Key Points
➤ The estimated incidence of sustained pulmonary hypertension
(PH) in children of all groups is 4–10 patients per million per
year in European countries and 5–8 patients per million per
year in the United States.
➤ In 2018, the 6th World Symposium on Pulmonary
Hypertension (WSPH) modified the definition for PH in adults
to a mean pulmonary artery pressure of >20 mmHg and
included pulmonary vascular resistance of ≥3 Woods Units
to identify pre-capillary PH. For consistency across all age
groups, the Pediatric Task Force of the WSPH followed suit
and adopted the same definition.
➤ This is the first document of its kind to use systematic
reviews and the GRADE approach to assess the roles of
atrial septal defect (ASD) creation/enlargement (what will be
termed ASD-intervention), pulmonary-to-systemic shunts, and
lung transplantation in children with PH.
➤ This document is largely focused on children with Group 1
PH.
• Under the correct circumstances, the interventions addressed in this guideline
may be appropriate for children with other forms of progressive PH.
