• Primary Pulmonary Hypertension
• Pulmonary Hypertension
• Pulmonary Arterial Hypertension
• PPH Symptoms
• PPH Diagnosis
• PPH Causes
• PPH Treatment
  • Ambrisentan
  • Tracleer
  • Flolan
  • Remodulin
  • Prostacyclin
  • Lung Transplants
  • Thelin
• Diet Drugs and PPH
• Legal Remedies for PPH

Pulmonary Hypertension

Pulmonary hypertension is a term used to describe a condition where the blood pressure in the pulmonary artery is higher than normal. There are two kinds of pulmonary hypertension: primary pulmonary hypertension and secondary pulmonary hypertension. In either case, the elevated blood pressure in the pulmonary artery (the large artery connecting the right heart ventricle and the lungs) causes the right ventricle to work harder than normal to pump blood into the lungs. Over time, this extra workload causes the heart muscles to become weakened and eventually fail. When the right ventricle can no longer pump blood into the lungs, the body cannot receive oxygen from the lungs and dies. A doctor should be consulted immediately after the onset of Primary Pulmonary Hypertension symptoms in order to allow for the greatest amount of treatment flexibility.

Hypertension

Hypertension is a medical term indicating that the blood pressure throughout the body is higher than normal. This elevated pressure is due to higher resistance to blood flow. Higher resistance can be caused by a number of factors, including constriction of blood vessels or excessive fluid in the blood. As blood vessels constrict, more force is required to pump the blood through the blood vessels and this increases the blood pressure. When more fluid is in the blood, again more force is needed to pump the blood through the body and thus blood pressure is higher. Pulmonary hypertension is a specialized type of hypertension because it does not occur throughout the body, but only in the vessels connecting the heart and lungs. The causes of pulmonary hypertension are related to changes only in these arteries.

Primary Pulmonary Hypertension

Primary pulmonary hypertension is the rarest type of pulmonary hypertension. Unlike other disorders, such as cerebral palsy, primary pulmonary hypertension can be inherited. In addition, it can occur for no apparent reason. Since the cause of primary pulmonary hypertension is sometimes unknown, it is also referred to medically as idiopathic pulmonary hypertension or unexplained pulmonary hypertension. Primary pulmonary hypertension causes have been shown to include use of the diet drugs Fen Phen, Redux (dexfenfluramine) and Pondimin (fenfluramine). The increased blood pressure in the pulmonary article can occur due to three types of changes:

• Muscles within the walls of the lung's arteries and vessels tighten up, narrowing the inside of the arteries.
• The walls of the pulmonary arteries thicken and scar tissue develops along the walls. As the walls thicken and scar, the arteries become narrower.
• Tiny blood clots form in the smaller arteries, causing blockages.

When there is less room for blood to flow through these narrowing arteries, blood pressure increases. Over time the arteries may also stiffen, increasing resistance. In some cases, the arteries become completely blocked. All of these changes lead to higher pressure in the pulmonary arteries.

Secondary Pulmonary Hypertension

Secondary Pulmonary Hypertension is much more common than primary pulmonary hypertension. It is caused or occurs due to other conditions, such as chronic heart disease, lung disease, blood diseases that cause clots in the lungs or diseases like scleroderma (autoimmune diseases that cause scar tissue or fibrosis in the skin or organs of the body). Essentially, any type of disease that blocks the passage of blood flow from the heart to the lungs can result in secondary pulmonary hypertension. When the cause of the blockage cannot be directly determined, then the disease is considered primary pulmonary hypertension.

Last Revision: June 9, 2009

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