Pulmonary arterial hypertension (PAH) is a rare progressive disorder characterized by high blood pressure (hypertension) in the pulmonary arteries.

Hypertension may be caused through narrowing of the blood vessels that carry blood from the right side of the heart through the lungs.

Symptoms include shortness of breath (dyspnea) specially while exercising, chest pain dizziness and fainting episodes. Some patients may experience peripheral swelling (edema) of ankles and legs, or also cough with blood. This condition usually affects women between 30-60 years. PAH may stay undiagnosed for years due to mild or non-specific symptoms. It is important to treat PAH since arterial hypertension causes the right heart to work much harder to pump blood through the lungs, eventually causing the heart muscle to weaken or fail over time. Hence, initially undiagnosed, it may progressively worsen and in advanced stages might become fatal. About 15-20% of the patients with PAH have heritable PAH caused by genetic mutations. The exact cause of PAH is unknown, and although there is currently no cure for PAH, several therapies help to manage the symptoms and slow down the disease progression.

Source: mayoclinic.org; rarediseaes.org; Flavell (2018), Pulmonaryhypertensionnews