Overview of some of the risk assessment tools for pulmonary arterial hypertension (PAH)
| Risk assessment tool | Year of publication | Patients included | PAH subgroup | Variables included | Survival estimation | ||||
| Clinical | Exercise | Laboratory | Imaging | Haemodynamic | |||||
| NIH equation [37] | 1991 | Incident | IPAH | # | # | # | # | 1. mPAP 2. mRAP 3. CI | 1-, 3-, 5-year |
| PH connection registry [38] | 2010 | Incident and prevalent | IPAH HPAH APAH | # | # | # | # | 1. mPAP 2. mRAP 3. CI | 1-, 3-, 5-year |
| FPHN registry risk equation [39, 40] | 2010 | Incident and prevalent | IPAH HPAH APAH | 1. Sex | 2. 6MWD | # | # | 3. CO | 1-, 2-, 3-year |
| REVEAL registry [41] | 2010 | Incident and prevalent | Group 1 PAH | 1. Age 2. Sex 3. PAH subgroup 4. Renal insufficiency 5. FC 6. SBP 7. HR | 8. 6MWD | 9. BNP | 10. Pericardial effusion 11. DLCO | 12. PVR 13. mRAP | 1-year |
| Scottish composite score [42] | 2012 | Incident | Group 1 PAH (CHD-PAH and CCB-R excluded) | 1. Age 2. Sex 3. PAH subgroup | 4. 6MWD | # | # | 5. mRAP 6. CO | 1-, 2-, 3-year |
| Nickel et al. [43] | 2012 | Incident | IPAH | # FC, NT-proBNP, CI, SvO2 at follow-up | 1. 6MWD | 2. NT-proBNP | # | 3. CI 4. SvO2 | 1-, 3-, 5-year |
| ESC/ERS 2015 Guidelines [3] | 2015 | # | Group 1 PAH | 1. Progression of symptoms 2. Syncope 3. FC 4. Clinical signs RHF | 5. 6MWD 6. Peak VʹO2 (CPET) 7. VʹE/ VʹCO2 slope (CPET) | 8. BNP/NT-proBNP | 9. RA area 10. Pericardial effusion | 11. RAP 12. CI 13. SvO2 | 1-year |
| FPHN registry [44] | 2017 | Incident | IPAH HPAH DPAH | 1. FC FC, 6MWD, BNP/NT-proBNP at first follow-up | 2. 6MWD | # | # | 3. mRAP 4. CI | 1–5 years |
| COMPERA registry [45] | 2017 | Incident | Group 1 PAH | 1. FC | 2. 6MWD | 3. BNP/NT-proBNP | # | 4. mRAP 5. CI | 1–5 years |
| SAPHR registry [46] | 2018 | Incident | Group 1 PAH | 1. FC | 2. 6MWD | 3. NT-proBNP | 4. RA area 5. Pericardial effusion | 6. mRAP 7. CI 8. SvO2 | 1-, 3-, 5-years |
| Modified Risk Assessment Score of PAH [47] | 2018 | Incident and prevalent | Group 1 PAH | 1. FC | 2. 6MWD | 3. BNP | 4. RA area | # | 1-year |
| COMPERA 2.0 [48] | 2021 | Incident | Group 1 PAH | 1. FC | 2. 6MWD | 3. BNP/NT-proBNP | # | # | 1-, 3-, 5-years |
| ESC/ERS 2022 Guidelines [2] | 2022 | # | Group 1 PAH | 1. Progression of symptoms 2. Syncope 3. FC 4. Clinical signs RHF | 5. 6MWD 6. Peak VʹO2 (CPET) 7. VʹE/ VʹCO2 slope (CPET) | 8. BNP/NT-proBNP | 9. Echocardiography: RA area Pericardial effusion TAPSE/PASP 10. cMRI: RVEF SVI RVESVI | 11. RAP 12. CI 13. SvO2 14. SVI | 1-year |
NIH: National Institutes of Health; PH: pulmonary hypertension; FPHN: French PH Network; REVEAL: US Registry to Evaluate Early and Long-term PAH Disease Management; ESC/ERS: European Society of Cardiology/European Respiratory Society; COMPERA: Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension; SAPHR: Swedish PAH register; IPAH: idiopathic PAH; HPAH: hereditary PAH; APAH: anorexigen-associated PAH; CHD: congenital heart disease; CCB-R: long-term responder to calcium channel blockers; DPAH: drug-associated PAH; FC: functional class; SBP: systolic blood pressure; HR: heart rate; RHF: right heart failure; 6MWD: 6-min walk distance; BNP: B-type natriuretic peptide; NT-proBNP: N-terminal pro-brain natriuretic peptide; DLCO: diffusion capacity of the lung for carbon monoxide; RA area: right atrium area; mPAP: mean pulmonary artery pressure; mRAP: mean right atrial pressure; CI: cardiac index; CO: cardiac output; PVR: pulmonary vascular resistance; SvO2: mixed venous oxygen saturation; TAPSE/PASP: tricuspid annular plane systolic excursion/pulmonary arterial systolic pressure; RVEF: right ventricular ejection fraction; SVI: stroke volume index; RVESVI: right ventricular end-systolic volume index; VʹO2: oxygen uptake; CPET: cardiopulmonary exercise testing; VʹE: minute ventilation. #: absent data.