Pulmonary hypertension: updated guidelines on diagnosing, treating children

  • J Heart Lung Transplant

  • International Clinical Digest
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Takeaway

  • The European Pediatric Pulmonary Vascular Disease Network (EPPVDN) has published new clinical practice guidelines for diagnosis and treatment of pediatric pulmonary hypertension (PH).
  • Since the last publication in 2016, the updated 2019 guidelines contain new diagnostic criteria, prognostic risk factors, treatment algorithms, and graded recommendations.
  • The 2019 guidelines have been endorsed by the Association for European Pediatric and Congenital Cardiology (AEPC), European Society for Pediatric Research (ESPR), and International Society of Heart and Lung Transplantation (ISHLT).

Why this matters

  • Mortality from pediatric PH is declining, placing more emphasis on the management of a more chronic condition.
  • Most of the prior guidance has been extrapolated from adults; this version relies strongly on pediatric data.

Study design

  • Literature review and recommendations developed by a 31-member Executive Writing Group.
  • Each recommendation is assigned a color-coded standardized classification for the consensus behind the recommendation and a classification from A to C covering the level of evidence.
  • Funding: None disclosed.

Highlights

  • The new definition of PH, developed in 2018 by the World Symposium on PH, lowers the lower limit of mean pulmonary arterial pressure from >24 to >20 mm Hg.
  • New table of prognostic risk factors that can guide treatment decisions.
  • New diagnostic and treatment algorithms.
  • New recommendations for care in middle- or low-income regions.