Table 1. Determinants of risk in paediatric pulmonary arterial hypertension.
Determinants of risk | Lower risk | Higher risk |
Clinical evidence of RV failure | No | Yes |
Progression of symptoms | No | Yes |
Syncope | No | Yes |
Growth | Failure to thrive | |
WHO functional class | I, II | III, IV |
BNP/NT-proBNP | Minimally elevated | Significantly elevated, increasing |
Echocardiography | Severe RV enlargement/dysfunction Pericardial effusion |
|
Haemodynamics | Systemic CI >3.0 L·min−1·m−2 | Systemic CI <2.5 L·min−1·m−2 |
mPAP/mSAP <0.75 mmHg | mPAP/mSAP >0.75 mmHg | |
Acute vasoreactivity | RAP >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.