People at risk are women between 20 and 40 years old, although the disease can affect any sex and age. The incidence is 8 out of 100,000 people. It is rare in children. More common is pulmonary hypertension in infants with persistent fetal circulation. The goal of treatment is control of the symptoms.
Some people respond to treatment with vasodilators.
Other medications used to relieve symptoms include diuretics and calcium channel blockers. As the disease progresses, oxygen may be needed for shortness of breath . Heart-lung transplantation for suitable candidates with available donors has been successful. The probable outcome is poor, most people develop progressive heart failure leading to death in 2 to 8 years. Primary pulmonary hypertension is a progressive disease, leading to congestive heart failure and respiratory failure.