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more_legal_areas PPHTwo out of every million people are affected by pulmonary hypertension, a rare progressive disorder that produces high blood pressure (hypertension) in the pulmonary artery, the artery that carries blood from the heart through the lungs. Pulmonary hypertension affects the blood vessels in the lungs with a pressure that may become life- threatening. When the disease exists without a known cause, it is referred to as primary or unexplained pulmonary hypertension; when a cause is known, the disorder is referred to as secondary pulmonary hypertension.
In the U.S., 500 to 1,000 new cases of pulmonary hypertension are diagnosed each year. The greatest number of cases is reported in women age 20 to 40, however men, women and children of all ages can be affected by pulmonary hypertension.
Pulmonary hypertension does not discriminate. It affects people of all ethnicities and may have many unknown causes. Pulmonary hypertension begins to develop when the small blood vessels that carry blood to the lungs begin to constrict, making it difficult for blood to pass through. This forces the heart to pump harder to get blood to the lungs, causing enormous stress, enlargement and loss of flexibility in the heart. Eventually, some blood vessels will become scarred and completely blocked, causing less blood to flow out of the heart and into the body. This is when symptoms of pulmonary hypertension become more noticeable to sufferers.
Once symptoms are recognizable, pulmonary hypertension is often in its later stages. By then, prognosis is grim, with many patients experiencing a poor survival rate. New treatments have been developed, however, and survival rates can be lengthened if pulmonary hypertension is diagnosed in time. New studies have found that some people have been able to manage the disorder for 15 to 20 years after diagnosis.
In 1998, the World Health Organization and pulmonary hypertension experts determined five different categories of pulmonary hypertension. The first category, pulmonary arterial hypertension, consists of primary pulmonary hypertension and secondary pulmonary hypertension. The second category, pulmonary hypertension associated with disorders of the respiratory system, refers to the disorder occurring in conjunction with emphysema, sleep apnea, chronic exposure to high altitude and interstitial lung disease. The third category refers to the disease as it relates to chronic thrombotic or embolic diseases, or diseases of the blood. Examples include blood clots, embolisms in the lungs or sickle cell disease. Category four is due to disorders that directly affect the pulmonary blood vessels, such as inflammatory diseases and capillary diseases. The final category, pulmonary venous hypertension, is caused by diseases to the left side of the heart, such as mitral valve disease or a poorly performing left ventricle.