO Group 1) to improve exercise capacity in individuals with NYHA Functional Class III-IV symptoms and etiologies of idiopathic or heritable PAH or PAH associated with connective tissue diseases.

EPOPROSTENOL (VELETRI®)
Veletri® (epoprostenol) is a potent vasodilator intended for the treatment of pulmonary artery hypertension (PAH) to improve exercise capacity. During clinical trials, data demonstrating efficacy was obtained predominantly from individuals with NHYA Functional Class III-IV symptoms with etiology from idiopathic or inherited PAH or PAH associated with connective tissue diseases. Veletri® (epoprostenol) is administered by continuous intravenous infusion via a central venous catheter using an ambulatory infusion pump.

SILDENAFIL (REVATIO®)
Sildenafil (Revatio®) is a phosphodiesterase-5 (PDE-5) inhibitor indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group I) in adult individuals to improve exercise ability and delay clinical worsening. Studies establishing effectiveness were short-term (12 to 16 weeks), and included predominately individuals with NYHA Functional Class II-III symptoms. Etiologies were idiopathic (71 percent) or associated with connective tissue disease (25 percent). Sildenafil (Revatio®) may be administered orally or by injection. Intravenous infusion is for the continued treatment of individuals with PAH who are currently prescribed oral Sildenafil (Revatio®) and who are temporarily unable to take oral medication.

AGENTS ADMINISTERED VIA INTRAVENOUS OR SUBCUTANEOUS INFUSION

TREPROSTINIL (REMODULIN®)
Treprostinil (Remodulin®) is a prostacyclin that causes direct vasodilation of pulmonary and systemic arterial vascular beds and inhibition of platelet aggregation. It is indicated in the treatment of pulmonary arterial hypertension (WHO Group 1) in individuals with NYHA Class II-IV symptoms. Treprostinil (Remodulin®) is administered as a continuous infusion via a self-inserted subcutaneous catheter using an infusion pump designed for subcutaneous drug delivery. Continuous subcutaneous infusion (undiluted) is the preferred mode of delivery. Intravenous infusion (with dilution required), is reserved for those who cannot tolerate continuous subcutaneous infusion.

Treprostinil (Remodulin®) was originally approved for administration by continuous subcutaneous and intravenous routes using external pumps. Medtronic Inc. (Minneapolis, MN) and United Therapeutics Corp. (Research Triangle Park, NC) received FDA approval for the Implantable System for Remodulin®. The system is comprised of the SynchroMed II Pump programmable implantable drug delivery system and a newly developed intravascular catheter to deliver Remodulin intravenously for adults with pulmonary arterial hypertension (Class I, II, and III) who were receiving Remodulin® via continuous intravenous infusion with an external infusion pump. The FDA approval was based on data reported from the DellVery for Pulmonary Arterial Hypertension (PAH) clinical study, a prospective, single-arm, nonrandomized, open-label multicenter study (Waxman 2017). The trial enrolled 64 adults with PAH (WHO group 1); four individuals exited the study prior to device implantation, and the remaining 60 individuals had successful implant procedures. The study showed the implantable intravascular delivery system was an effective option for delivery of treprostinil (Remodulin®), with a low rate of catheter-related complications, and a high rate of individual satisfaction. Clinically significant implant procedure-related complications included one pneumothorax, two infections unrelated to catheter placement, and one episode of atrial fibrillation. Three catheter dislocations in two individuals occurred early in the trial; catheters were removed and replaced via surgical procedure. Larger well designed studies with long-term follow-up are needed to determine safety and effectiveness of fully implantable intravenous pumps used to deliver pharmacological agents (prostanoids) for the treatment of pulmonary hypertension.

