Tracleer
Tracleer , or Bosentan, is an alternative therapy to the more invasive prostacyclin agents Flolan and Remodulin. This drug is taken orally and is the first oral PPH therapy to get full FDA approval in the US. In tests and clinical trials the drug proved its effectiveness by improving arterial capacity and reducing pressure in the blood vessels of the lungs.
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Remodulin
Remodulin (treprostinil sodium) also called UT-15 and Uniprost, is a form of prostacyclin, a very powerful vasodilator that helps most PPH patients that take it. The drug is made by United Therapeutics, Inc. and was approved by the FDA for use within the United States in May 2002. Remodulin is only the second FDA approved treatment (after Flolan) for PPH patients.

Remodulin is injected through a catheter into the patient, usually near the gut. The drug is slowly absorbed after being injected into fat cells. It then works it way to the blood vessels of the lung, relaxing them enabling the patient to breath easier. Some researchers believe it may also slow the PPH scarring process.

Remodulin has several advantages over Flolan treatment.

1) Remodulin is stable at room temperature for about 3 months (although it should be kept refrigerated), while Flolan requires an ice pack.

2) Remodulin comes packaged and does not need to be mixed. Flolan, which comes as a freeze-dried powder, needs to be mixed each day.

3) The Remodulin pump is a small pager size item, makes little noise and can be placed in a waterproof pack. Flolan requires a larger pump packed with ice and makes a whirling noise.

4) Remodulin lasts longer in the body than Flolan does. Remodulin stays in the body for several hours after injection, making a sudden interruption in treatment less severe than Flolan. Flolan only lasts a few minutes and must be constantly infused.

5) Perhaps the biggest advantage is that Remodulin, avoids the dangerous infections that can arise from the indwelling Flolan catheter.

The downside is that the injections are extremely painful. Other side effects can include jaw pain, foot cramps and flushing. The drug is very expensive, with the reported wholesale price at $65 per milligram, the treatment runs well into the tens of thousands of dollars.

Beraprost
Beraprost is an oral form of prostacyclin currently being developed by United Therapeutics Corporation. Currently in Phase III clinical trials in the United States and Canada, Beraprost is intended to be used in early stages of pulmonary hypertension and peripheral vascular disease only as oral doses of Beraprost do not provide the continuous, consistent levels of prostacyclin required to treat PPH. United Therapeutics Corporation has commenced a 100+ patient study of Beraprost for pulmonary hypertension. In Japan, small, uncontrolled studies have led to Beraprost being approved for the treatment of primary pulmonary hypertension.

Ilioprost
Iloprost is an inhalable form of prostacyclin, an analog (imitator) of our body's own vasodialator, prostaglandin. Prostaglandin prevents blood clots, and researchers believe that people with PH do not have enough of it. The advantage of inhalation, is that the drug is introduced only to the lungs so that side effects that might affect the rest of the body are avoided. Prostacyclins have been shown to improve survival rate, exercise capacity, and hemodynamics (blood circulation) of patients with severe PPH.

Like Flolan, Iloprost is very similar to naturally occurring prostaglandin with regard to its effect on blood vessels. When used intravenously its effects as well as its side effects are nearly identical to those of Flolan. Ilioprost's chemical stability, however, is considerably superior. Iloprost is stable at room temperature and is resistant to normal light, while in contrast, Flolan has to be freshly dissolved, continuously cooled, and protected from light to preserve its full effectiveness.

Iloprost is effective and reduces shortness of breath, and may lower pulmonary pressures according to an August 2002 study that appeared in the New England Journal of Medicine. So far Iloprost has only been approved for treatment of pulmonary arterial hypertension (PAH) in a few countries, and is not available for Primary Pulmonary Hypertension in the United States except through clinical trial.