PPH is treated in a number of effective ways. While there is no cure for PPH, the effects of the disease can be minimized. New treatments, notably vasodialators such as prostacyclin, discovered in recent years have made significant improvements in the lives of PPH patients. Before the development of these drug treatments, PPH was rapidly fatal with nearly two thirds of patients having only three years after the date of diagnosis. After the development of these new therapies more than 65% of patients will survive longer than 5 years.

The most significant advances were made in the late 1990s by Sir John Vane, a Nobel Prize winner, whose work with prostaglandins has revolutionized PPH treatments. These treatments have focused on very specific chemicals the body uses to control the behavior of the lung's blood vessels.

Course of Treatment

After a positive diagnosis of PPH, doctors will usually follow a PPH management program (developed by researchers Dr. Rubin and Rich). The treatment will proceed in stages depending on the patient's response. The initial stages consist of evaluating the extent of the PPH by measuring the vasoreactivity or degree to which the pulmonary arteries can be dilated or opened with drug therapy. The patient will be given known vasodilators: inhalation of nitric oxide or given intravenous prostacyclin or adenosine.

If the these treatments create a notable fall in pulmonary vascular resistance the patient will be given oral calcium channel blockers and anticoagulants which help control pulmonary pressure. If the fall in pressure is moderate or minimal the patient will be given higher doses and more potent vasodilators and may be a candidate for a heart and lung transplant.

Drug Therapy

Anti Coagulation Agents
Most PPH studies, have shown longer survival rates when patients are treated with anticoagulant therapy. Agents like Warfarin are commonly used to prevent pulmonary blood clotting and scarring. The blood is thinner and can travel through the constricted pulmonary blood vessels with less resistance.

Calcium Channel Blockers (CCBs)
CCBs are considered to be a more conventional oral vasodilator therapy. CCBs are the most widely used type of drugs used to treat PPH. Doctors believe that these drugs work by triggering the vascular smooth muscles in the lungs to open, which lowers resistance and in turn lowers the pulmonary artery pressure. CCBs nearly always bring about a reduction in pulmonary vascular resistance by lowering pulmonary artery pressure and thereby increasing the hearts output of needed blood flow.

New Drugs

The three main pulmonary vasodilator therapies currently used are:
Epoprostenol (Flolan)
Treprostinil (Remodulin)
Bosentan (Tracleer)

These substances are widely hailed as major advances in PPH treatment. These molecules work by triggering a reaction in the tiny vascular endothelium cells (see What is PPH) and cause pulmonary vasodilation (opening of the lung blood vessels).

Flolan or Epoprostenol was approved by the FDA in 1995 for patients with PPH. The drug is extremely effective in opening up the lung arteries and takes immediate effect, unfortunately its effect only lasts about 5 minutes. The treatment is given intravenously through an indwelling catheter connected to a pump that continuously infuses the lungs with the substance. Patients who elect Flolan treatment must wear a battery operated backpack or bag that keeps the drug at a proper temperature. The treatment is very expensive, in the $150,000 per year range.

Treprostinil or Remodulin is still pending full FDA approval, but the drug is in the final clinical trials. The drug is very similar to Flolan but does not need to be stored at as low a temperature as does Flolan and it lasts much longer so that it does not need to be constantly infused and uses a smaller pump. In some patients Remodulin may be introduced under the skin in a process called subcutaneous infusion. Testing indicates Remodulin carries with it a lower risk of infections caused by the catheter.
Read more on Remodulin >>

Bosentan (Tracleer) This drug 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. This drug is called an "endothelin-A and endothelin-B receptor antagonist." In tests and clinical trials the drug proved its effectiveness by improving arterial capacity and reducing pressure in the blood vessels of the lungs.

Lung Transplants

If the PPH symptoms cannot be controlled using drug therapy, a patient may opt for a lung transplant. This operation is very risky, but can extend a patients life for an average of five to seven years. There are three types of transplants:

1) Heart and Lung
2) Single Lung
3) Double lung

A Heart and Lung Transplant will replace all the damaged organs, but is generally disfavored because the heart's right ventricle can recover if the pulmonary resistance is lessened with a new set of lungs.

A single lung may be an option, for older patients who do no qualify for a double lung transplant. Additionally the surgery is easier and many patients tolerate the procedure better. On the down side there may be a mismatch with the blood all flowing in the new lung, causing complications.

The most favored of these options is the double lung transplant. Doctors believe it avoids the complications of a single transplant, while offering more protection if something goes wrong. Unfortunately the surgery is more difficult to perform and the wait for two lungs is much longer.

If you or a loved one has been diagnosed with PPH and have used Fen Phen or related diet drugs, you may be entitled to substantial compensation to help pay for treatment.
Contact us to see if we can help you.


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