Epoprostenol is a manufactured prostacyclin, a hormone produced by the innermost layer of blood vessels. Prostacyclin helps those blood vessels relax and widen. It also helps prevent blood clots and may reduce inflammation. Many PH patients don’t make enough prostacyclin.

Relaxing and widening blood vessels in the lungs decreases lung pressures, reduces strain on the right heart and improves heart function. Lower lung pressure and improved heart function generally improves the ability to be more active.

Epoprostenol for injection is administered continuously via a portable infusion pump. It is delivered through a thin tube called a catheter into a large vein near the heart. One end of the catheter is surgically implanted in the chest and the other end is attached to a small, portable pump. The pump stores the medication and keeps it flowing into your body. Your pump should always be on, and the drug infusion must always be running. You must carry or wear the pump at all times.

When you begin treatment, you typically will be hospitalized for several days. Your health care team, including your PH doctor and nurse coordinator, pharmacists and specialty nurses, will guide you step-by-step to prepare, use and store your medication. Your health care team also will show you how to care for your central line, prevent infection, operate the pump and troubleshoot problems with the pump.

Available portable pumps:

• CADD-SOLIS VIP pump
• CADD-Legacy pump (limited supply available due to its older generation status)

Mix vials of Veletri powder and dilute it with commercially available sterile water for injection USP, or sodium chloride 0.9% injection USP. Don’t mix Veletri with other solutions or medications before or during administration. Each vial is for single use only. Discard unused solution.

After mixing the powder with the sterile water or sterile saline, transfer 100 mL to an infusion cassette that fits in the small battery-powered pump. This process must be done carefully to avoid accidentally adding bacteria to the medicine, which could cause infection.

To avoid bloodstream infection, proper hand hygiene is imperative when you prepare your medication and clean the infusion catheter. Your care team will teach you strict procedures to eliminate germs to prevent infection.

Your health care provider will determine the dose you need. Generally, you will start epoprostenol at a low dose (1-2 ng/kg/min) in the hospital. Your dose will increase incrementally a few times a day depending on how well you tolerate the medicine.

After you’re discharged, you can increase your dose every few days. Your dose may change during the course of your therapy. Dosage varies for each patient.

Epoprostenol is a limited-distribution medication, which means you can’t buy it at a local pharmacy. You usually need approval from your insurance company before starting this therapy. Your insurance company will identify a specialty mail-order pharmacy to fill your prescription. The specialty pharmacy has teams of clinical pharmacist and nurses to support people on epoprostenol IV infusion therapy. Specialty pharmacies include Accredo Health Group and CVS specialty.

Your specialty pharmacy team will help with all aspects of long-term epoprostenol use, including insurance issues and education about pump function and central line care. It also will provide pumps, supplies and 24-hour technical troubleshooting.

Epoprostenol is generally well tolerated. The most common side effects are:

• Headache
• Diarrhea
• Nausea
• Jaw pain
• Flushing of the skin
• Dizziness
• Muscle or joint pain
• Low systemic blood pressure
• Tingling or numbness or itching of hands, arms, legs or feet

In addition to side effects from the medicine, you might have side effects from the infusion system. Infection and bleeding can occur at the IV infusion site.

Allergic reactions to the medication are possible but unlikely.

Note: Don’t infuse other medications with IV epoprostenol. If you are admitted to a hospital that isn’t a Pulmonary Hypertension Care Center, tell your care team not to flush your central line for epoprostenol or use it for other medications or blood draws. Tell them they shouldn’t stop your infusion in any circumstance, including an emergency, unless your PH provider orders it.

Prostacyclins like epoprostenol often are used in combination with other PAH medications. Drug interactions are more likely when multiple medications are used to treat PAH and other chronic health conditions. Consult a pharmacist and your PH care team to identify and manage potential drug interactions, which might require adjusting your dose.

Certain patient populations must take precautions while taking ambrisentan, while others should avoid it. This section addresses special populations and considerations.

Pregnant and breastfeeding patients
Limited published data is available about epoprostenol when used during pregnancy. The data available hasn’t shown a link to major birth defects, miscarriages or adverse maternal or fetal outcomes when used during pregnancy.

When untreated, pulmonary arterial hypertension carries severe risks during pregnancy and after birth, including:

• Heart failure
• Stroke
• Preterm delivery
• Maternal and fetal death

Make sure you discuss risks and potential benefits of epoprostenol infusion therapy during pregnancy with your PH provider.

There is no data on the presence of epoprostenol in human milk or the effects on breastfed infants. The developmental and health benefits of breastfeeding on the child and the mother’s clinical need for epoprostenol should be considered with potential adverse effects from epoprostenol on the child or underlying medical conditions.

Children and teens
Safety and efficacy for pediatric patients haven’t been established. Epoprostenol has been prescribed for children.

People with liver disease
Liver disease may decrease a patient’s ability to clear epoprostenol and tolerance for the medications. Patients with mild or moderate liver insufficiency might need start infusion at lower doses, and they could be more sensitive to dose increases. Epoprostenol hasn’t been studied in people with severe liver disease. The manufacturer doesn’t provide dosage adjustment recommendations for people with impaired liver function.

People with kidney disease
There is limited research about epoprostenol use in patients with impaired kidney function. Likewise, the effect of dialysis is unknown. The manufacturer doesn’t provide dosage adjustment recommendations for people with impaired kidney function.