Table 1. Determinants of risk in paediatric pulmonary arterial hypertension
Determinants of riskLower riskHigher risk
Clinical evidence of RV failureNoYes
Progression of symptomsNoYes
GrowthFailure to thrive
WHO functional classI, IIIII, IV
BNP/NT-proBNPMinimally elevatedSignificantly elevated, increasing
EchocardiographySevere RV enlargement/dysfunction
Pericardial effusion
HaemodynamicsSystemic CI >3.0 L·min−1·m−2Systemic CI <2.5 L·min−1·m−2
mPAP/mSAP <0.75 mmHgmPAP/mSAP >0.75 mmHg
Acute vasoreactivityRAP >10 mmHg
PVRI >20 WU·m−2
  • RV: right ventricular; WHO: World Health Organization; BNP: B-type natriuretic peptide; NT-proBNP: N-terminal pro-BNP; CI: cardiac index; mPAP: mean pulmonary artery pressure; mSAP: mean systemic artery pressure; RAP: right atrial pressure; PVRI: pulmonary vascular resistance index; WU: Wood unit. Reproduced from [18] with permission from the publisher.