摘要：Pulmonary arterial hypertension (PAH) is considered a rare disease in the pediatric population, is idiopathic or associated with congenital heart disease, it is rarely associated with connective tissue disease, it represents an important cause of morbidity and mortality. According to data from the Netherlands, the incidence and prevalence is 0.7 and 4.4 for idiopathic PAH and 2.2 and 15.6 for PAH associated with congenital heart disease, cases per one million children. Selective vasodilator therapy has significantly improved the prognosis and life expectancy for affected children. This article addresses current knowledge about PAH in children, reviewing diagnostic criteria and therapeutic management.
关键词：Pulmonary Arterial Hypertension; Congenital Heart Disease; Vasodilators; Children
