日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Pulmonary arterial hypertension (PAH) is a crucial determinant of morbidity and mortality in children and adults. It can present at any age from infancy to adulthood. The clinical features of pediatric PAH, however, differ from those of adults, and in children, there is a predominance of idiopathic PAH (IPAH) or that associated with congenital heart disease (CHD). Without appropriate treatment, the median survival rate after diagnosis of IPAH in children is considerably worse than that in adults. Additionally, though PAH related to CHD in most children may resolve after surgical correction, some children may develop an irreversible pulmonary vascular disease. Recent studies of pediatric PAH have highlighted the unique aspects of pathogenesis and challenging treatments in IPAH or PAH associated with CHD. Treatment with new selective pulmonary vasodilators offers hemodynamic and functional improvement in pediatric populations. Survival within the first 5 years following diagnosis has been reported to be between 62％ and 90％, and the survival rate has been similar between IPAH and PAH associated with CHD. However, there is currently limited data from randomized controlled trials in children with PAH for evaluating the safety and efficacy of vasodilator therapies, which are approved for adult patients. Therefore, further studies are required for development of specific strategies for treating children with PAH. This review provides a brief overview of recent information regarding current approaches to PAH in children.