TABLE 3

Comparison of survival time and mortality prediction scores for pulmonary arterial hypertension (PAH)

[Ref.]Risk assessment toolsPredictors of survivalRegistry used for validation
NIH registry[7]IPAHRisk equationNYHA, RAP, PAP, CI, DLCO
PHC registry[6]PAHAge, CTD, NYHA, RAP, PAP, CI
IPAHRisk equationAge, NYHA, RAP, PAP, CI
French registry[8]PAHREVEAL
IPAHRisk equationSex, 6MWD, CO
REVEAL[46]PAHRisk equation and risk score tablePVR, RAP, PO-PAH-FPAH-CTD, NYHA, sex, age, renal, BP, HR, 6MWD, BNP, pericardial effusion, DLCOFrench registry
UK registry
Spanish registry
IPAH
Scottish/UK[10]PAHRisk scoreAge, sex, aetiology, 6MWD, RAP, COUK registry
IPAH
UK[47]
Spanish[11]PAHSex, NYHA, RAP, CI, CTD-PO-PAH-PVOD
IPAH
COMPERA[12]IPAHSex, 6MWD
ESC/ERS[36]Risk score tableSwedish 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.