Pulmonary Arterial Hypertension: A New Challenge

Pulmonary hypertension represents a new challenge for the current clinical practice, as recent advances in the field of diagnostic technologies led a to significant increase in the number of patients diagnosed with this disease and in the same time new therapeutic classes proved to have a significant role in improving survival of these patients.
The main types of pulmonary hypertension are the idiopathic form, called primary pulmonary arterial hypertension (PAH) and the secondary one, developed in the evolution of a cardiac disease, in many cases consequence of a congenital heart disease. Once occurred, PAH severely limits the life expectancy. Despite the recent introduction of many new therapeutic agents which are expected to have a revolutionary role in the improvement of life expectancy and quality of life of these patients, PAH continues to be associated with a mortality as high as 35% at 3 years after the diagnosis. The new therapies are mainly represented by vasodilators that target the nitric oxide, endothelin and prostacyclin pathways.
The diagnosis of PAH is usually established when a typical pattern of pre-capillary pulmonary hypertension is encountered in the hemodynamic profile, in the absence of any lung disease. [More]