AGENTS ADMINISTERED VIA INHALATION

ILOPROST (VENTAVIS®) INHALATION SOLUTION
Ventavis® (iloprost) inhalation solution is indicated for the treatment of pulmonary arterial hypertension to improve exercise tolerance and NYHA symptoms and prevent deterioration. During clinical trials, data demonstrating efficacy was obtained mostly from individuals with NYHA Functional Class III-IV symptoms who had idiopathic or inherited PAH or PAH associated with connective tissue diseases. Ventavis® is administered via an I-neb® Adaptive Aerosol Delivery (AAD) system typically obtained from a specialized pharmacy. The I-neb® ADD nebulizer is not the same type of device that is commonly used to deliver nebulized medications for conditions such as asthma.

TREPROSTINIL (TYVASO®) INHALATION SOLUTION
Tyvaso® (treprostinil) inhalation solution is a prostacyclin vasodilator indicated for the treatment of PAH (WHO Group 1) to improve exercise ability. Studies establishing effectiveness included predominately individuals with NHYA Functional Class III symptoms with etiologies of idiopathic or inherited PAH or PAH associated with connective tissue diseases. Tyvaso® is given by oral inhalation only via a special nebulizing device, the Optineb®, and its accessories; the Optineb® is typically obtained from a specialized pharmacy. The Optineb® is not the same type of device that is commonly used to deliver nebulized medications for conditions such as asthma.

COMPARISON OF PRODUCTS

DrugRoute of Administration NYHA Functional Class Symptoms
epoprostenol (Flolan®) *Continuous intravenous infusion Class III and Class IV
epoprostenol (Veletri®) * Continuous intravenous infusion Class III and Class IV
treprostinil (Remodulin®) *Continuous intravenous or subcutaneous infusion Class II, Class III, Class IV
iloprost (Ventavis® Inhalation Solution) *Inhaled Class III and Class IV
treprostinil (Tyvaso® Inhalation Solution)*Inhaled Class III
sildenafil (Revatio®)* Oral or intravenous bolus injection Class II and Class III
*Safety and effectiveness in the pediatric population have not been established.

There may be additional indications contained in the Policy section of this document due to evaluation of criteria highlighted in the Company’s off-label policy, and/or review of clinical guidelines issued by leading professional organizations and government entities.
References

Actelion Pharmaceuticals Ltd. Pulmonary Arterial Hypertension. [Actelion Web site]. 10/01/2019. Available at: https://www1.actelion.com/about-actelion/target-diseases/pulmonary-arterial-hypertension. Accessed January 18, 2019.

American Heart Association. Heart failure classification. [American Heart Association Web site]. 05/31/2017. Available at:http://www.heart.org/HEARTORG/Conditions/HeartFailure/AboutHeartFailure/Classes-of-Heart-Failure_UCM_306328_Article.jsp. Accessed January 18, 2019.

Bourge RC, Waxman AB, Gomberg-Maitland M, et al. Treprostinil administered to treat pulmonary arterial hypertension using a fully implantable programmable intravascular delivery system: Results of the DelIVery for PAH trial. Chest. 2016;150(1):27-34.

ClinicalTrials.gov. Sildenafil IV bolus study. ClinicalTrials.gov Identifier: NCT00800592. First Posted: December 02, 2008; Last Update Posted: March 10, 2017. Available at: https://clinicaltrials.gov/ct2/show/NCT02417064. Accessed August 18, 2019.

Draper S. Medtronic’s implantable system for Remodulin to treat patients with pulmonary arterial hypertension gets FDA approval. [Medtronic Web site]. 08/09/2018. Available at:
https://www.wearable-technologies.com/2018/08/medtronics-implantable-system-for-remodulin-to-treat-patients-with-pulmonary-arterial-hypertension-gets-fda-approval/. Accessed January 18, 2019.

Dugdale D, Hadjiliadis, D, Pulmonary Hypertension. [Medline Plus Web site]. 01/07/2019. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/000112.htm. Accessed January 18, 2019.

[Micromedex® Web site]. 02/20/2019. Available at: http://www.micromedexsolutions.com/micromedex2/librarian [via subscription only]. Accessed August 18, 2019.

