The above 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, LOE). The complete list of references on the above subtopic can be found in ref. 22.

ACVRL1 (ALK1), activin-like kinase-type 1; BMPR2, bone morphogenetic protein receptor 2; BNP, brain natriuretic peptide; CAV1, caveolin 1; CHD, congenital heart disease; COR, class of recommendation; EIF2AK4, eukaryotic translation initiation factor 2-α kinase 4; ENG, endoglin; HPAH, hereditary pulmonary arterial hypertension; IPAH, idiopathic pulmonary arterial hypertension; KCNK3, potassium channel subfamily K, member 3; LOE, level of evidence; NGS, next-generation sequencing; NT-proBNP, N-terminal fragment of pro-brain natriuretic peptide; PH, pulmonary hypertension; PVOD, pulmonary veno-occlusive disease.