Talk to your health care provider or specialty pharmacist about side effects and interactions before starting new medications. 

Inform your medical team about all medications and supplements you take, including:

  • Antibiotics

  • Vitamins

  • Herbal supplements

  • Over-the-counter medications (like ibuprofen and acetaminophen)

It’s important to prevent interactions with your PH medications and avoid worsening your condition. Please remember to keep a list of current medications with you and always take medications as prescribed.

Managing your PH medications

Oral medications

Endothelin receptor antagonists

These ERAs are approved for pulmonary arterial hypertension.

  • Headache
  • Flushing
  • Peripheral edema (more common with ambrisentan)
  • Anemia
  • Nasal congestion or sinusitis
  • Nosebleeds
  • Decreased sperm count
  • Decreased appetite and metallic taste in mouth
  • Liver toxicity (primarily bosentan)
  • Possible fetal harm

Bosentan (Tracleer)

  • Leg and abdominal swelling associated with weight gain 

Ambrisentan (Letairis)

  • Yellow skin and eyes 
  • Recurring sinus infections 

Macitentan (Opsumit)

  • Easy bruising
  • Increased nosebleeds
  • Cyclosporine
  • Glyburide
  • rifampin
  • ketoconazole
  • ritonavir

 

Endothelin receptor antagonists

These ERAs are approved for pulmonary arterial hypertension.

  • Headache
  • Flushing
  • Peripheral edema (more common with ambrisentan)
  • Anemia
  • Nasal congestion or sinusitis
  • Nosebleeds
  • Decreased sperm count
  • Decreased appetite and metallic taste in mouth
  • Liver toxicity (primarily bosentan)
  • Possible fetal harm

Bosentan (Tracleer)

  • Leg and abdominal swelling associated with weight gain 

Ambrisentan (Letairis)

  • Yellow skin and eyes 
  • Recurring sinus infections 

Macitentan (Opsumit)

  • Easy bruising
  • Increased nosebleeds

  • Cyclosporine
  • Glyburide
  • rifampin
  • ketoconazole
  • ritonavir

 

Why ERAs require monitoring

ERAs are harmful to fetuses, so women of childbearing age should use contraceptives to avoid pregnancy. Patients must undergo pregnancy testing before starting treatment, every month during treatment and a month after stopping medication. Patients will be asked for confirmed negative test results before starting treatment or scheduling refill shipments.

Macitentan, bosentan and ambrisentan can affect blood counts, causing anemia. Therefore, your provider must check your blood counts before and during treatment.

Bosentan can cause liver damage, so monthly liver function blood tests are mandatory to continue therapy. Expect your pharmacy to ask if your monthly lab tests are complete before shipping refills. Macitentan and ambrisentan are less likely to cause liver damage so your provider might test liver function before therapy starts and as needed.

Phosphodiesterase Type 5 inhibitors

These medications are approved for PAH.

  • Headache
  • Heartburn
  • Low blood pressure
  • Back and muscle pain
  • Increased likelihood of erection
  • Vision or hearing problems
  • Seek emergency medical assistance for erection lasting more than four hours.

Do not take nitrates (nitroglycerine, isosorbide), nitric oxide donors, alpha blockers (terazosin, doxazosin, prazosin) or riociguat (Adempas) when taking tadalafil of sildenafil.

Phosphodiesterase Type 5 inhibitors

These medications are approved for PAH.

  • Headache
  • Heartburn
  • Low blood pressure
  • Back and muscle pain
  • Increased likelihood of erection

  • Vision or hearing problems
  • Seek emergency medical assistance for erection lasting more than four hours.

Do not take nitrates (nitroglycerine, isosorbide), nitric oxide donors, alpha blockers (terazosin, doxazosin, prazosin) or riociguat (Adempas) when taking tadalafil of sildenafil.

Soluble guanylate cyclase stimulator

This medication is approved for PAH and chronic thromboembolic pulmonary hypertension (CTEPH).