Epoprostenol (Flolan®; Veletri®). American Hospital Formulary Service (AHFS). AHFS Drug Information 2019. [Lexicom Web site]. 02/22/2019. Available at: http://online.lexi.com/lco/action/doc/retrieve/docid/complete_ashp/413206 [via subscription only]. Accessed August 18, 2019.

Hopkins W, Rubin L. Treatment of pulmonary hypertension in adults. Wolters Kluwer Health. [Up to Date Web site]. 09/26/2018. Available at: http://www.uptodate.com [via subscription only]. Accessed January 18, 2019.

Iloprost (Ventavis®). American Hospital Formulary Service (AHFS). AHFS Drug Information 2019. [Lexicom Web site]. 02/22/2019. Available at:
http://online.lexi.com/lco/action/doc/retrieve/docid/complete_ashp/413242 [via subscription only]. Accessed August 18, 2019.

Iloprost (Ventavis®). Micromedex® Healthcare Series. [Micromedex® Web site]. Last modified 06/24/2019. Available at: http://www.micromedexsolutions.com/micromedex2/librarian [via subscription only]. Accessed August 18, 2019.

McLaughlin VV, Archer SL, Badesch DB, et al. 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. J Am Coll Cardiol. 2009;53(17):1573-619.

National Institutes of Health. DelIVery for Pulmonary Arterial Hypertension (PAH) (DelIVery). [ClinicalTrials.gov Web site]. 05/04/2018. Available at:
https://www.clinicaltrials.gov/ct2/show/results/NCT01321073?term=NCT01321073&rank=1. Accessed January 18, 2019.

Pulmonary Hypertension News. FDA Approves Medtronic’s Implantable System for Remodulin for Treatment of PAH. [PH News Web site]. 08/01/2018. Available at:
https://pulmonaryhypertensionnews.com/2018/08/01/fda-approves-medtronics-implantable-system-for-remodulin-treatment-pah/. Accesssed January 18, 2019.

Remodulin® [package insert]. Transition from Flolan® (epoprostenol sodium) protocol. Research Triangle Park, NC: United Therapeutics Corporation; 2018. Available at: https://hcp.remodulin.com/dosing/transition. Accessed January 18, 2019.

Richter MJ, Ewert R, Warnke C, et al. Procedural safety of a fully implantable intravenous prostanoid pump for pulmonary hypertension. Clin Res Cardiol. 2017;106(3):174-182.

Richter MJ, Harutyunova S, Bollmann T, et al. Long-term safety and outcome of intravenous treprostinil via an implanted pump in pulmonary hypertension. J Heart Lung Transplant.2018;37(10):1235-1244.

Ronald J. Oudiz RJ, Rubin LJ. Exercise-Induced Pulmonary Arterial Hypertension. A New Addition to the Spectrum of Pulmonary Vascular Diseases. Circulation.2008;118:2120-2121. Also available at: http://circ.ahajournals.org/content/118/21/2120. Accesses January 18, 2019.

Sildenafil (Revatio®). American Hospital Formulary Service (AHFS). AHFS Drug Information 2019. [Lexicom Web site]. 05/22/2019. Available at:
http://online.lexi.com/lco/action/doc/retrieve/docid/essential_ashp/410719?searchUrl=%2Flco%2Faction%2Fsearch%3Fq%3DSildenafil%2520Citrate%26t%3Dname%26va%3Dsildenafil#dosage-admin-nested-0-0. Accessed August 18, 2019.

Sildenafil (Revatio®). Micromedex® Healthcare Series. [Micromedex® Web site]. 08/28/2019. Available at: https://www.micromedexsolutions.com/micromedex2/librarian/PFDefaultActionId/evidencexpert.DoIntegratedSearch?navitem=headerLogout#. Accessed August 18, 2019.

Treprostinil (Remodulin®; Tyvaso®). American Hospital Formulary Service (AHFS). AHFS Drug Information 2019. [Lexicom Web site]. 02/22/2019. Available at:
http://online.lexi.com/lco/action/doc/retrieve/docid/complete_ashp/413127[via subscription only]. Accessed August 18, 2019.

