Frequently Asked 
Questions about 
VELETRI^® for Injection

VELETRI^® is a prescription medicine that is given intravenously (in a vein). It is used to treat adults with certain kinds of severe pulmonary arterial hypertension (PAH) (WHO Group 1), a condition in which blood pressure is too high in the blood vessels between the heart and the lungs. VELETRI^® may improve your ability to exercise as measured by how far you can walk in 6 minutes (6-minute walk test).

Studies showing VELETRI^® is effective included mainly patients with NYHA Functional Class III-IV PAH. In these patients, PAH was caused by unidentified or hereditary factors or connective tissue disease.

Epoprostenol belongs to a group of drugs called prostacyclins. Prostacyclin occurs naturally in the body. Epoprostenol dilates the blood vessels in the lungs.^1-2

Intravenous (IV) therapy is drug therapy that is delivered continuously through an IV line.² This line is connected to a catheter that is placed in the heart via a surgical procedure. VELETRI^® has a half-life of approximately 6 minutes—meaning, within 6 minutes after the drug is absorbed, half the drug has already been used by the body or degraded.¹ Because of this, VELETRI^® cannot be given as a pill that can be swallowed.

A doctor (surgeon) places a very thin tube (the catheter) into your heart during a surgical procedure using local anesthesia. The doctor then connects the catheter line to a small, battery-powered pump that delivers the medication. VELETRI^®, in liquid form, is stored in a small, removable cassette attached to the pump.¹

VELETRI^® is a prescription medicine that is given intravenously (in a vein). It is used to treat adults with certain kinds of severe pulmonary arterial hypertension (PAH) (WHO Group 1), a condition in which blood pressure is too high in the blood vessels between the heart and the lungs. VELETRI^® may improve your ability to exercise as measured by how far you can walk in 6 minutes (6-minute walk test).

Studies showing VELETRI^® is effective included mainly patients with New York Heart Association (NYHA) Functional Class III-IV PAH. In these patients, PAH was caused by unidentified or hereditary factors or connective tissue disease.

VELETRI^® should not be used if you have heart failure due to severe left heart disease, if you develop fluid in the lungs (pulmonary edema) when starting therapy, or if you are allergic to epoprostenol.

Some medications may interact with VELETRI^®. Please talk to your healthcare professional about all of your medications.

In a 12-week study, epoprostenol plus conventional therapy was proven to improve survival in New York Heart Association (NYHA) Functional Class III and IV patients with idiopathic or heritable PAH (IPAH/HPAH) versus conventional therapy alone.¹

No statistical difference in survival was observed between patients on epoprostenol plus conventional therapy versus conventional therapy alone in patients with PAH due to connective tissue disease (PAH-CTD).¹

In 8- and 12-week studies of epoprostenol plus conventional therapy, people showed improvements in their ability to exercise (measured by how far they could walk in 6 minutes); some even showed improvement in as early as 1 week.¹ There was also a reduction in symptoms, such as shortness of breath and fatigue.¹

The most common side effects seen in at least 1% of patients were flushing, headache, nausea/vomiting, low blood pressure, anxiety/nervousness, chest pain, dizziness, slow heartbeat, abdominal pain, pain in the muscles and/or ligaments and bones, shortness of breath, back pain, sweating, upset stomach, numbness/increased sensitivity, and fast heartbeat.

The most common side effects in patients with PAH due to unidentified or hereditary factors with at least 10% difference between the group that received epoprostenol and the group that received conventional therapy alone were flu-like symptoms, fast heartbeat, flushing, diarrhea, nausea/vomiting, jaw pain, pain in the muscles and/or ligaments and bones, anxiety/nervousness, dizziness, headache, and numbness/increased sensitivity/tingling.

The most common side effects in patients with PAH due to connective tissue disease (PAH-CTD) with at least 10% difference between the group that received epoprostenol and the group that received conventional therapy alone were flushing, low blood pressure, lack of appetite, nausea/vomiting, diarrhea, jaw pain, neck/joint pain, headache, skin ulcer, and rash.

