The recommendations of the European Paediatric Pulmonary Vascular Disease Network relate to the grading system currently suggested by the European Society of Cardiology and the American Heart Association and are based on paediatric data only (class, level of evidence). The complete list of references on the above subtopic can be found in ref. 29. Recommendations on PH therapy, specifically in neonatal chronic lung disease and PPHN,27 and PAH associated with congenital heart disease,26 and the recommendations on acute PH in the intensive care unit,28 are outlined in the according subtopic articles of this expert consensus statement.

AVT, acute vasodilator testing; CCB, calcium channel blocker; COR, class of recommendation; HPAH, hereditary pulmonary hypertension; iNO, inhaled nitric oxide; IPAH, idiopathic pulmonary arterial hypertension; LOE, level of evidence; LPA, left pulmonary artery; LV, left ventricle; PAH, pulmonary arterial hypertension; PAP, pulmonary artery pressure; PH, pulmonary hypertension; PPHN, persistent pulmonary hypertension of the newborn; PPHVD, paediatric pulmonary hypertensive vascular disease; RCT, randomised controlled trial; RV, right ventricle.