Treprostinil (Remodulin®; Tyvaso®). Micromedex® Healthcare Series. [Micromedex® Web site]. Last modified 07/30/2019. Available at: http://www.micromedexsolutions.com/micromedex2/librarian [via subscription only]. Accessed August 18, 2019.

US Food and Drug Administration (FDA). Center for Devices and Radiological Health. Devices@FDA. Device details: Implantable System for Remodulin. [FDA Web site]. 01/26/2018. Available at: https://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/DeviceApprovalsandClearances/Recently-ApprovedDevices/ucm594154.htm. Accessed January 18, 2019.

US Food and Drug Administration (FDA). Center for Devices and Radiological Health. Implantable System for Remodulin®. Summary of safety and effectiveness data. [FDA Web site]. 12/22/2017. Available at: https://www.accessdata.fda.gov/cdrh_docs/pdf14/P140032B.pdf. Accessed January 18, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs@FDA. Accelerated and Restricted Approvals Under Subpart H (drugs) and Subpart E (biologics) labeling. [FDA Web site]. 08/26/2014. Available at: https://www.fda.gov/Drugs/DevelopmentApprovalProcess/HowDrugsareDevelopedandApproved/DrugandBiologicApprovalReports/ucm121597.htm. Accessed January 18, 2019.

US Food and Drug Administration. Center for Drug Evaluation and Research. Drugs @FDA. Approval letter for Epoprostenol. [FDA Web site]. 06/27/2008. Available at:http://www.accessdata.fda.gov/drugsatfda_docs/appletter/2008/022260s000ltr.pdf. Accessed January 18, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs@FDA. Flolan® (epoprostenol sodium). Prescribing information. [FDA Web site]. 12/12/2018. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2018/020444s024lbl.pdf. Accessed January 18, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs@FDA. Iloprost (Ventavis®) Inhalation Solution. Prescribing information. [FDA Web site]. October 2017. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2017/021779s018lbl.pdf. Accessed January 18, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs@FDA. Remodulin® (treprostinil sodium). Prescribing information. [FDA Web site]. May 2002. (Revised July 2018). Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2018/208276s000,021272s025lbl.pdf. Accessed January 18, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs@FDA. Sildenafil (Revatio®) . Prescribing information. [FDA Web site]. 01/17/2019. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/022473s012,203109s012lbl.pdf. Accessed August 18, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs@FDA. Tyvaso® (treprostinil) inhalation solution. Prescribing information. [FDA Web site]. 2002 (Revised October 2017). Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2017/022387s015lbl.pdf. Accessed January 18, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs@FDA. Veletri® (epoprostenol). Prescribing information. [FDA Web site]. 1995 (Revised June 2012). Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2012/022260s005lbl.pdf. Accessed January 18, 2019.

Waxman AB, McElderry HT, Gomberg-Maitland M, et al. Totally implantable IV treprostinil therapy in pulmonary hypertension assessment of the implantation procedure. Chest.2017;152(6):1128-1134.

Wirth JA, Palevsky HI. Prostacyclin therapy for pulmonary arterial hypertension evolves again with the development of an implantable delivery system. [Chest Web site]. December 2017. Available at:
https://journal.chestnet.org/article/S0012-3692(17)31253-9/pdf . Accessed January 18, 2019.





Coding

Inclusion of a code in this table does not imply reimbursement. Eligibility, benefits, limitations, exclusions, precertification/referral requirements, provider contracts, and Company policies apply.

The codes listed below are updated on a regular basis, in accordance with nationally accepted coding guidelines. Therefore, this policy applies to any and all future applicable coding changes, revisions, or updates.

In order to ensure optimal reimbursement, all health care services, devices, and pharmaceuticals should be reported using the billing codes and modifiers that most accurately represent the services rendered, unless otherwise directed by the Company.

The Coding Table lists any CPT, ICD-9, ICD-10, and HCPCS billing codes related only to the specific policy in which they appear.

