Comparison of European Society of Cardiology (ESC)/European Respiratory Society (ERS) and REVEAL 2.0 prognostic tools
| Variable | ESC/ERS guidelines | REVEAL 2.0 risk score calculator# | ||||
| Low risk: <5% | Intermediate risk: 5–10% | High risk: >10% | −1 unless indicated | +1 | +2 unless indicated | |
| WHO PH Group 1 subgroup | APAH-CTD | Heritable PoPH (+3) | ||||
| Demographics | Male, age >60 years | |||||
| Clinical signs of right heart failure | Absent | Absent | Present | |||
| Comorbidities | eGFR<60 mL·min−1/1.73 m2 or renal insufficiency | |||||
| Symptom progression | No | Slow | Rapid | |||
| Vital signs | SBP <110 mmHg HR >96 beats·min−1 | |||||
| Syncope | No | Occasional syncope | Repeated syncope | |||
| All-cause hospitalisations ≤6 months | ≥1 | |||||
| NYHA/WHO FC | I, II | III | IV | I | III | IV |
| 6MWD | >440 m | 165–440 m | <165 m | ≥440 m (−2) 320–<440 m | <165 m | |
| CPET | Peak V′O2 >15 mL·min−1·kg−1 (>65% pred) V′E/V′CO2 slope <36 | Peak V′O2 11–15 mL·min−1·kg−1 (35–65% pred) V′E/V′CO2 slope 36–44.9 | Peak V′O2 <11 mL·min−1·kg−1 (<35% pred) V′E/V′CO2 slope ≥44.9 | |||
| BNP/NT-proBNP plasma levels | BNP <50 ng·L−1 NT-proBNP <300 ng·L−1 | BNP 50–300 ng·L−1 NT-proBNP 300–1400 ng·L−1 | BNP >500 ng·L−1 NT-proBNP >1400 ng·L−1 | BNP<50 ng·L−1 NT-proBNP <300 ng·L−1 | BNP 200–800 ng·L−1 | BNP ≥800 ng·L−1 NT-proBNP ≥1100 ng·L−1 |
| Imaging/echocardiography | RA area <18 cm2 No pericardial effusion | RA area 18–26 cm2 No or minimal pericardial effusion | RA area >26 cm2 Pericardial effusion | Pericardial effusion | ||
| Haemodynamics/right heart catheterisation | RAP <8 mmHg CI ≥2.5 L/min/m2 SvO2 >65% | RAP 8–14 mmHg CI 2.0–2.4 L/min/m2 SvO2 60–65% | RAP >14 mmHg CI <2.0 L/min/m2 SvO22 <60% | PVR <5 Wood units | Mean RAP >20 mmHg within 1 year | |
| Pulmonary function test | DLCO <40% pred | |||||
ESC/ERS guidelines state that, while not all variables need to be assessed, WHO FC and at least one measurement of exercise capacity (6MWD or CPET) should be taken as a minimum, and assessment of right ventricular (RV) function (BNP/NT-proBNP or echocardiography) is recommended. REVEAL 2.0 includes 11 variables including modifiable and non-modifiable with each score corresponding to a risk for mortality at 1 year. To mirror the ESC/ERS approach the REVEAL score can also be split into three groups (low risk ≤6, intermediate risk 7–8, high risk ≥9) [1, 9]. REVEAL: Registry to Evaluate Early And Long-term PAH Disease Management; WHO: World Health Organization; PH: pulmonary hypertension; NYHA: New York Heart Association; FC: functional class; CPET: cardiopulmonary exercise test; 6MWD: 6-min walk distance; BNP: B-type natriuretic peptide; NT-proBNP: N-terminal pro-hormone of BNP; APAH-CTD: pulmonary arterial hypertension associated with connective tissue disease; PoPH: portopulmonary hypertension; eGFR: estimated glomerular filtration rate; SBP: systolic blood pressure; HR: heart rate; V′O2: oxygen consumption; V′E: minute ventilation; V′CO2: carbon dioxide production; RA: right atrium; RAP: right atrial pressure; CI: cardiac index; SvO2: mixed venous oxygen saturation; PVR: pulmonary vascular resistance; DLCO: diffusing capacity of the lungs for carbon monoxide. #: add/subtract for each variable to get overall score.