What is PPH?

By Jane Mundy

Primary or unexplained pulmonary hypertension (PPH) is a rare and serious lung disorder in which the blood pressure in the heart's pulmonary artery rises dangerously above normal levels for no apparent reason. (The pulmonary artery is a blood vessel carrying oxygen-poor blood from the right ventricle of the heart to the lungs.) Once in the lungs, the blood picks up oxygen and flows to the heart's left side, where the left ventricle pumps it to the rest of the body through the aorta.

The American Heart Association reports that in the US alone, an estimated 500 to 1,000 new cases of PPH are diagnosed each year and the greatest number is reported in women between ages 20 and 40. But PPH can develop in anyone, from young children to older males.

The cause is not clear but one theory is that people who develop PPH may have blood vessels that are sensitive to certain factors that trigger this disease. People with Raynaud's syndrome have a higher incidence than others to develop PPH. As well, external factors believed to trigger the narrowing of the pulmonary artery are cocaine, HIV and appetite suppressants/diet drugs.

Diet Drugs - Fen Phen and Redux

In 1996, a study published in theNew England Journal of Medicine found that the use of diet drug Fen Phen (fenfluramine and phentermine) for three months or longer increased the risk of developing PPH by 23%. One year later, the FDA recalled Fen Phen along with other popular appetite-suppressants Redux (dexfenfluramine), Pondimin (fenfluramine).

By the time it was taken off the market, about six million people in the U.S. had used Fen Phen. The drug was never approved for combined use by the FDA nor was it tested for long-term safety. Studies have shown that first symptoms of the disease may not develop until up to ten or more years after taking these diet drugs.

Symptoms of Primary Pulmonary Hypertension

The most common early symptoms relate to breathing problems and include: Later symptoms can include coughing up blood, cyanosis (blue tinge to lips, hands and feet), ankle and lower leg swelling, increased chest pain, and sometimes, death.

A physical examination of patients with PPH may show: A diagnosis of PPH can be tricky and rarely found in a routine medical exam, partly due to the time lapse of PPH symptoms. As well, the symptoms can often be confused with other heart and lung conditions. One determining test is a cardiac catheterization along with an electrocardiogram, a chest X-ray and ultrasound.

Lawsuits against Diet Drugs

More than a decade has passed since Fen Phen was removed from the market yet lawsuits are growing in number, in part due to the time lapse of symptoms. Some people who took diet drugs in the early 1990s are just now developing signs of PPH and coming to realize the long-term serious side effects of these drugs.

In April 2004, a Beaumont, Texas jury reached a verdict against drugmaker Wyeth and awarded $1 billion to the family of plaintiff Cynthia Cappel-Coffey who took Fen Phen and died when she was 41 years old.

In December 2004, a $25 million settlement was granted court approval in the class action lawsuit filed on behalf of Canadians (excluding those residing in Quebec) who ingested the diet drugs Ponderal and Redux and suffered from Valvular Heart Disease ("VHD") or PPH.

In March 2005 two Utah women were awarded $5.5 million for damages connected to their use of Fen Phen; two months later, a Pennsylvania jury awarded two former users of Fen Phen $200 million in damages for heart problems caused by the drug.

In May of last year, Wyeth reached a settlement with 40,000 users of the Fen Phen diet combination. (Fen Phen was a cocktail of diet drugs: patients were given either Pondimin (fenfluramine) or Redux (dexfenfluramine) in combination with the generic phentermine in what became known as fen-phen.) The settlement allowed users least affected by the drugs to receive compensation from a $1.28 billion supplemental fund. Claimants had to pass a medical review in order to receive pro-rated shares of the supplemental fund. Those who did not pass the medical review received $2,000 each.

Get Legal Help

PPH is a serious medical condition and requires immediate treatment. Usually, a cardiologist or pulmonologist will diagnose PPH. It is advisable to find a doctor who specializes in PPH (Visit phassociation.org for a list of doctors who specifically treat PPH.) Your next step is to contact a lawyer -- once diagnosed, you need to take action.

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