Primary Pulmonary Hypertension

Primary Pulmonary Hypertension: A Rare But Significant disease. It's a perplexing and relatively rare disease that can cause debilitating symptoms in its sufferers. Primary pulmonary hypertension is a disease characterized by narrowing of the blood vessels that carry blood to the lungs. As a result of the narrowing of these blood vessels, resistance to blood flow through these vessels occurs giving rise to a range of serious symptoms such as shortness of breath, dizziness, fainting, weakness, fatigue, ankle swelling, poor exercise tolerance, and chest pain. These symptoms may come on very slowly with the individual attributing the symptoms to "being old" or "out of shape". This may result in the diagnosis being delayed until the disease is in its more advanced stages. To add to the seriousness of the situation, this disease is considered incurable, although with early diagnosis and treatment, many patients can return to normal and productive lives.

What are the causes of primary pulmonary hypertension? Unfortunately, we still don't have clear cut answers to this vexing question.

There's some thought that individuals with primary pulmonary hypertension have unusually sensitive blood vessels making them more susceptible to factors that increase blood vessel resistance. This is supported by the fact that persons who suffer from Raynaud's Syndrome, a disease characterized by spasms of the peripheral blood vessels, have a higher incidence of primary pulmonary hypertension.

Another potential factor related to the cause of primary pulmonary hypertension, is the use of a particular prescription appetite suppressant combination known as Phen- Fen. When these drugs were prescribed for weight loss in the 1990's, it was noted that persons using these drugs had a higher incidence of primary pulmonary hypertension, resulting in this medication combination being removed from the market. How did Phen- Fen increase the risk of primary pulmonary hypertension? No one knows for sure, but it seemed the combination somehow promoted constriction of the critical blood vessels of the lungs.

Of interest in the cause of primary pulmonary hypertension is relatively recent research that shows a particular mutation in a gene called BMPR2 may result in symptoms of primary pulmonary hypertension. This doesn't account for all cases of primary pulmonary hypertension since most cases are not found to be associated with gene mutations. In addition to the small number of cases attributed to gene mutations, the cause of primary pulmonary hypertension has been associated with a variety of other disease states including HIV infection and liver disease. There is also an association between the incidence of primary pulmonary hypertension and cocaine use.

Although much progress has been made, it's essential that continuing research be performed in order to determine the exact cause of pulmonary hypertension in hopes of finding new and better treatments for this serious disease.