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Is Pulmonary Hypertension Always Fatal?
My Aunt died from pulmonary hypertension after a long series of complications from  using a combination of the weight loss drugs, Phen-Fen.  Did everyone who used Phen-Fen going to ultimately die from Pulmonary Hypertension, or were there other problems in those who did?  If so, was there a genetic factor that perhaps caused some to die and others not to die?

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1884349 tn?1353818598
Dear Sandy,

I am terribly sorry to hear about your aunt.

Weight loss drugs, including phen-fen, have clearly been shown to increase the risk of developing pulmonary hypertension. However, not everybody who took phen-fen developed the disease. As you suggested, this is probably because of underlying differences in genetic factors from person to person.  

In fact, we are beginning to think of pulmonary hypertension much in the same way we think about cancer.  Specifically, we believe that the “two-hit hypothesis” in cancer might apply to pulmonary hypertension as well.  In the two-hit hypothesis, the patient has a genetic predisposition to developing a particular disease (i.e. the 1st hit) and then a second factor such as exposure to phen-fen (i.e. the 2nd hit) actually triggers the disease (this is what may have happened to your aunt).

As far as prognosis is concerned, pulmonary hypertension (from any cause including phen-fen) is quite complex and treatment approaches and the response to the treatments can differ depending on the severity of the disease.  Many patients with true pulmonary hypertension due to phen-fen will ultimately die from the disease.  Others may be more fortunate to not have as severe of a case and, with the right treatment, might live many years with the disease under good control (again, genetic factors likely influence this to some extent as well).

Once again, I am very sorry about your loss.

Dr. Rich

P.S. In case you are interested, this is the reference to the landmark study published in the New England Journal of Medicine that clearly linked phen-fen with pulmonary hypertension, entitled, “Appetite-Suppressant Drugs and the Risk of Primary Pulmonary Hypertension.”  http://www.nejm.org/doi/full/10.1056/NEJM199608293350901
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