  • Low blood pressure
  • Dizziness
  • Heartburn
  • Nausea/vomiting
  • Anemia
  • Nose bleeds
  • Low blood pressure
  • Dizziness
  • Nosebleeds
  • Coughing blood
  • Nitrates (nitroglycerine, isosorbide)
  • Nitric oxide donors
  • Alpha blockers (terazosin, doxazosin, prazosin)
  • Inhibitors (tadalafil, sildenafil)

Soluble guanylate cyclase stimulator

This medication is approved for PAH and chronic thromboembolic pulmonary hypertension (CTEPH).

  • Low blood pressure
  • Dizziness
  • Heartburn
  • Nausea/vomiting
  • Anemia
  • Nose bleeds

  • Low blood pressure
  • Dizziness
  • Nosebleeds
  • Coughing blood

  • Nitrates (nitroglycerine, isosorbide)
  • Nitric oxide donors
  • Alpha blockers (terazosin, doxazosin, prazosin)
  • Inhibitors (tadalafil, sildenafil)

Why riociguat requires monitoring

Riociguat is harmful to fetuses, so women of childbearing age should use sufficient contraceptives to avoid pregnancy. Patients must undergo pregnancy testing before starting treatment, every month during treatment and a month after stopping medication. Patients will be asked for confirmed negative test results before starting treatment or scheduling refill shipments.

Oral prostacyclin

This medication is approved for PAH.

  • Headache
  • Diarrhea
  • Nausea
  • Vomiting
  • Jaw pain
  • Muscle pain
  • Flushing
  • Uncontrollable vomiting and/or inability to keep anything down
  • Dizziness
  • Low blood pressure

Oral treprostinil (Orenitram)

  • Gemfibrozil 

Selexipag (Uptravi)

  • Gemfibrozil
  • Clopidogrel
  • Rifampin

Oral prostacyclin

This medication is approved for PAH.

  • Headache
  • Diarrhea
  • Nausea
  • Vomiting
  • Jaw pain
  • Muscle pain
  • Flushing

  • Uncontrollable vomiting and/or inability to keep anything down
  • Dizziness
  • Low blood pressure

Oral treprostinil (Orenitram)

  • Gemfibrozil 

Selexipag (Uptravi)

  • Gemfibrozil
  • Clopidogrel
  • Rifampin

Warning: Do not split, chew, crush, or break. Do not abruptly discontinue medication. 

Caution: In patients with diverticulosis, oral treprostinil (Orenitram) tablets can become lodged in a pouch in the bowel wall (diverticulitis). 

Inhaled therapies

Inhaled prostacyclin therapies.

Iloprost and treprostinil

  • Flushing
  • Cough
  • Headache
  • Throat pain
  • Nausea
  • Diarrhea
  • Low blood pressure
  • Vomiting
  • Muscle pain
  • Jaw pain
  • Tongue pain
  • Palpitations
  • Fainting
  • Muscle cramps

Yutrepia

  • Low blood pressure
  • Bleeding
  • Cough/Throat irritation
  • Bronchospasm (difficulty breathing, coughing and wheezing)
  • Chest discomfort
  • Oropharyngeal pain
  • Diarrhea
  • Nausea
  • Flushing
  • Headache

Iloprost

  • Excessive diarrhea
  • Nausea
  • Vomiting

Treprostinil

  • Dizziness
  • Low blood pressure

Yutrepia

  • Dizziness 
  • Low blood pressure 
  • Bronchospasms 

Iloprost and treprostinil

  • Discuss with your PH provider before starting a new medication. 

Inhaled therapies

Inhaled prostacyclin therapies.

Iloprost and treprostinil

  • Flushing
  • Cough
  • Headache
  • Throat pain
  • Nausea
  • Diarrhea
  • Low blood pressure
  • Vomiting
  • Muscle pain
  • Jaw pain
  • Tongue pain
  • Palpitations
  • Fainting
  • Muscle cramps

Yutrepia

  • Low blood pressure
  • Bleeding
  • Cough/Throat irritation
  • Bronchospasm (difficulty breathing, coughing and wheezing)
  • Chest discomfort
  • Oropharyngeal pain
  • Diarrhea
  • Nausea
  • Flushing
  • Headache

Iloprost

  • Excessive diarrhea
  • Nausea
  • Vomiting

Treprostinil

  • Dizziness
  • Low blood pressure

Yutrepia

  • Dizziness 
  • Low blood pressure 
  • Bronchospasms 

Iloprost and treprostinil

  • Discuss with your PH provider before starting a new medication. 

