Abstract
Background
Risk scores are important tools for the prognostic stratification of pulmonary arterial
hypertension (PAH). Their performance and the additional impact of comorbidities across
age groups is unknown.
Methods
Patients with PAH enrolled from 2001 to 2021 were divided in ≥65 years old vs <65 years old patients. Study outcome was 5-year all-cause mortality.
FPHN, FPHN non-invasive, COMPERA and REVEAL 2.0 risk scores were calculated and patients
categorized at low, intermediate and high risk. Number of comorbidities was calculated.
Results
Among 383 patients, 152 (40%) were ≥65 years old. They had more comorbidities (number of comorbidities 2, IQR 1-3, vs
1, IQR 0-2 in <65 years patients). 5-year survival was 63% in ≥65 vs 90% in <65 years. Risk scores correctly discriminated the different classes
of risk in the overall cohort and in the older and younger groups. REVEAL 2.0 showed
the best accuracy in the total cohort (C-index 0.74, standard error -SE- 0.03) and
older (C-index 0.69, SE 0.03) patients, whereas COMPERA 2.0 performed better in younger
patients (C-index 0.75, SE 0.08). Number of comorbidities was associated with higher
5-year mortality, and consistently increased the accuracy of risk scores, in younger
but not in older patients.
Conclusions
Risk scores have similar accuracy in the prognostic stratification of older vs younger
PAH patients. REVEAL 2.0 had the best performance in older patients and COMPERA 2.0
had it in younger patients. Comorbidities increased the accuracy of risk scores only
in younger patients.
Keywords
List of Abbreviation:
PAH (Pulmonary arterial hypertension), ESC (European Society of Cardiology), ERS (European Respiratory Society), REVEAL 2.0 (Registry to Evaluate Early and Long-term PAH Disease Management 2.0), WHO (World Health Organization), CTD (connective tissue diseases), RHC (right heart catheterization), PH (pulmonary hypertension), mPAP (mean pulmonary artery pressure), PAWP (pulmonary artery wedge pressure), PVR (pulmonary vascular resistance), FPHN (French Pulmonary Hypertension Network), COMPERA 2.0 (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension 2.0), sHTN (systemic hypertension), IHD (ischemic heart disease), AF (atrial fibrillation), DM (diabetes mellitus), CKD (chronic kidney disease), COPD (chronic obstructive pulmonary disease), BNP (brain natriuretic peptide), NT-proBNP (n-terminal pro BNP), 6MWD (6-minute walking test), CI (cardiac index), DLCO (diffusing lung capacity for carbon monoxide)To read this article in full you will need to make a payment
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