Currently, treatment options are unable to cure PPH. However, certain treatments can relieve some of its symptoms. Certain drugs, known as vasodilators, dilate the blood vessels in the lungs which reduces blood pressure helping to relieve strain on the heart. These drugs must be evaluated with heart tests, as they can cause serious side effects in some patients. Another issue with vasodilators is that they rarely normalize the elevated blood pressure and must be consumed in high doses to achieve the desired effect.

Another option is a blood thinning drug. Anticoagulants or drugs that discourage blood clotting, especially in the lungs, may be beneficial. Supplemental oxygen is also often recommended by doctors as low blood oxygen levels is a side effect of PPH.

Lung transplants, although sometimes rare, are also a viable option for patients with severe PPH. In some cases, if the heart is also diseased, then a heart-lung transplant may also be performed. Lung transplants are an extreme measure and in order to qualify for a transplant, patients aged twelve and older are evaluated according to the Lung Allocation Scoring system. This system evaluates their health and need for the transplant. Lungs are then awarded based on patient need, rather than how long they have been on a waiting list. Survival rates are, “generally very high at the one year mark but lower at the five and ten year follow-ups.”

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The diet drug Fen-phen was once viewed as a miracle cure for obesity, and a wonder drug for weight loss. However, these thoughts were quickly retracted after numerous reports came in of patients suffering from heart attacks and strokes after taking the drug. In fact, around thirty percent of patients using Fen-phen experienced problems in their heart valve function, or ultimately primary pulmonary hypertension. This caused Fen-phen to be pulled from the market in 1997.

The connection between Fen-phen and primary pulmonary hypertension is done through a series of invasive tests, as well as an electrocardiogram of the heart to determine if the hearts valves are functioning properly.

To date, former Fen-phen users have filed approximately 175,000 claims against the company. American Home Product’s, the company responsible for the drug, knew about a possible link between Fen-phen use and PPH yet they didn’t speak up. Even on the product’s adverse effects label there was no warning. The FDA believed that American Home Products knew there was a possible problem with Fen-phen and covered it up.

This debilitating heart condition can take months to years after use of the drug to develop. Pay attention to any warning signs and get the necessary help as quickly as possible.

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Back in June, we wrote about a new drug called Tracleer (bosentan) that was being tested in clinical trials and was showing improvements in the quality of life for patients suffering from PAH. Moreover, it was recently announced that Tracleer has been approved in the European Union for the treatment of patients with mildly symptomatic pulmonary arterial hypertension (PAH WHO FC ‘Functional Class’ II). Since 2002, Tracleer had been available in the European Union for PAH patients with WHO FC III, and now it is approved for WHO FC II, as well.

Tracleer is the first PAH treatment to be investigated in a clinical study that exclusively enrolled patients with mildly symptomatic WHO FC II. The controlled study of 185 patients provided the basis for this EU approval. Tracleer has proved to delay the progression of the disease in these patients. In fact, throughout the three placebo-controlled clinical studies, Tracleer “is the only PAH medicine to have demonstrated delay in disease progression.” These clinical findings will now work toward early diagnosis and intervention of the disease.

Tracleer in an oral drug that in licensed for treatment of PAH in the U.C. for PAH Functional Class III and IV to improve exercise capacity and decrease the rate of clinical worsening. In Europe it is for PAH Functional Class III to improve exercise capacity and symptoms; and is now used for Functional Class II as well which has also shown improvements. Tracleer has been made commercially available by Actelion subsidiaries in the U.S., the European Union, Japan, Australia, Canada, Switzerland and other markets worldwide since 2001. In these seven years of clinical experience, “more than 50,000 patients have been treated with Tracleer.”

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