Warning for inhaled therapies

Don’t abruptly discontinue medication. Consult your PH care team.

Follow device cleaning requirements daily.

Infused therapies

Intravenous epoprostenol

  • Nausea
  • Vomiting
  • Headache
  • Jaw pain
  • Low blood pressure
  • Flushing
  • Chest pain
  • Dizziness
  • Low heart rate
  • Shortness of breath
  • Abdominal pain
  • Musculoskeletal pain
  • Increased heart rate
  • Flu-like symptoms
  • Anxiety/nervousness
  • Uncontrollable vomiting and/or inability to keep anything down
  • Dizziness
  • Low blood pressure
  • Fever

Discuss with your PH provider before starting a new medication.

Infused therapies

Intravenous epoprostenol

  • Nausea
  • Vomiting
  • Headache
  • Jaw pain
  • Low blood pressure
  • Flushing
  • Chest pain
  • Dizziness
  • Low heart rate
  • Shortness of breath
  • Abdominal pain
  • Musculoskeletal pain
  • Increased heart rate
  • Flu-like symptoms
  • Anxiety/nervousness

  • Uncontrollable vomiting and/or inability to keep anything down
  • Dizziness
  • Low blood pressure
  • Fever

Discuss with your PH provider before starting a new medication.

Monitoring for infused therapies

Watch for pump or catheter malfunction; infection signs, including fever, chills, more than usual fatigue; warmth, pain and redness at catheter insertion site.

Warning for infused therapies

Don’t abruptly lower dose or withdraw dosing. If infusion is inadvertently disrupted, immediately contact your PH provider about how to restart infusion. Depending on the length of interruption, you might have to restart infusion at a lower rate and increase at frequent intervals until you reach pre-interruption rate. Restarting at current rate could cause intense and intolerable side effects.

Activin signaling inhibitor

* Low platelet counts that increases risk of bleeding (Nosebleeds, gastrointestinal bleeding, intracranial bleeding) Particularly with concurrent use of prostacyclin, blood thinners, ASA

  • Increase red blood cells (increasing risk to blood clots).
  • Headaches
  • Rash
  • Telangiectasia
  • Dizziness
  • Weight gain (May be linked to fluid retention)
  • Bleeding of any kind
  • Blood clots
  • Pregnancy

No noted interaction has been reported with any other drugs.

Activin signaling inhibitor

* Low platelet counts that increases risk of bleeding (Nosebleeds, gastrointestinal bleeding, intracranial bleeding) Particularly with concurrent use of prostacyclin, blood thinners, ASA

  • Increase red blood cells (increasing risk to blood clots).
  • Headaches
  • Rash
  • Telangiectasia
  • Dizziness
  • Weight gain (May be linked to fluid retention)

  • Bleeding of any kind
  • Blood clots
  • Pregnancy

No noted interaction has been reported with any other drugs.

Monitoring for sotatercept

Before starting your first dose of sotatercept, your doctor will order a complete blood cell count test to check your levels of hemoglobin (protein in red blood cells that carries oxygen) and platelets (blood cells that help blood clot).

You will need a complete blood cell count test every 21 days before each dose. Your sotatercept dose may need to be adjusted if your hemoglobin level is too high and/or platelet count is too low. Your doctor may delay your next injection or change the dose. Patients should contact their physicians to discuss.

*Laboratory monitoring is recommended for at least the first five doses. You might need more frequent lab tests if your results are abnormal.

Warning for sotatercept

Winrevair is harmful to fetuses, so women of childbearing age should use effective contraception while receiving the drug and for at least 4 months after the final dose. Women should not breastfeed during treatment with Winrevair and for 4 months after the final dose.

Managing your PH medications and side effects

  • Best Practices in Catheter Care

    If you are receiving medication through a pump, it’s important to prevent catheter-related infections and what to do in case of an emergency.

  • Managing Nausea and Vomiting

    Nausea and vomiting can be side effects of some pulmonary hypertension drugs. Here’s what you can do. Since these symptoms can also be caused by heart failure common in PH, let your medical team know what you are experiencing.