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Pediatric pulmonary vascular disease contributes to morbidities and death in diverse clinical settings, ranging from idiopathic or heritable forms of pediatric arterial hypertension, congenital heart disease, developmental lung disorders, chronic lung disease, left heart disease, sickle cell disease, oncologic disease, and systemic disorders. Despite its impact on the clinical courses in so many diseases, information is limited on how to best approach the diagnosis and evaluation of pediatric pulmonary hypertension.To address this issue, a group of clinical experts from several subspecialties, including pulmonology, cardiology, neonatology, and others, were selected to form a task force to tackle this topic with support from the American Heart Association and American Thoracic Society. A joint guidelines study presenting their findings was recently published.This review highlights a few key topics underlying guidelines for the care of children with pulmonary hypertension that are especially important for the practicing pediatrician and others, and presents some of the major recommendations from the published guidelines report. Overall, the author emphasizes that these guidelines are based on the best current evidence and clinical experience of experts in the field, yet much more clinical research is needed to improve long-term outcomes in pediatric pulmonary hypertension.