2019 Updated Consensus Statement on the Diagnosis and Treatment of Pediatric Pulmonary Hypertension. The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT

ABSTRACT The European Pediatric PVD Network (EPPVDN) is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia (BPD), PH associated with congenital heart disease (CHD), persistent PH of the newborn (PPHN), and related cardiac dysfunction. The executive writing group (EWG) members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2018), held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles which include pediatric data were searched using the terms ´pulmonary hypertension´ and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on ESC/AHA definitions and on pediatric data only, or on adult studies that included > 10% children, or on studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated ten tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, CMR/chest CT, associated forms of PH, ICU/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle and low income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.