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 |