Treatment Advancements

A better understanding. More treatment options.

The understanding of () and the number of treatment options has grown over the past two decades. PAH-specific treatment has advanced from one treatment approved in 1996, to nine approved options available in 2012. Every advancement towards better diagnostic tools may offer new hope for people living with PAH.

Three treatment pathways of PAH

In PAH, there is an imbalance of 3 different substances in the body that are responsible for helping to keep the blood vessels open. These imbalances can cause the blood vessels — specifically those in the lungs — to narrow, leading to PAH.

Prostacyclin, endothelin, and nitric oxide are three chemicals naturally produced in the body. When the levels of these chemicals become imbalanced, blood vessels in the lungs can narrow, leading to PAH.

Three types of medicine are approved to treat PAH (). Each one corrects the imbalance in the body of one of those substances. You may hear the treatments referred to as:

  • (phosphodiesterase type 5 inhibitors)
  • (endothelin receptor antagonists)

These treatments work to dilate (open) blood vessels, making it easier for the heart to pump blood to the lungs.

Prostacyclin class therapy is a PAH treatment

Prostacyclin is a natural substance found in the body. It helps keep arteries in the lungs open and working properly. But, if you have PAH, your body may not produce enough prostacyclin.

Prostacyclin class therapy medications mimic the effects of the natural prostacyclin that your body lacks, making it easier for your heart to pump blood through your lungs.


Remodulin is a prostacyclin vasodilator indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to diminish symptoms associated with exercise. Studies establishing effectiveness included patients with NYHA Functional Class II-IV symptoms and etiologies of idiopathic or heritable PAH (58%), PAH associated with congenital systemic-to-pulmonary shunts (23%), or PAH associated with connective tissue diseases (19%). It may be administered as a continuous subcutaneous infusion or continuous intravenous infusion; however, because of the risks associated with chronic indwelling central venous catheters, including serious blood stream infections, continuous intravenous infusion should be reserved for patients who are intolerant of the subcutaneous route, or in whom these risks are considered warranted.

In patients with PAH requiring transition from Flolan® (epoprostenol sodium), Remodulin is indicated to diminish the rate of clinical deterioration. The risks and benefits of each drug should be carefully considered prior to transition.

Important Safety Information

  • Chronic intravenous infusions of Remodulin are delivered using an indwelling central venous catheter. This route is associated with the risk of blood stream infections (BSI) and sepsis, which may be fatal. Therefore, continuous subcutaneous infusion is the preferred mode of administration
  • Remodulin should be used only by clinicians experienced in the diagnosis and treatment of PAH
  • Remodulin is a potent pulmonary and systemic vasodilator. It lowers blood pressure, which may be further lowered by other drugs that also reduce blood pressure
  • Remodulin inhibits platelet aggregation and therefore, may increase the risk of bleeding, particularly in patients on anticoagulants
  • Remodulin dosage adjustment may be necessary if inhibitors or inducers of CYP2C8 are added or withdrawn
  • Initiation of Remodulin must be performed in a setting with adequate personnel and equipment for physiological monitoring and emergency care
  • Therapy with Remodulin may be used for prolonged periods, and the patient’s ability to administer Remodulin and care for an infusion system should be carefully considered
  • Remodulin dosage should be increased for lack of improvement in, or worsening of, symptoms and it should be decreased for excessive pharmacologic effects or for unacceptable infusion site symptoms
  • Abrupt withdrawal or sudden large reductions in dosage of Remodulin may result in worsening of PAH symptoms and should be avoided
  • Caution should be used in patients with hepatic or renal insufficiency
  • The most common side effects of Remodulin included those related to the method of infusion. For subcutaneous infusion, infusion site pain and infusion site reaction (redness and swelling) occurred in the majority of patients. These symptoms were often severe and could lead to treatment with narcotics or discontinuation of Remodulin. For intravenous infusion, line infections, sepsis, arm swelling, tingling sensations, bruising, and pain were most common. General side effects (>5% more than placebo) were diarrhea, jaw pain, vasodilatation, and edema

For more information about REMODULIN, please see the Full Prescribing Information.
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