TABLE 3.
[Ref.] | Risk assessment tools | Predictors of survival | Registry used for validation | ||
NIH registry | [7] | IPAH | Risk equation | NYHA, RAP, PAP, CI, DLCO | |
PHC registry | [6] | PAH | Age, CTD, NYHA, RAP, PAP, CI | ||
IPAH | Risk equation | Age, NYHA, RAP, PAP, CI | |||
French registry | [8] | PAH | REVEAL | ||
IPAH | Risk equation | Sex, 6MWD, CO | |||
REVEAL | [46] | PAH | Risk equation and risk score table | PVR, RAP, PO-PAH-FPAH-CTD, NYHA, sex, age, renal, BP, HR, 6MWD, BNP, pericardial effusion, DLCO | French registry UK registry Spanish registry |
IPAH | |||||
Scottish/UK | [10] | PAH | Risk score | Age, sex, aetiology, 6MWD, RAP, CO | UK registry |
IPAH | |||||
UK | [47] | ||||
Spanish | [11] | PAH | Sex, NYHA, RAP, CI, CTD-PO-PAH-PVOD | ||
IPAH | |||||
COMPERA | [12] | IPAH | Sex, 6MWD | ||
ESC/ERS | [36] | Risk score table | Swedish registry | ||
COMPERA registry French registry |
NIH: National Institute of Health; PHC: Pulmonary Hypertension Connection registry; REVEAL: Registry To Evaluate Early and Long-Term Pulmonary Arterial Hypertension; COMPERA: Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension; ESC/ERS: European Society of Cardiology/European Respiratory Society; IPAH: idiopathic PAH; NYHA: New York Heart Association; RAP: right atrial pressure; PAP: pulmonary arterial pressure; CI: cardiac index; DLCO: diffusion capacity for carbon monoxide; CTD: connective tissue disease; 6MWD: 6-min walking distance; CO: cardiac output; PVR: pulmonary vascular resistance; PO-PAH: porto-PAH; FPAH: familial PAH; BP: blood pressure; HR: heart rate; BNP: brain natriuretic peptide; PVOD: pulmonary veno-occlusive disease.