Better prognosis | Determinants of prognosis | Worse prognosis |
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 |
RV: right ventricle; WHO FC: World Health Organization functional class; 6MWT: 6-min walking test; V′O2: oxygen uptake; BNP: brain natriuretic peptide; TAPSE: tricuspid annular plane systolic excursion; RAP: right arterial pressure; CI: cardiac index. #: depending on age; ¶: TAPSE and pericardial effusion have been selected as they can be measured in the majority of patients. Reproduced from [4] with permission from the publisher.