[Primary pulmonary hypertension and its management]

Primary pulmonary hypertension (PPH) is a disease of un-known etiology that is characterized by an increase in pulmonary arterial pressure. Traditionally its treatment has been based on the administration of the vasodilator therapy with calcium channel blockers. At present there are some treatments which not only correct the vasoconstriction but also other pathogenic PPH associated effects, such a vascular remodelling and the thrombosis. These treatments include: a) analogs of prostacyclin with different administration routes; b) endothelin-receptor antagonists; and c) drugs that affect the metabolic pathway of nitric oxide. These pharmacological advances have reduced the number of pulmonary transplants in the patients with HPP, which is the last therapeutic alternative.