BMC cardiovascular disorders · 2025
The prognostic value of HFA-PEFF score in connective tissue disease-associated PAH: evidence from a cohort study.
СтатьяНаблюдательное исследованиеПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Dai Jiayi, Ma Li, Zhang Yixin, Shan Linwei, Li Dongyu, Li Lin, Hu Qi, Zhou Zhangdi, Sun Xiaoxuan, Wang Qiang, Zhou Lei
Аннотация
BACKGROUND: Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) patients with left heart dysfunction may have worse prognosis. This study was to investigate the prognostic value of HFA-PEFF score in connective tissue disease-associated PAH.
METHODS: This single-center retrospective cohort study enrolled 147 CTD-PAH patients diagnosed via right heart catheterization (RHC), divided into two groups based on their HFA-PEFF scores: < 5 (n = 74) and ≥ 5 (n = 73). The clinical characteristics were compared between the two groups. The primary end point was all-cause mortality, and the secondary end point was clinical worsening events. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards models.
RESULTS: Compared to the HFA-PEFF score < 5 group, the ≥ 5 group exhibited significantly higher levels of N-terminal pro-brain natriuretic peptide (NT-proBNP), a greater proportion of WHO functional class III-IV, shorter 6-min walk distance (6MWD), larger right ventricular (RV) volume, worse RV function, smaller left ventricular (LV) volume, and higher native T1 values. An HFA-PEFF score ≥ 5 was a predictor for all-cause mortality in CTD-PAH (HR 5.022, P = 0.020) and for clinical worsening events (HR 2.670, P = 0.020). At follow-up, 17.9% of CTD-PAH had an HFA-PEFF score ≥ 5. Patients with follow-up HFA-PEFF scores ≥ 5 had a significantly lower event-free survival rate (P < 0.001).
CONCLUSION: An HFA-PEFF score ≥ 5 was associated with all-cause mortality and clinical worsening events in CTD-PAH patients.
TRIAL REGISTRATION: NCT05980728. Connective Tissue Disease Patients With Pulmonary Hypertension. Oct 17, 2023.
METHODS: This single-center retrospective cohort study enrolled 147 CTD-PAH patients diagnosed via right heart catheterization (RHC), divided into two groups based on their HFA-PEFF scores: < 5 (n = 74) and ≥ 5 (n = 73). The clinical characteristics were compared between the two groups. The primary end point was all-cause mortality, and the secondary end point was clinical worsening events. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards models.
RESULTS: Compared to the HFA-PEFF score < 5 group, the ≥ 5 group exhibited significantly higher levels of N-terminal pro-brain natriuretic peptide (NT-proBNP), a greater proportion of WHO functional class III-IV, shorter 6-min walk distance (6MWD), larger right ventricular (RV) volume, worse RV function, smaller left ventricular (LV) volume, and higher native T1 values. An HFA-PEFF score ≥ 5 was a predictor for all-cause mortality in CTD-PAH (HR 5.022, P = 0.020) and for clinical worsening events (HR 2.670, P = 0.020). At follow-up, 17.9% of CTD-PAH had an HFA-PEFF score ≥ 5. Patients with follow-up HFA-PEFF scores ≥ 5 had a significantly lower event-free survival rate (P < 0.001).
CONCLUSION: An HFA-PEFF score ≥ 5 was associated with all-cause mortality and clinical worsening events in CTD-PAH patients.
TRIAL REGISTRATION: NCT05980728. Connective Tissue Disease Patients With Pulmonary Hypertension. Oct 17, 2023.
Предметные рубрики MeSH
AdultAgedFemaleHumansMaleMiddle AgedArterial PressureBiomarkersCardiac CatheterizationConnective Tissue DiseasesDisease ProgressionNatriuretic Peptide, BrainPeptide FragmentsPredictive Value of TestsPrognosisPulmonary Arterial HypertensionRetrospective StudiesRisk AssessmentRisk FactorsTime FactorsVentricular Dysfunction, LeftVentricular Function, LeftVentricular Function, Right