Treatment
Recommendation 1
➤ We suggest ASD-intervention for children with progressive
PH and right ventricular (RV) failure despite optimal
therapy (conditional recommendation, very low certainty of
evidence).
Recommendation 2
➤ We suggest creation of a pulmonary-to-systemic shunt in
children with progressive PH and suprasystemic RV pressure
despite optimal therapy (conditional recommendation, very
low certainty of evidence).
Recommendation 3
➤ We suggest lung transplantation in children with progressive
PH and RV failure despite optimal therapy (conditional
recommendation, very low certainty of evidence).
Recommendation 4
➤ We suggest lung transplantation in children with progressive
PH and RV failure despite optimal therapy who are on
extracorporeal membrane oxygenation (ECMO) support
without reversible cause (conditional recommendation, very
low certainty of evidence).
Recommendation 5
➤ We suggest against pulmonary-to-systemic shunt creation for
children with progressive PH and suprasystemic RV pressure
despite optimal therapy who are on ECMO support without
reversible cause (conditional recommendation, very low
certainty of evidence).