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. 2019 Dec 18;28(154):190050. doi: 10.1183/16000617.0050-2019

TABLE 3.

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

[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.