Treatment of pulmonary hypertension in infants, children and young adults. 2026 consensus statement from the European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AHA, AEPC, EACTS, ESPR, PASCAR, PCS, and PICS

The European Pediatric Pulmonary Vascular Disease Network (EPPVDN) is a registered, non-profit organization dedicated to advancing innovative diagnostic methods and treatment options for all forms of pediatric pulmonary vascular disease, including pulmonary hypertension (PH) related to congenital heart disease (CHD), PH associated with developmental lung disease (DEVLD-PH), persistent PH of the newborn (PPHN), postcapillary PH in left heart disease (PH-LHD), and associated cardiac dysfunction/heart failure. In the past, the EPPVDN has published consensus statements on the diagnosis and treatment of pediatric PH (2016, 2019), which have been endorsed by multiple societies. The executive writing group conducted comprehensive searches of the PubMed/MEDLINE bibliographic database (2000 to 2025) and engaged in multiple web-based and in-person meetings. Thirteen section task forces discussed and created consented recommendations. Prospective clinical studies, clinical trials, meta-analyses, expert consensus guidelines, and other articles with pediatric data were included in the search, using the terms 'pulmonary hypertension' and related keywords. Classes of recommendation (COR) and levels of evidence (LOE) were assigned based on AHA/ESC definitions, pediatric PH studies, adult studies including a minimum N number of 15 children, or studies involving adults with CHD. Classifications and definitions from the 7th World Symposium on Pulmonary Hypertension (WSPH) in 2024 were incorporated and discussed. We produced 6 graded recommendation tables (COR, LOE) and 3 algorithms for the treatment of pulmonary hypertension in infants, children, and young adults. A total of 16 main and 17 supplemental display items cover a range of topics, including risk assessment, pharmacological therapy, transcatheter interventions and surgery, recommendations specific to PH subgroups/special populations and clinical settings. Adaptation of management recommendations for middle-to-low-income regions is provided. Together, these up-to-date guidelines provide a comprehensive, detailed, and practical framework for the treatment of pulmonary hypertension in infants, children and young adults, aiming to improve outcomes and quality of life for affected patients.