Determinants of paediatric idiopathic/heritable pulmonary arterial hypertension risk
Lower risk | Determinants of risk | Higher risk |
No | Clinical evidence of RV failure | Yes |
No | Progression of symptoms | Yes |
>350 | 6MWT (>6 years old) m | <350 |
Normal | Growth | Failure to thrive |
I, II | WHO FC | III, IV |
Minimally elevated | Serum BNP/NT-proBNP | Significantly elevated |
Rising level | ||
Echocardiography | RA/RV enlargement | |
Reduced LV size | ||
Increased RV/LV ratio | ||
Reduced TAPSE | ||
Low RV FAC | ||
Pericardial effusion | ||
Systemic CI >3.0 L·min−1·m−2 | Haemodynamics | Systemic CI <2.5 L·min−1·m−2 |
Systemic venous saturation >65% | mRAP >10 mmHg | |
Acute vasoreactivity | PVRI >20 WU·m2 | |
Systemic venous saturation <60% | ||
PACI <0.85 mL·mmHg−1·m−2 |
RV: right ventricle; 6MWT: 6-min walk test; WHO: World Health Organization; FC: Functional Class; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-BNP; RA: right atrium; LV: left ventricle; FAC: fractional area change; TAPSE: tricuspid annular plane systolic excursion; CI: cardiac index; mRAP: mean right atrial pressure; PVRI: pulmonary vascular resistance index; WU: Wood Units; PACI: pulmonary arterial compliance index.