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Diagnosing PAH

PAH can develop slowly. The early symptoms of PAH can be mild and vague, and are often similar to those of other heart and lung conditions, such as asthma. This makes diagnosing PAH difficult. Many people go through years of tests to rule out other conditions before finally getting diagnosed. It is important to be diagnosed as early as possible, so you can begin treatment.⁹

There are several tests your doctor may use to check your lungs and heart and diagnose PAH:⁹

• Physical exam
• Chest x-ray
• Electrocardiogram (EKG)
• Echocardiogram (echo)
• Pulmonary function testing (PFT)

Test to Confirm Diagnosis: Right Heart Catheterization

Once PAH is suspected, a diagnosis can only be confirmed with a procedure known as right heart catheterization.¹⁰ Right heart catheterization involves inserting a long, thin, flexible tube called a catheter through the blood vessels and into the heart. This procedure allows your doctor to test heart function and blood pressure in the heart and pulmonary artery. It also helps evaluate the severity of PAH.⁹

If you believe you have PAH but haven’t been diagnosed, be persistent. The earlier PAH is diagnosed, the sooner you can begin treatment.

Functional Classifications of PAH

Once you've been diagnosed with PAH, your doctor may describe the severity of your disease using a well-known functional classification system created by the New York Heart Association (NYHA).⁵

The World Health Organization (WHO) developed a similar functional classification system that is also commonly used in assessing patients with PAH.⁵

Indication

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

• 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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