Pediatric PH: New Challenges and Perspectives

This issue of Advances in Pulmonary Hypertension highlights the important changes in the recognition of the causes of PH in children and its treatment. PH in children differs from adults as children frequently have multifactorial disease, with a combination of a congenital heart defect, chronic lung disease, and an abnormality of lung development. The “fetal origin” of PH in children has been emphasized by Dr Steve Abman in his article on diagnosis and treatment of the child with broncho-pulmonary dysplasia. Currently there are no FDA-approved therapies for children with PH/PAH; however, therapy for these children has improved quality of life in many. As described in the article by Dr Delphine Yung, therapy for children is based on experience, whereas therapy for adults is based on controlled clinical trials. These advances have improved the quality of life for children with congenital heart disease and also those with idiopathic PAH and heritable PAH as described by Dr Mary Mullen. Dr Mullen's article also describes a pediatric-specific algorithm for the diagnosis of IPAH/HPAH in children. Finally, Aimee Doran, an experienced pediatric PH nurse practitioner, describes the unique challenges to family life when a child is diagnosed with PH. Our patient perspective this issue highlights that many children with PH are growing up to be energetic young adults with new challenges and perspectives. While dramatic improvements have been seen in the diagnosis and treatment of children with PH, many hurdles still remain.