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Rare diseases

Pulmonary arterial hypertension

Pulmonary arterial hypertension is a rare disease that affects about 15 in a million people1. It is caused by an abnormal blood pressure increase in the pulmonary arteries conveying blood from the heart to the lungs; this results in heart overload, which can even impair right heart function.

In some cases, pulmonary hypertension is primitive, i.e. there are no causes to explain the condition; in other cases it is associated with other disorders like connective tissue diseases (scleroderma, lupus), liver diseases (portal hypertension), viral infections (HIV), congenital heart diseases, and recurrent pulmonary thromboembolism. Patients affected by this disorder experience dyspnea; they may feel breathless, particularly on exertion, and therefore even minimal physical effort becomes impossible. The symptoms, which may appear on exertion or at rest, include: cough, sudden fainting spells and fluid retention, with swollen ankles or a sense of abdominal heaviness.

Treatment is based essentially on medications in three therapeutic classes: endothelin receptor antagonists, phosphodiesterase-5 antagonists, and prostanoids.

Besides early diagnosis, it is essential that patients follow a healthy lifestyle, paying attention to their diet, controlling weight and avoiding smoking and alcohol consumption.

Notes

1 Source:McLaughlin VV et al. Pulmonary arterial hypertension, Circulation 2006; 114:1417-1431