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Pulmonary Arterial Hypertension

(Primary Pulmonary Hypertension)



PAH/PPH Treatment Options

There are two types of treatment options for pulmonary arterial hypertension (PAH), also known as primary pulmonary hypertension (PPH):
Drugs and lung transplantation.


Prostacyclin analogues
These medicines enable the vessels in the lungs to expand and allow the blood to move through them with less resistance (vasodilation). The primary drugs in this category are prostacyclin, which is given as a continuous intravenous infusion, and treprostinil, which is given as a continuous infusion under the skin.

Endothelin receptor antagonists
These drugs, in pill form, help to reverse the effect of endothelin, a substance in blood vessels that causes the vessels to constrict. As a result, these drugs also produce vasodilation. Bosentan or Tracleer is the endothelin receptor antagonist approved for use in pulmonary hypertension; others are being studied.

High-dose calcium channel blockers
For some patients, these medications also help to relax the muscles in the blood vessels, and promote vasodilation.

These drugs help to prevent the blood from clotting.

These medications help to remove excess fluid from body tissues which may accumulate due to high pressures in blood vessels.

With a higher concentration of oxygen in the air, more oxygen enters the bloodstream even though blood flow is reduced.

Many drugs for treating primary pulmonary hypertension or pulmonary arterial hypertension have strong, serious side effects. The level of dosage must be carefully set and monitored to avoid dangerous consequences.

Common Drugs and Medications

Remodulin (treprostinil sodium) Injection is a man-made chemical cousin (analogue) to prostacyclin (a naturally occurring chemical in the body that helps to regulate the size or diameter of the blood vessels). Remodulin is given as a continuous subcutaneous (into the skin) infusion by a small portable electronic infusion pump. Remodulin is used to diminish or reduce symptoms associated with exercise.

Flolan vs. Remodulin
Flolan is chemically similar to Remodulin but they are not "generic" or identical. Both are prostacyclins, and both are injections requiring an electronic portable pump. Flolan is given by a continuous (24 hours a day, 7 days a week) intravenous infusion into a surgically placed catheter (tube) into a vein under the collarbone while Remodulin is given by a continuous infusion directly into the skin (subcutaneous).

Although both therapies use a portable infusion pump, the pump used for Flolan therapy is fairly large and not easy to conceal. Also, if the medication container (called a cassette) is to be used for more than 8 hours, ice packs need to be placed into the pump's carry pouch to keep Flolan cold. This adds to the weight of the set-up.

The infusion pump for Remodulin is small, about the size of a pager/beeper, is light weight, and is easily concealed under clothing. Since Remodulin is stable at room temperature, there is no need for ice packs or other refrigerants. With special devices that go over the pump, patients can shower or bathe.

Tracleer (bosentan)
Tracleer improves ones breathing and exercise capacity. In two research studies involving about 250 people, improvement in how far patients receiving Tracleer could was noticeable on average four weeks after beginning therapy and maximal by eight weeks.

The major reported side effects of Tracleer(bosentan) include: Nasopharyngitis (nasal stuffiness), flushing, headache, hypotension (low blood pressure), syncope (passing out), and abnormal elevation of liver function blood tests. Clinically, the most important side effect has been elevation of liver function tests and as a result, liver function tests should be monitored monthly in patients receiving Tracleer. It should be noted that no patient has yet developed permanent liver damage, and so far, all abnormal liver function tests have returned to normal after discontinuing Tracleer.

Tracleer(bosentan) has been shown to be teratogenic (harmful to fetuses) of pregnant animals; therefore women of childbearing potential should not become pregnant while using Tracleer. Pregnancy tests should be obtained monthly. While testicular atrophy (shrinkage of testes) in animals has been reported for other endothelin blockers, this has not been reported for Tracleer. However, we do not yet know if long-term Tracleer use in males will cause sterility.

There are no guidelines at this time for switching from UT-15 or Flolan to Tracleer. This is being considered in only a small number of patients and should be done only by a pulmonary hypertension expert. Patients starting Tracleer should not expect to discontinue UT-15 or Flolan, and, as with other aspects of Tracleer use, this should be determined on a case-by-case basis.

Iloprost (Ventavis). A new form of the prostacyclin drug, iloprost (Ventavis), was approved for the treatment of PAH. This alternative medicine can be inhaled through a nebulizer. This makes it more convenient and less painful to take. In addition, the medicine goes directly to the lungs, where it is needed.

Sildenafil (Viagra), another drug that causes the pulmonary arteries to open, has recently been shown to improve the condition of PAH patients and is expected to be approved soon. This drug is available in pill form.

Revatio is a medicine called a phosphodiesterase type 5 (PDE5) inhibitor with the same ingredients as Viagra. In studies, Revatio increased the distance people walked and decreased pressure in the pulmonary artery. Revatio was not studied in patients taking bosentan (a medicine used in patients with PAH) so consult with your doctor. Revatio works by controlling how narrow or wide the blood vessels in the lining of the lungs can be by helping the blood vessels relax so blood and oxygen can then flow more freely.

Ambrisentan is an endothelin selective receptor antagonist being developed as an oral therapy for patients with pulmonary arterial hypertension. Treatment with ambrisentan appear to be helpful for patients with pulmonary arterial hypertension (PAH). In a recent study, patients were able to increase distance in the 6 minute walk test. Click Here for detailed information on the recent alternative medicine study.

Lung Transplantation
The first heart-lung transplant was performed in this country in 1981. Many of these operations were performed for patients with PAH/PPH. The survival rate is the same as for other patients with heart-lung transplants, about 60 percent for 1 year, and 37 percent for 5 years.

The single lung transplant is the most common method of transplant used in cases of PAH/PPH. This procedure, in which one lung--either the left or right--is replaced, was first performed in 1983 in patients with pulmonary fibrosis. Double lung transplants are also done to treat PPH, but are less common than the single lung transplant for treatment of pulmonary arterial hypertension or PPH.

There are fewer complications with the single lung transplant than with the heart-lung transplant, and the survival rate is on the order of 70 percent for 1 year. A surprising finding is the remarkable ability of the right ventricle to heal itself. In patients with lung transplants, both the structure and function of the right ventricle markedly improve.

Complications of lung transplants include rejection by the body of the transplanted organ, and infection. Patients take medications for life to reduce their body's immune system's ability to reject "foreign" organs.

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