Pulmonary arterial hypertension in the USA: an epidemiological study in a large insured pediatric population

Pulmonary arterial hypertension (PAH) is rare in children and few data are available in a pediatric general population. This study aims to calculate the annual incidence and prevalence of PAH and to describe these children in a large US population of patients aged under 18 years. Using the US MarketScan claims database we identified 695 children with PAH in 2010–2013. We calculated annual incidence rates and prevalence overall, by age and PAH type (idiopathic and non-idiopathic) using Byar’s method. We also described characteristics, co-morbidities, treatment patterns, and diagnostic procedures for these children. In 2010–2013, the annual incidence rates of PAH per 1,000,000 children-years was in the range of 4.8–8.1; 0.5–0.9 for idiopathic PAH and 4.3–7.3 for non-idiopathic PAH. The annual prevalence of PAH was in the range of 25.7–32.6 per 1,000,000 children; 4.4–6.0 for idiopathic PAH and 21.3–27.0 for non-idiopathic PAH. Incidence rates and prevalence were highest in children under age 2 years. Around...