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Pulmonary Arterial Hypertension (PAH)

Pulmonary arterial hypertension (PAH) is a rare and deadly lung disease that is characterized by a higher-than-normal blood pressure in the pulmonary artery. PAH is also known as primary pulmonary hypertension. Instances of pulmonary arterial hypertension develop subtly with a gradual increase in pulmonary arterial blood flow. However, as the disease continues to develop it fosters a damaging arterial pressure that eventually escalates to the point where it is capable of destroying the heart and lungs.

Pulmonary arterial hypertension is considered to be present when "at rest" arterial blood pressure exceeds 25 mmHg.

Pulmonary Arterial Hypertension - The Causes

Pulmonary arterial hypertension has been linked with a variety of autoimmune disorders like HIV and scleroderma (disease of the skin and connective tissue). Some of the other diseases known to cause PAH include cirrhosis, portal hypertension, sickle cell disease, congenital heart disease, and various types of thyroid disease.

It is now known that that pulmonary arterial hypertension can also be caused by the use of certain diet drugs. Fen Phen, Pondimin (fenfluramine) and Aminorex, are three such diet drugs that have been linked with PAH.

Fen Phen - A type of anti-obesity medication (anorectic), Fen Phen is no longer widely available because of its link to valvular heart disease and pulmonary hypertension. Fen Phen consisted of two drugs, fenfluramine and phentermine.

Pondimin - Pondimin is the brand name for fenfluramine, an anti-obesity drug that is part of the Fen Phen family. Pondimin manipulates the central nervous system by increasing the levels of serotonin, producing a sensation of being full. An appetite suppressant, Pondimin was withdrawn from the US market in 1997 after links were drawn between the drug and various heart conditions (such as PAH).

Aminorex - A type of anorectic stimulant drug (appetite suppressant) that is somewhat related to 4-methyl-aminorex (banned stimulant).

The manufacturers of the aforementioned diet drugs have been faced with personal injury litigation aimed at compensating the victims of diet drug side effects like PAH.

Pulmonary Arterial Hypertension - The Diagnosis

Pulmonary arterial hypertension affects an approximate 100,000 people in the United States, only 15,000 to 20,000 of which have been diagnosed. Because PAH is such a rarity, it is often overlooked by some doctors and is therefore misdiagnosed as a variety of other diseases, some of which include asthma, chronic obstructive pulmonary disease (COPD) or congestive heart failure (CHF).

A proper diagnosis of pulmonary arterial hypertension requires the use of cardiac catheterization in the form of a Swan-Ganz catheter. The catheter is inserted into the pulmonary artery, allowing for a pressure sampling to be taken and measured against the 25 mmHg arterial blood pressure limit.

Additional tools used to diagnose pulmonary arterial hypertension include:

Pulmonary Arterial Hypertension - The Treatments

All cases of pulmonary arterial hypertension are, to an extent, treatable. In recent years, great strides forward have been made in the development of new and experimental drugs designed to combat pulmonary arterial hypertension. Some of these breakthrough drugs include:

In addition to drug treatments, there are other modalities to be considered, such as lung transplantation. Up until a decade ago, the only method through which to treat PAH was via heart or lung transplantation. Although lung transplantation is considered to be a curative modality, it produces unavoidable complications associated with such an invasive surgery. As such, the mean survival time for PAH patients who undergo lung transplantation is approximately five years.

Pulmonary Arterial Hypertension - The WHO Class System

Pulmonary arterial hypertension is one of five classes of pulmonary hypertension. In 2003, the World Symposium on Pulmonary Hypertension decided to modify the classification system so as to incorporate five groups:

Last Revision: December 18, 2007

MediaFact is a major online resource about drug side effects connected with Stevens Johnson Syndrome and Primary Pulmonary Hypertension.

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