Primary Pulmonary Hypertension Treatment, PPH Treatment

Primary Pulmonary Hypertension has no treatment that can stop the progression of the disease; however, the treatments can aid in slowing disease progression and improving patients' quality of life. Treatment is critical because without it, patients typically live less then five years.

Types of Treatment for PPH

The treatments for primary pulmonary hypertension are similar to those for systemic hypertension. Systemic hypertension is the elevation of blood pressure throughout the body, whereas for patients with PPH, the blood pressure is elevated specifically in the pulmonary artery. For systemic hypertension, a number of different drugs can be used, the most common of which are vasodilators that widen the blood vessels (vasodilate) and help to increase blood flow. Diuretic drugs can also reduce blood pressure by lowering the amount of fluid in the blood and thus requiring less force by the heart to pump the blood. Anticoagulants are common blood pressure lowering drugs. These drugs function by reducing the tendency of the blood to clot, thus allowing the blood to flow more freely.

Individual reactions to drugs vary dramatically for PPH patients. As such, different drugs have to be tried before long term treatment begins with a specific drug. Further, during the course of the disease, the drug treatment regimen may have to be changed. In some cases, to find out which drugs work best for each patient, physicians treat patients with different drugs during cardiac catheterization.

Vasodilators for PPH

Calcium channel blocking drugs are commonly used to treat systemic hypertension. These drugs dilate blood vessels throughout the body and reduce blood pressure. These drugs are taken orally in a wide range of dosages. At present, about 25% of PPH patients respond favorably to calcium channel blocking agents. It is best, if possible, for the patient to be treated with a standard oral vasodilator because these drugs are easier for the patients to maintain the treatment regimen and have less potential side effects. Other vasodilator drugs include anti angiotensin, endothelin receptor antagonist, Prostacyclin, and sildenafil (a form of Viagra).

Prostacyclin is a highly potent naturally occurring vasodilator. It is used for patients who do not respond to the oral calcium channel blocking agents. This drug is provided intravenously and must be administered continuously. Prostacyclin is also known by its generic name, epoprostenol. Flolan and Remodulin are vasodilator drugs that require continuous intravenous infusion and both are a form of Prostacyclin. Tracleer is an oral drug that is a potent vasodilator and its mechanism of action is as an endothelin receptor antagonist. Endothelin is a naturally occurring vasoconstrictor. By blocking the vasoconstricting action of endothelin, Tracleer dilates blood vessels.

Although these drugs cannot stop the progression of PPH, they can greatly enhance the quality of life by allowing the patients to indulge in less strenuous everyday activities that they could not perform otherwise, such as walking up stairs. Additionally, these drugs slow the progression of disease and are known to extend the lives of PPH patients.

Oxygen Treatment

In some cases, PPH patients are provided with oxygen treatment. This helps to increase the oxygen in the blood and alleviate the PPH symptoms.

The exact treatment of PPH depends on the cause of the disease. For example, if the cause of PPH is a hormonal imbalance, a doctor might prescribe certain medication designed to improve this condition. However, if a patient's PPH has been linked to the use to certain diet drugs, such as Fen Phen, a different type of treatment might be needed. In the latter case, doctors also typically recommend that patients consult skilled personal injury attorneys as they might help obtain rightful compensation from the manufacturers of Fen Phen. In addition to contacting a personal injury lawyer, many PPH patients also require the help of a family lawyer and/or a divorce attorney as this medical condition often wreaks havoc in their family lives.

Last Revision: September 9, 2013