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What are common medication side effects? What effects should I report?

Prostacyclins as a class all have several side effects that are important to know about. These include jaw pain (sometimes this is most noticeable on the first bite), diarrhea, joint aches, flushing, leg and/or back pain, and nausea. In addition, epoprostenol (Flolan® and Veletri) and treprostinil (Remodulin®) are infused via an indwelling central venous catheter (a permanent "IV line"), which carries its own adverse effects, such as thrombosis and infection of the catheter. Pain or redness at the site of your indwelling catheter is something that is important to watch for and tell your medical team about immediately. For patients who are receiving the subcutaneous form of treprostinil (Remodulin®), most patients will develop pain, redness, and swelling at the site of their infusion. This is a very common adverse effect and there are a number of gels and topical solutions for this problem that your doctor can prescribe for you. The inhaled prostacyclins including iloprost (Ventavis®) and treprostinil (Tyvaso®) can also cause similar side effects if used at higher doses. In clinical trials of the oral form of treprostinil (Orenitram®), the most commonly reported side effects were headache, nausea and diarrhea.

Endothelin receptor antagonists, such as bosentan (Tracleer®), ambrisentan (Letairis®) and macitentan (Opsumit®) can cause leg swelling and other types of fluid retention. Checking your weight daily is an important part of monitoring your response to medications. These medications are metabolized through your liver and sometimes can cause irritation of the liver resulting in laboratory (blood) tests specific for the liver to be too high (called transaminitis). This is an important adverse effect of some of these medications, so your liver tests must be monitored.

Female patients of childbearing age must also document through blood tests that they are not pregnant because endothelin receptor antagonists are not safe in pregnancy. Similarly, any female patient of childbearing age on endothelin receptor antagonists should be counseled to use two forms of birth control if they are having sexual intercourse while on these medications. Endothelin receptor antagonists can cause nasal congestion and they can interact with other medications so your doctor will need to know all the medications, over the counter preparations, and supplements or herbal medications that you take to ensure no reactions.

Phosphodiesterase inhibitors such as sildenafil (Revatio®) and tadalafil (Adcirca®) can cause headache and changes in your vision. It is very important that these medications not be taken in combination with nitrates. If you are taking any sort of nitrate preparation (like sublingual nitroglycerin), you should not be on a medication like sildenafil or tadalafil.

The soluble guanylate cyclase stimulator riociguat (Adempas®) has been reported to cause side effects including headache, dizziness, nausea, vomiting, tissue swelling (edema), indigestion (dyspepsia), and diarrhea. This medication can also cause harm to a developing fetus so similar warnings as described above regarding females of childbearing age apply to the use of this medication as well.

Calcium channel blockers are less specific for the pulmonary circulation than other medicines for PAH. Therefore, low systemic blood pressure (hypotension) is a more common side effect of calcium channel blockers than other PAH medications. Specific side effects of calcium channel blockers include leg swelling.

Any of these medications that lower pulmonary artery pressures can also lower your systemic blood pressure. If your systemic blood pressure is low enough, you can feel lightheaded, dizzy, or weak. Different people tolerate the same blood pressure differently, so it can be difficult to predict at what systemic blood pressure you may feel these symptoms.