Coverage for treatment of pulmonary hypertension is determined according to individual or group customer benefits. 

A diagnosis of pulmonary hypertension is substantiated by results from Doppler echocardiography and/or direct measurement of pulmonary arterial pressure. Pulmonary arterial hypertension is defined as a mean pulmonary arterial pressure of greater than or equal to 25 mmHg, with a pulmonary capillary wedge pressure of less than 15 mmHg.

The patient must meet the criteria specific to the individual drug as listed below.

Flolan

Treatment with continuous intravenous infusion of epoprostenol sodium (Flolan®)(J1325) is covered for patients who meet the following criteria:

1. Primary pulmonary hypertension (416.0) and associated New York Heart Association Class III or IV symptoms that do not respond adequately to conventional therapy (e.g., oral vasodilator therapy).
   
   
2. Secondary pulmonary hypertension (416.8) related to congenital heart disease and associated New York Heart Association Class III or IV symptoms that do not respond adequately to conventional therapy (e.g., oral vasodilator therapy).
   
   
3. Secondary pulmonary hypertension (416.8) related to connective tissue diseases (i.e., scleroderma, CREST syndrome, systemic lupus erythematosus) and associated New York Heart Association Class III or IV symptoms that do not respond adequately to conventional therapy (e.g., oral vasodilator therapy).

Remodulin

Treatment with continuous subcutaneous infusion of treprostinil (Remodulin®)(Q4077, S0114) is covered for patients who meet the following criteria:

1. Primary pulmonary hypertension (416.0) and associated New York Heart Association Class II, III or IV symptoms that do not respond adequately to conventional therapy (e.g., oral vasodilator therapy).
   
   
2. Secondary pulmonary hypertension (416.8) related to congenital heart disease and associated New York Heart Association Class II, III or IV symptoms that do not respond adequately to conventional therapy (e.g., oral vasodilator therapy).
   
   
3. Secondary pulmonary hypertension (416.8) related to connective tissue diseases (i.e., scleroderma, CREST syndrome, systemic lupus erythematosus) and associated New York Heart Association Class II, III or IV symptoms that do not respond adequately to conventional therapy (e.g., oral vasodilator therapy).

Ventavis

Treatment with inhalation administration of iloprost (Ventavis®)(Q4080) is covered for patients who meet the following criteria:

1. Primary pulmonary hypertension (416.0) and New York Heart Association (NYHA) Class III or Class IV symptoms that do not respond adequately to conventional therapy (e.g., oral vasodilator therapy).
   
   
2. Secondary pulmonary hypertension (416.8) related to collagen vascular disease, congenital systemic-to-pulmonary shunt, portal hypertension, Human Immunodeficiency Virus (HIV) infection, drugs and toxins, and appetite suppressants; and persistent pulmonary hypertension of the newborn and New York Heart Association (NYHA) Class III or Class IV symptoms that do not respond adequately to conventional therapy (e.g., oral vasodilator therapy).
   

Ventavis has not been studied in children under the age of 18.

Ventavis is formulated for inhalation only via the Prodose® Adaptive Aerosol Delivery (AAD) System (K0730). On August 24, 2005, the I-Neb Adaptive Aerosol Delivery (AAD) System was FDA approved as an additional drug delivery system.

Bosentan (Tracleer^TM) and sildenafil (Revatio^TM) are indicated for the treatment of pulmonary hypertension and are only available through the retail prescription drug benefit.

Tracleer and Revatio do not have FDA approval to be used with Flolan, Remodulin, or Ventavis.

Flolan, Remodulin, and Ventavis do not have FDA approval to be used as combination therapy with each other.

Refer to the Text Attachment below for New York Heart Association Classifications.

Any use for conditions other than those with FDA approval will be denied as not medically necessary, and therefore, not covered. A participating, preferred, or network provider cannot bill the member for the denied service. 

Refer to Medical Policy Bulletin E-17 for information on portable (external) infusion pumps.

Description

Flolan is a naturally occurring prostacyclin that decreases pulmonary vascular resistance and increases cardiac output.  It is administered as a continuous intravenous infusion. Surgical implantation of a central venous access device is required. A backup pump is utilized to assure continuous administration. Flolan has a rapid onset of action, within seconds of infusion, and has a short half-life of approximately six minutes.  The medication must be kept cold at all times during administration with ice packs to cool the pump cassette.

Remodulin is a prostacyclin analogue that decreases pressure in the pulmonary artery, causing patients to experience significant improvements in the signs and symptoms of this disease. It is administered as a continuous, subcutaneous infusion. The patient is taught to self-administer via a MiniMed 407C infusion pump. Two pumps are required to ensure continuous delivery of the therapy. This is necessary and even critical since interruption in the infusion may cause the patient to begin to experience rebound symptoms. Remodulin is chemically stable at room temperature and has a half-life of three hours.

Ventavis is an inhaled formulation of iloprost, a synthetic prostacyclin analogue. Inhaled iloprost acts as a vasodilator directly on the pulmonary arterial circulation to lower pulmonary artery pressure and improve cardiac output and oxygen saturation. The effects of the inhaled drug last approximately 60 minutes; therefore, multiple inhalations are required daily.

NOTE:

This policy is designed to address medical guidelines that are appropriate for the majority of individuals with a particular disease, illness, or condition. Each person's unique clinical circumstances may warrant individual consideration, based on review of applicable medical records.

Flolan, Physician's Desk Reference, Edition 55, 2001, Medical Economics Company, Inc.

USPDI, Vol.1, Edition 24, 2004, Micromedex, Inc.

United Therapeutics Corporation, Remodulin® (treprostinil), Product Information, United Therapeutics; March, 2002

CoTherix^TM, Ventavis® (iloprost), Product Information, CoTherix, Inc.; 2005

In 1928, the New York Heart Association published a classification of patients with cardiac disease based on clinical severity and prognosis. This classification has been updated in seven subsequent editions of Nomenclature and Criteria for Diagnosis of Diseases of the Heart and Great Vessels (Little, Brown & Co.). The ninth edition, revised by the Criteria Committee of the American Heart Association, New York City Affiliate, was released March 4, 1994. The new classifications are summarized below:

Functional Capacity

Class I. Patients with cardiac disease but without resulting limitation of physical activity. Ordinary physical activity does not cause undue fatigue, palpitation, dyspnea, or anginal pain.

Class II. Patients with cardiac disease resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity results in fatigue, palpitation, dyspnea, or anginal pain.

Class III. Patients with cardiac disease resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes fatigue, palpitation, dyspnea, or anginal pain.

Class IV. Patients with cardiac disease resulting in inability to carry on any physical activity without discomfort. Symptoms of heart failure or the anginal syndrome may be present even at rest. If any physical activity is undertaken, discomfort is increased.
Objective Assessment

1. No objective evidence of cardiovascular disease
2. Objective evidence of minimal cardiovascular disease
3. Objective evidence of moderately severe cardiovascular disease
4. Objective evidence of severe cardiovascular disease