VELETRI^® is administered continuously for 24 hours via a portable pump.¹ It is delivered through a very thin tube called a catheter into a large vein near the heart. One end of the catheter is surgically implanted in the chest while the other end is attached to a small, portable pump that holds the medication. Your pump should be on and you must carry or wear the pump at all times.^1-2

To avoid potential interruptions in drug delivery, be sure to have access to a backup infusion pump and intravenous infusion sets, including a backup cassette.¹

The amount of VELETRI^® you need on a continuous basis will be determined by your healthcare professional and may change during your course of therapy. Your portable infusion pump will control the rate of medication you receive. Do not, on your own, abruptly lower your dose of VELETRI^® or stop taking it. All dose changes must be closely monitored by your healthcare professional.¹

Vials of VELETRI^® powder must be mixed (reconstituted) and then further diluted with commercially available Sterile Water for Injection, USP, or Sodium Chloride 0.9% Injection, USP. VELETRI^® should not be mixed with any other solutions or medications before or during administration.² Each vial is for single use only; discard any unused solution.

When you start VELETRI^® IV therapy, you have the option to prepare and use your medicine immediately, or to prepare your medicine and store it in the refrigerator (36°F to 46°F/2°C to 8°C) for up to 8 days.

In both cases, your healthcare professional will work closely with you and give you specific directions for preparing, storing, and using your medicine.

Remember, never change the way you prepare or store VELETRI^® unless instructed to do so by your healthcare professional.

VELETRI^® gives you the option of up to once-weekly preparation (up to 8 cassettes at one time can be stored in the refrigerator at a temperature of 36°F to 46°F/2°C to 8°C), and when prepared, stored, and used as directed, does not require ice packs.¹

VELETRI^® must be prescribed by a healthcare professional. It is not available through retail pharmacies; it is only carried by a specialty pharmacy. The specialty pharmacy will ship VELETRI^® and the pump, along with needed supplies, directly and conveniently to your home or healthcare professional’s office each month.

Your healthcare professional and specialty pharmacy will monitor your dose of VELETRI^® while you are on therapy. It is important to use VELETRI^® as directed by your doctor. VELETRI^® should be used only with Sterile Water for Injection, USP, or Sodium Chloride 0.9% Injection, USP. Do not mix VELETRI^® with other intravenous medications. Each vial is for single use only; discard any unused medication. Use at room temperature (77°F/25°C). Do not expose VELETRI^® to direct sunlight.

When you take epoprostenol for the first time, you must be in a setting (hospital or clinic) where you can be monitored for any serious side effects or in case of emergency. Your blood pressure and heart rate should also be monitored with any dose changes. If you are taking VELETRI^®, your doctor may prescribe another kind of medicine used to prevent blood clots. It is the use of these medications concomitantly (in combination) with VELETRI^® that may potentially cause an increase in the risk of bleeding.

Sudden and dramatic changes in dose may lead to unstable blood pressure, a return of pulmonary hypertension symptoms, or fatal low blood pressure. Do not stop using VELETRI^® without first talking to your healthcare professional.

To reduce the risk of infection in the bloodstream, it is important to know how to properly care for the catheter and infusion pump.

Your decision to receive VELETRI^® should be based on the understanding that there is a high likelihood that therapy with VELETRI^® will be needed for prolonged periods, possibly years. Your healthcare professional will carefully consider your ability to accept and care for a permanent intravenous (IV) catheter and infusion pump.¹

You should know how to respond to a catheter-related problem if one occurs. Some problems may include the catheter oozing, leaking, falling out, or developing a crack. If you have a problem with your catheter, see a medical professional as soon as possible or call 9-1-1.³

The Pulmonary Hypertension Association website offers additional tips for how to develop an emergency plan to deal with catheter-related problems.

Your healthcare team is the best source of information for any questions or concerns you may have about your therapy or your condition. They will be able to provide the personal care and attention you need. Specialty pharmacy patient support is also available 24 hours a day to answer any questions you might have about VELETRI^® or PAH. Call Accredo Health Group, Inc., at 1-866-344-4874 or Caremark, L.L.C., at 1-877-242-2738.

If you have any questions about paying for VELETRI^®, contact your healthcare professional or insurance company to review your options. For further information, you can call the specialty pharmacies Accredo Health Group, Inc., or Caremark, L.L.C., who will help answer any questions you may have about VELETRI^® and your insurance. Both pharmacies provide support on reimbursement and pharmacy benefits. Call Accredo Health Group, Inc., at 1-866-344-4874 or Caremark, L.L.C., at 1-877-242-2738.