Ipertensione arteriosa polmonare: Una patologia da (ri)scoprire

Pulmonary arterial hypertension (PAH) is a rare disease, characterized by an increase of pulmonary arterial resistances that lead to right heart failure. We recognize a sporadic form (idiophatic or primary pulmonary hypertension IPAH), a familial form (FPAH, related to mutation in BMPR2, a gene that codifies for receptor type 2 of TGFb), and also, forms related to collagen-vascular diseases, congenital heart disease, portal hypertension, human immunodeficiency virus (HIV) infection, ingestion of drugs or dietary products. The incidence of idiophatic PAH, estimated in 2 cases per million per year, is probably underestimated because of aspecific symptoms. Transthoracic doppler echocardiography may suggest the presence of PAH but right heart catheterization is mandatory to confirm the diagnosis of PAH. Hemodynamic studies estimate the increase of mean pulmonary artery pressure (mPAP > 25 mmHg) with normal pulmonary capillary pressure (<12 mmHg) in the absence of left heart disease. A vasodilator study should be performed during right heart catheterization. Recent therapeutic progress (continuous venous infusion of prostacyclin, inhaled iloprost and endothelin receptor antagonists) have significantly improved the prognosis of this rare disease. Phosphodiesterase inhibitors of type 5 and new selective endothelin receptor antagonists are still in evaluation in clinical trial for this indication. Lung transplantation is indicated in patients that are refractory to medical treatments.