Pulmonary arterial hypertension is a recently classified as primary and secondary entity according to its etiology. It is characterized by increased pulmonary artery pressure that results in progressive dilation, right ventricular failure and premature death. The incidence of this disease is low, but has severe physiopathologic implications. Many endogenous and exogenous causes that contribute to the development of the disease have been determined. Treatment is focused on improving ventricular function for which purpose vasodilators and new drugs directed to induce vascular smooth muscle relaxation are used. Besides, oxygen is used when it improves patients condition, as well as oral anticoagulation.