No | Clinical evidence of RV failure | Yes |
Slow | Rate of progression of symptoms | Rapid |
No | Syncope | Yes |
I and II | WHO FC | IV |
Longer (>500 m)# | 6MWT | Shorter (<300 m) |
Peak V′O2 >15 mL·min−1·kg−1 | Cardiopulmonary exercise testing | Peak V′O2 <12 mL·min−1·kg−1 |
Normal or near normal | BNP/NT-proBNP plasma levels | Very elevated and rising |
No pericardial effusion TAPSE¶ >2.0 cm | Echocardiographic findings¶ | Pericardial effusion TAPSE¶ <1.5 cm |
RAP <8 mmHg and CI ≥2.5 L·min−1·m−2 | Haemodynamics | RAP >15 mmHg or CI ≤2.0 L·min−1·m−2 |