Prostacyclin is an synthetic chemical that mimics a substance called prostaglandin that is naturally produced in the body to dilate our blood vessels. Doctors believe that people with pulmonary hypertension do not produce enough prostaglandin. Giving PH patients prostacyclin therefore makes up for this underproduction and helps the body dilate the blood vessels in the lungs. Most PH patients respond well to prostacyclin treatment and it is considered to be the leading therapy for severe PH. Studies show that between 70 and 80% of patients have lower pulmonary pressure and higher cardiac output after using a popular prostacyclin commonly called Flolan.

Although doctors are not certain exactly how Prostacyclin works. It does have several measurable effects.

Prostacyclin dilates blood vessels to some extent and it reduces blood clotting by stopping platelets (in the blood) from sticking together. Researchers believe that an abnormally low ratio of prostacyclin in relation to endogenous thromboxane (a vasoconstrictor that serves the opposite function: it stimulates platelet aggregation) may be partially responsible for the development of primary pulmonary hypertension. Thus prostacyclin improves cardiac output- the blood vessels are cleared out and it may slow the growth of muscle cells that cause these blood vessels to constrict.

Today, intravenous prostacyclin infusions are used to treat patients who fail to respond to oral calcium antagonists, and it is usually used as long-term therapy for people with pulmonary hypertension. As stated above prostacyclin has been shown to improve blood circulation, allow exercise and extend the life of PPH patients who have been unresponsive to conventional therapy.

A downside to the treatment is that prostacyclin expires quickly and must be infused continuously by an automated syringe pump and through a permanent catheter. Also, the delivery system is complex and requires patients to learn the techniques of sterile preparation, operation of the pump, and care of the intravenous catheter. In some cases this type of infusion may create serious complications (e.g., sepsis, thromboembolism, or syncope) related to the use of an implanted central vein catheter.

Trade names for Prostacyclin include Flolan, Remodulin (also called UT-15 and Uniprost), Iloprost and Beraprost.

Remodulin (Treprostinil sodium), is a stable form of prostacyclin is an alternative that does not require a catheter. Remodulin lasts longer than most prostacyclins like Flolan and is injected subcutaneously (under the skin) with a needle. This method of infusion greatly reduces the risk of sepsis infection and hospitalization, but many patients report that the injections are very painful.

Generally prostacyclin is a very expensive treatment, but it is the most promising treatment developed for patients with end stage pulmonary hypertension.


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