Ivy DDPediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado. Electronic address: dunbar.ivy@childrenscolorado.org.
Abman SHPediatric Pulmonary Medicine, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado.
Barst RJColumbia University, College of Physicians and Surgeons, New York, New York.
Berger RMCentre for Congenital Heart Diseases, Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Bonnet DCentre de Référence Malformations Cardiaques Congénitales Complexes, Necker Hospital for Sick Children, Assistance Publique des Hôpitaux de Paris, Pediatric Cardiology, University Paris Descartes, Paris, France.
Fleming TRDepartment of Biostatistics, University of Washington, Seattle, Washington.
Haworth SGGreat Ormond Street Hospital, London, United Kingdom.
Raj JUDepartment of Pediatrics, University of Illinois at Chicago, Chicago, Illinois.
Rosenzweig EBColumbia University, College of Physicians and Surgeons, New York, New York.
Schulze Neick IGreat Ormond Street Hospital, London, United Kingdom.
Steinhorn RHDepartment of Pediatrics, University of California Davis Children's Hospital, Davis, California.
Beghetti MPediatric Cardiology Unit, University Hospital, Geneva, Switzerland.
English
Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.