CPT Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD - 10 Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD -10 Diagnosis Code Number(s)

I27.0 Primary pulmonary hypertension

I27.20 Pulmonary hypertension, unspecified

I27.21 Secondary pulmonary arterial hypertension

I27.22 Pulmonary hypertension due to left heart disease

I27.23 Pulmonary hypertension due to lung diseases and hypoxia

I27.24 Chronic thromboembolic pulmonary hypertension

I27.29 Other secondary pulmonary hypertension

I27.83 Eisenmenger's syndrome

P29.30 Pulmonary hypertension of newborn




HCPCS Level II Code Number(s)



MEDICALLY NECESSARY

J1325 Injection, epoprostenol, 0.5 mg

J3285 Injection, treprostinil, 1 mg

J7686 Treprostinil, inhalation solution, FDA-approved final product, noncompounded, administered through DME, unit dose form, 1.74 mg

Q4074 Iloprost, inhalation solution, FDA-approved final product, noncompounded, administered through DME, unit dose form, up to 20 mcg

S0155 Sterile dilutant for epoprostenol, 50 ml

THE FOLLOWING CODES ARE USED TO REPRESENT REVATIO (sildenafil) injection IV:
C9399 Unclassified drugs or biologicals
J3490 Unclassified drugs

EXPERIMENTAL/INVESTIGATIONAL

THE FOLLOWING CODES ARE CONSIDERED EXPERIMENTAL/INVESTIGATIONAL WHEN REPORTED WITH J1325, J3285, S0155
C1751 Catheter, infusion, inserted peripherally, centrally or midline (other than hemodialysis)

C1772 Infusion pump, programmable (implantable)

E0783 Infusion pump system, implantable, programmable (includes all components, e.g., pump, catheter, connectors, etc.)

E0786 Implantable programmable infusion pump, replacement (excludes implantable intraspinal catheter)


Revenue Code Number(s)

N/A

Coding and Billing Requirements


Cross References

Attachment A: Treatment of Pulmonary Artery Hypertension with Intravenous, Subcutaneous, and Inhaled Pharmacologic Agents
Description: Dosing and Frequency Requirements




Policy History

08.00.25l:
01/01/2020This version of the policy will become effective 01/01/2020.

The policy was updated to communicate coverage criteria, for sildenafil (Revatio®), intravenous infusion for pulmonary arterial hypertension (PAH) consistent with the US Food and Drug Administration (FDA) labeling.

The following HCPCS codes have been added to this policy to represent sildenafil (Revatio®) IV injection:

C9399 Unclassified drugs or biologicals
J3490 Unclassified drugs

Dosing and frequency requirements were added for all the agents to Attachment A of this policy.

08.00.25k:
05/06/2019This version of the policy will become effective 05/06/2019.

This policy was updated to communicate the following:
  • Company's position of experimental/investigational for a fully implantable, intravenous drug delivery system for individuals with pulmonary arterial hypertension.
  • Additon of criteria for Treprostinil sodium (Remodulin®), intravenous or subcutaneous infusion when the individual requires transition from Flolan® to diminish the rate of clinical deterioration.

The following HCPCS codes have been removed from this policy:
    S9061 Home administration of aerosolized drug therapy (e.g., Pentamidine); administrative services, professional pharmacy services, care coordination, all necessary supplies and equipment (drugs and nursing visits coded separately), per diem
    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

The following HCPCS codes have been added to this policy and are considered experimental/investigational when reported with J1325, J3285, S0155:
    C1772 Infusion pump, programmable (implantable)
    C1751 Catheter, infusion, inserted peripherally, centrally or midline (other than hemodialysis)
    E0783 Infusion pump system, implantable, programmable (includes all components, e.g., pump, catheter, connectors, etc.)
    E0786 Implantable programmable infusion pump, replacement (excludes implantable intraspinal catheter)


Effective 10/05/2017 this policy has been updated to the new policy template format.


Version Effective Date: 01/01/2020
Version Issued Date: 12/31/2019
Version Reissued Date: N/A

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