Key Points

• Pulmonary arterial hypertension (PAH) is high blood pressure in the pulmonary arteries in the lungs.
• In PAH, the small arteries in the lungs narrow and may eventually become blocked. As a result, the heart must work harder to pump the blood through them. Over time, the overworked heart muscle becomes weak and loses its ability to pump enough blood to the lungs.
• PAH is called primary pulmonary arterial hypertension (PPAH) when no cause can be found for it. Secondary pulmonary arterial hypertension (SPAH) refers to PAH that is caused by or occurs with another condition—usually heart or lung disease.
• Certain factors appear to increase your chances of developing PAH, including use of appetite suppressants or cocaine and the presence of other diseases like HIV infection, scleroderma, and liver disease.
• PPAH may be caused by an abnormality in a gene.
• Common symptoms of PAH include difficulty breathing, fatigue, dizziness, fainting spells, swelling in the ankles or legs, bluish lips and skin, chest pain, and palpitations.
• PAH often is not diagnosed right away because its symptoms are similar to those of other conditions affecting the heart and lungs, and those conditions must be ruled out. Tests that can help confirm a PAH diagnosis include electrocardiogram, echocardiogram, pulmonary function tests, and cardiac catheterization.
• Right heart catheterization is the only way to obtain a precise measure of the blood pressure in the right side of the heart and the pulmonary artery. It is necessary to confirm suspected PPAH.
• Treatments for PAH include anticoagulants, calcium channel blockers, continuous intravenous epoprostenol (prostacyclin and other forms of prostacyclin), new drugs (Bosentan), and sildenafil.
• Surgery to replace one or both diseased lungs with healthy lungs from a human donor can help some people with PAH.
• Relaxation exercises, stress reduction activities, and adoption of a positive attitude can help PAH patients keep up with their normal activities. Eating a healthy diet, getting plenty of rest, and not smoking are also beneficial.
• Ongoing medical care, usually by a cardiologist or pulmonologist who specializes in pulmonary vascular medicine, is important.