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What is primary pulmonary hypertension?

Primary pulmonary hypertension is a rare, progressive disorder characterized by high blood pressure (hypertension) of the main artery of the lungs (pulmonary artery). The pulmonary artery is the blood vessel that carries blood from the heart through the lungs. Symptoms of primary pulmonary hypertension include shortness of breath (dyspnea) especially during

exercise, chest pain, and fainting episodes. The exact cause of primary pulmonary hypertension is unknown.

Primary pulmonary hypertension (PPH) is considered a genetic disorder. It has been linked to mutations in the BMPR2 gene, which encodes a receptor for bone morphogenic proteins (Deng et al, 2000), as well as the 5-HT(2B) gene, which codes for a serotonin receptor (Blanpain et al, 2003). Recently, characteristic proteins of human herpesvirus 8 (also known for causing Kaposi sarcoma) were identified in vascular lesions of PPH patients (Cool et al, 2003). However, it is not understood what roles these genes and viral particles play in PPH. PPH has also been associated to the use of appetite suppressants (e.g. Fen-phen, see Abenhaim et al, 1996). While genetic susceptibility to adverse drug reactions is suspected, the cause of the disease is still largely unknown.

PPH is very rare but often fatal. Patients usually have no symptoms until they reach their late twenties or early thirties. It is characterized by elevated pulmonary vascular resistance attributable to the abnormal thickening of the vessel wall and narrowing of the lumen of arterioles in the lungs. Primary pulmonary hypertension is an unusually nasty and often fatal form of pulmonary hypertension that commonly affects young people. Whereas it is known that the arterial obstruction is caused by a building up of the smooth muscle cells that line the arteries, the underlying cause of the disease has long been a mystery.

More information on pulmonary hypertension

What is pulmonary hypertension? - Pulmonary hypertension (PH) is a rare lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels.
What is primary pulmonary hypertension? - Primary pulmonary hypertension is a rare, progressive disorder characterized by high blood pressure of the main artery of the lungs.
What is secondary pulmonary hypertension? - Secondary pulmonary hypertension is a disorder of the blood vessels in the lungs. It is the result of other lung diseases.
What causes pulmonary hypertension? - Pulmonary hypertension is the result of greater resistance to blood flow. Secondary pulmonary hypertension can be associated with breathing disorders.
What're the symptoms of pulmonary hypertension? - Symptoms of primary pulmonary hypertension include shortness of breath especially during exercise, chest pain, and fainting episodes.
How is pulmonary hypertension diagnosed? - Diagnostic tests for pulmonary hypertension involve blood tests, electrocardiography, arterial blood gas measurements, X-rays.
What's the treatment for pulmonary hypertension? - Treatment of pulmonary hypertension involves treating the underlying causes, using supplemental oxygen to increase blood oxygen levels, diuretics.
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