Clinical Hub,Patient Education,Health and Nutrition Facts For You,Medication Instructions

Pulmonary Arterial Hypertension Medications – Prostacyclin Analogs (6717)

Pulmonary Arterial Hypertension Medications – Prostacyclin Analogs (6717) - Clinical Hub, Patient Education, Health and Nutrition Facts For You, Medication Instructions


Pulmonary Arterial Hypertension Medications-
Prostacyclin Analogs and Prostacyclin Receptor Agonists
Treprostinil (SC or IV Remodulin®), Epoprostenol (IV Flolan®, Veletri®),
Inhaled Iloprost (Ventavis®), inhaled Treprostinil (Tyvaso®), Selexipag (Uptravi®)

The Name of your medicine is: ____________________________.

Prostacyclins and prostacyclin receptor
agonists are used to treat an illness called
Pulmonary Arterial Hypertension (PAH).
There are different types and forms of these
medicines used to manage PAH. The
currently available prostacyclins are given
either intravenously (IV), subcutaneously
(SC), or by inhalation (INH). The currently
available prostacyclin receptor agonist
(Uptravi) is in pill form and can be

These medicines work to open up or dilate
blood vessels. This lowers the pressure
inside the vessel. Lower pressure allows
blood to flow more easily through blood
vessels. Pulmonary arteries are found in the
lungs. The right side of the heart pushes
blood through these arteries to pick up
oxygen. By lowering the pressure, the heart
doesn’t have to work so hard to do this job.
This may delay or prevent right sides heart
failure. Both men and women with PAH can
use this type of medicine. Your doctor will
decide if this type of medicine is right for
you to use.

Please ask your health care team if you have
any questions about this medicine.

Remember to keep this and all other
medicines out of the reach of children.

Never share your medicines with other

Before using this medicine

Tell your health care team if you:
▪ Are allergic to any medicine, either
prescription or over the counter (OTC)
▪ Have congestive heart failure
▪ Are taking any other medicines for
high blood pressure
▪ Have a systolic blood pressure less
than 85 mmHg
▪ Have asthma
▪ Have a lung infection
▪ Have liver or kidney disease

Proper use of this Medicine

Take these medicines exactly as directed by
your doctor. Do not skip doses of the
medicine. Do not stop taking the medicine
without your doctors’ direction.

Because some of these medicines are given
by IV or SC infusion, it is very important to
know your “dosing weight”. This is the
weight used to decide your dose. It may or
may not be your current weight. It is
important to know how to care for your IV
or SC site. Your health care team will teach
you how to use the pump that will deliver

the medicine. They will also teach you how
to mix and store the medicine, if
needed. They will provide all the supplies to
do this. You need to let them know if you
need more supplies or medicine. If the
pump or any supplies fail, call your
doctor right away or report to the nearest
emergency department.
Ask health care team if you have any
questions at any time during the use of your

Usual dose of these medicines

Treprostinil (Remodulin®)
1ng/kg/min continuous IV or SC infusion,
increasing by 1 or 2ng/kg/min per week
until desired response or dose limiting

Epoprostenol (Flolan® or Veletri®)
1-2ng/kg/min continuous IV infusion;
increasing by 2ng/kg/min every 15 minutes
or longer until desired response or dose
limiting effects

Treprostinil (Tyvaso®)
1 to 3 breaths (maximum 9 breaths) 4 times

Iloprost (Ventavis®)
2.5mcg to 5mcg inhaled 6 to 9 times daily
(no more than every 2 hours while awake)

Precautions while using this type of

Your doctor will do a complete history and
physical exam before you begin this
medicine. You will most likely have other
tests, too

This type of medicine may be used along
with other medicine for PAH . It is
important that all members of your
healthcare team know what medicines you
are taking. Medicines for PAH can cause a
decrease in overall blood pressure. It is very
important that everyone on your health care
team knows you are on this medicine. They
need to know that your medicine is being
delivered by a pump (if this applies to you).
You should carry a card with the name and
phone number of the Specialty Pharmacy
that provides your medicine. This should
include your current dose, infusion rate, and
your “dosing weight (for SC or IV

Also carry the dosing sheets provided by the
Specialty Pharmacy. It is important to keep
track of when your pump will be empty or
close to empty. Always carry an extra
supply of PAH medicine with you. It is very
important that you call your health care team
if you have significant lightheadedness or
fainting. It is best to get up slowly from a
lying position to avoid fainting.

Possible side effects from these medicines:

Call your doctor right away if you notice
any of these side effects:
▪ Fever, redness, or swelling near the
infusion site.
▪ Change in heart rate or rhythm
▪ Bleeding that does not stop in a
reasonable amount of time
▪ Lightheadedness or fainting
▪ Large drop in blood pressure
▪ Diarrhea, nausea, or vomiting
▪ Swelling of the ankles, legs or other
areas of the body.
▪ Severe jaw or other pain
▪ Increased shortness of breath or
trouble breathing
▪ Pain and swelling at the site of catheter

Side effects that most often do not need
medical attention:
These side effects may go away during
treatment. If they persist or bother you,
check with your health care team. Do not
stop the medicine suddenly.

▪ Tolerable injection site pain or
infusion site reaction
▪ Headache
▪ Mild diarrhea or nausea
▪ Mild dizziness or itchiness
▪ Congestion

▪ Eczema, facial rash
▪ Body pain
▪ Mild anxiety
▪ Mild flu like illness
▪ Cough (following doses of Ventavis®
or Tyvaso®)

Other side effects not listed above may also
occur in some patients. If you notice any
other side effects, check with your health
care team.

Your health care team may have given you this information as part of your care. If so, please use it and call if you
have any questions. If this information was not given to you as part of your care, please check with your doctor. This
is not medical advice. This is not to be used for diagnosis or treatment of any medical condition. Because each
person’s health needs are different, you should talk with your doctor or others on your health care team when using
this information. If you have an emergency, please call 911. Copyright © 12/2016 University of Wisconsin Hospitals
and Clinics Authority. All rights reserved. Produced by the Department of Nursing HF#6717