Ivy DD Abman SH Barst RJ Berger RM Bonnet D Fleming TR Haworth SG Raj JU Rosenzweig EB Schulze Neick I Steinhorn RH Beghetti M 2013 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. https://sonar.ch/global/documents/118219 eng info:eu-repo/semantics/altIdentifier/doi/10.1016/j.jacc.2013.10.028 info:eu-repo/semantics/altIdentifier/pmid/24355636 Journal of the American College of Cardiology. - 2013 APAH-CHD AVT CHD HPAH IPAH PAPm PH PHVD PPHN PVR SVR acute vasodilator testing congenital heart disease hereditary pulmonary arterial hypertension idiopathic pulmonary arterial hypertension mean pulmonary artery pressure pediatrics persistent pulmonary hypertension of the newborn pulmonary arterial hypertension associated with congenital heart disease pulmonary hypertension pulmonary hypertensive vascular disease pulmonary vascular resistance systemic vascular resistance Age Factors Animals Child Humans Hypertension, Pulmonary Incidence Infant Infant, Newborn Netherlands Pediatric pulmonary hypertension. http://purl.org/coar/resource_type/c_6501