Definition of Pulmonary hypertension

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Pulmonary hypertension: High blood pressure in the pulmonary artery that conveys blood from the right ventricle to the lungs. The pressure in the pulmonary artery is normally low compared to that in the aorta. Pulmonary hypertension can irrevocably damage the lungs and cause failure of the right ventricle.

Pulmonary hypertension is conventionally divided into primary and secondary types. Primary pulmonary hypertension is considered idiopathic (of unknown origin). It occurs sporadically with no family history of the disorder and in a familial form.

Secondary pulmonary hypertension may be due to congenital heart disease, pulmonary embolism, portal hypertension, collagen vascular disorders (such as lupus), sarcoidosis, and HIV infection.

The goals of treatment for pulmonary hypertension are to treat the underlying cause, to reduce symptoms and improve quality of life, to slow the growth of the smooth muscle cells and the development of blood clots; and to increase the supply of blood and oxygen to the heart, while reducing its workload. Treatments include medications, oxygen, and lung transplant. The main medications for pulmonary hypertension include:

• anticoagulants (to reduce the formation of blood clots);
• calcium channel blockers, which relax blood vessels and increase the supply of blood and oxygen to the heart, while reducing its workload;
• continuous intravenous epoprostenol (prostacyclin currently considered the most effective therapy) which widens the lung arteries and prevents blood clot formation;
• treprostinil, another prostacyclin, also relaxes blood vessels and increases the supply of blood to the lungs, reducing the workload of the heart;
• bosentan, a relatively new treatment that widens the lung arteries and reduces blood pressure;
• nitric oxide inhalation, which causes the pulmonary arteries to widen or open;
• Viagra (sildenafil) which causes the pulmonary arteries to open; and
• diuretics which may help ease symptoms and improve the heart's performance in some patients with pulmonary hypertension.

The term "pulmonary hypertension" is composed of "pulmonary" for lung, "hyper" for high, and "tension" for blood pressure = pulmonary high blood pressure, or pulmonary hypertension.