Pulmonary hypertension is high blood pressure in the lungs. Patients have pulmonary artery pressures greater than 25 mmHg at rest or greater than 30 mmHg with exercise. (Normal pulmonary artery pressures are between 10-15 mmHg.)
Pulmonary hypertension is rare and its cause is unknown. When accompanied by another disease, it is referred to as secondary pulmonary hypertension. These diseases commonly include thromboembolic disease, liver disease, autoimmune disease, cardiac disease and HIV infection. Connective tissue diseases such as scleroderma, rheumatoid arthritis and lupus also may be associated with pulmonary hypertension.
People with pulmonary hypertension slowly develop shortness of breath with exertion that progresses to distressed breathing at rest.
Currently, there is no cure for pulmonary hypertension. Treatment is to control symptoms. Some people respond to treatment with oral medications, such as calcium channel blockers and diuretics. Others may need drugs like intravenous epoprostenol. As the disease progresses, oxygen therapy may be necessary. If all else fails, suitable candidates may be helped by lung or heart-lung transplantation .
