Persistent pulmonary hypertension, or PPH, is an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary arteries. Dr. Ernst von Romberg first identified PPH in 1891.
PPH is generally diagnosed by physical examination. Typical signs of persistent pulmonary hypertension include altered heart sounds, parasternal heave, pulmonary regurgitation, elevated jugular venous pressure, peripheral edema (swelling of the feet/ankles), ascites (abdominal swelling due to fluid), hepatojugular reflux, and clubbing.
Further testing can include pulmonary function tests, electrocardiograph (ECG), arterial blood gas measurements, chest x-rays, CT scans, and ventilation or perfusion. Normal pulmonary pressure is 12-16 mm Hg — pulmonary hypertension is present when the pressure exceeds 25 mm Hg.
There are five different types of PPH:
- Arterial PPH;
- Venous PPH;
- Hypoxic PPH;
- Thromboembolic PPH; and
- Miscellaneous PPH.
PPH is often difficult to diagnose because the symptoms begin very gradually. Symptoms of persistent pulmonary hypertension include shortness of breath, dizziness, fainting — all of which are exacerbated by physical exertion. Other symptoms include non-productive cough, angina pectoris, fainting or syncope, lips and skin that turn blue, peripheral edema (swelling around the feet and ankles), and hemotysis (coughing up blood). Finally, PPH can result in a significant decrease in exercise tolerance, and even lead to heart failure.
Treatment is determined based on the typed of PPH. Arterial PPH can be treated with lifestyle changes, oxygen therapy, oral anti-coagulants, diuretics, and digoxin.
Pulmonary venous hypertension is synonymous with congestive heart failure, meaning treatment options center on optimizing left ventricular function. This can be achieved though the use of diuretics, ACE inhibitors, beta-blockers, or to simply repair/replace the valve.
Left untreated, PPH patients have a survival rate of 2-3 years. However, with proper treatment, expected survival rates can exceed 10 years.
Fen-Phen and PPH
The drug combination fenfluramine/phentermine, usually called Fen-Phen, is an anti-obesity treatment that utilizes two anorectics. Fen-Phen was sold under two trade names: fenfluramine (Pondimin) and dexfenfluramine (Redux).
A three-year study, published in 1992 showed that Fen-Phen was extremely effective in maintaining a significant weight loss. However, when the patients stopped taking Fen-Phen, most regained their weight.
In 1996, an article in the New England Journal of Medicine showed a statistically significant rise in persistent pulmonary hypertension in patients taking Fen-Phen. Due to these and other side effects, in late 1997, the FDA recommended that Fen-Phen no longer be prescribed.
The FDA ordered fenfluramine and dexfenfluramine off the market in September 1997 after those drugs were linked to heart valve problems. Fenfluramine is one of the ingredients in Fen-Phen, and dexfenfluramine is closely related to fenfluramine. These drugs are manufactured by Wyeth Pharmaceuticals and American Home Products.
Clearly, this drug combination used for rapid weight loss was extremely unsafe. The manufacturers of Fen-Phen promoted this drug as safe and effective. As shown in the study referenced above, the drug is not safe — thousands of patients taking Fen-Phen now have various cardiovascular and lung problems, such as persistent pulmonary hypertension. In addition, the drug is not effective — studies show that patients who stop taking Fen-Phen almost always regain the weight they originally lost.
Upon the release of the information regarding the pulmonary and cardiac risks of Fen-Phen, the Association of Trial Lawyers of America formed a group to seek damages from American Home Products, the distributor of fenfluramine (Pondimin) and dexfenfluramine (Redux).
Estimates of total liability ran as high as $14 billion. As of February 2005, Wyeth was still in negotiations with injured parties, offering settlements of $5,000 to $200,000 to some of those who had sued, and stating they might offer more to those who were most seriously injured.
According to a 1999 article in The New York Times, a Texas woman was awarded $23.3 million for heart damage she suffered after taking the diet drug combination known as Fen-Phen. The final settlement amount is undisclosed.
If you or someone you know has primary pulmonary hypertension, you should contact a Fen-Phen lawyer today to discuss your case. If you have been diagnosed with primary pulmonary hypertension (PPH), please contact a PPH lawyer today for a free consultation.
If you are suffering from dizziness, chest pains, skin turning blue, or difficulty breathing, you may have persistent pulmonary hypertension — a difficult disease to diagnose. The best step you can take is to contact your medical provider for further testing. If you believe you have PPH, you should contact an experienced PPH lawyer today to assist you with your Fen-Phen claim.