PAH Overview

PAH is a progressive disease1

Pulmonary arterial hypertension (PAH; WHO Group 1) is a serious, complex, often fatal illness.1 It is a rare disease, affecting 15 out of 1 million adults.1,2 It is characterized by remodeling of the small-to-medium pulmonary arteries, leading to restricted blood flow and increased pulmonary vascular resistance.1

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PAH affects the blood vessels, lungs, and heart1,3

In PAH, narrowing of the arteries in the lungs causes increased pulmonary arterial pressure and vascular resistance.1 The right ventricle must work harder to pump blood through narrowed arteries.3 This right ventricle compensation may go unnoticed early in the disease.1

Increases in pulmonary vascular resistance cause morphologic and functional changes in the right ventricle.3 Unlike the left ventricle, the right ventricle cannot sustain long-term increases in pressure.3,4 Right ventricular failure is the primary cause of death in patients with PAH.5



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 for Remodulin

Warnings and Precautions
  • Chronic intravenous (IV) 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 (SC) infusion is the preferred mode of administration.
  • Avoid abrupt withdrawal or sudden large reductions in dosage of Remodulin, which may result in worsening of PAH symptoms.
  • Titrate slowly in patients with hepatic or renal insufficiency because such patients will likely be exposed to greater systemic concentrations relative to patients with normal hepatic or renal function.
  • Remodulin dosage adjustment may be necessary if inhibitors or inducers of CYP2C8 are added or withdrawn. Co-administration of Remodulin with a CYP2C8 inhibitor increases exposure to treprostinil, or with an inducer, decreases exposure to treprostinil.
Drug Interactions/Specific Populations
  • Remodulin is a potent pulmonary and systemic vasodilator. Concomitant administration of Remodulin with blood pressure lowering agents, such as diuretics, antihypertensive agents, or other vasodilators, may increase the risk of symptomatic hypotension.
  • Since Remodulin inhibits platelet aggregation, there may be an increased risk of bleeding, particularly among patients receiving anticoagulants.
  • Safety and effectiveness of Remodulin in pediatric patients have not been established. It is unknown if geriatric patients respond differently than younger patients. Caution should be used when selecting a dose for geriatric patients.
  • There are no adequate and well-controlled studies with Remodulin in pregnant women. It is not known whether treprostinil is excreted in human milk.
Adverse Reactions
  • Adverse Reactions: In clinical studies of SC Remodulin infusion, the most common adverse events reported were infusion site pain and infusion site reaction (redness and swelling). These symptoms were often severe and sometimes required treatment with narcotics or discontinuation of Remodulin. The IV infusion of Remodulin has been associated with a risk of blood stream infections, arm swelling, paresthesias, hematoma, and pain. Other common adverse events (≥3% more than placebo) seen with either SC or IV Remodulin were headache, diarrhea, nausea, jaw pain, vasodilatation, and edema.

Please click here for Full Prescribing Information for Remodulin.

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  1. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association. J Am Coll Cardiol. 2009;53(17):1573-1619.
  2. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023-1030.
  3. Badano LP, Ginghina C, Easaw J, et al. Right ventricle in pulmonary arterial hypertension: haemodynamics, structural changes, imaging, and proposal of a study protocol aimed to assess remodelling and treatment effects. Eur J Echocardiogr. 2010;11(1):27-37.
  4. Voelkel NF, Quaife RA, Leinwand LA, et al. Right ventricular function and failure: report of a National Heart, Lung, and Blood Institute working group on cellular and molecular mechanisms of right heart failure. Circulation. 2006;114(17):1883-1891.
  5. McLaughlin VV, Gaine SP, Howard LS, et al. Treatment goals of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 suppl D):D73-D81.