ABSTRACT
Prior to the current treatment era, the prognosis of pulmonary arterial hypertension (PAH) was extremely poor with a high mortality rate (1,2) and less than one-year survival in untreated children (2-4); prognosis has improved markedly in recent years. This has coincided with improvements in diagnosis and general management, as well as the off-label application of adult PAH-specific therapies to children (5-8). The prognosis of PAH has changed dramatically with a 97% five-year survival in children with severe PAH responding to acute vasodilator testing treated with calcium channel blockade and a 92% five-year survival in nonresponders treated with intravenous (IV) prostacyclin (9,10). Nevertheless, PAH has no cure, and the aim of the treatment is to improve quality of life, symptoms, hemodynamics, and exercise capacity and to prolong survival. This chapter reviews the current understanding of pediatric pulmonary hypertension, classification, diagnostic evaluation, and available treatment with description of targeted pharmacological therapy and new treatments in children.
