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Background <p>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.</p> Methods <p>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: &lt; 5 (<i>n</i> = 74) and ≥ 5 (<i>n</i> = 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.</p> Results <p>Compared to the HFA-PEFF score &lt; 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, <i>P</i> = 0.020) and for clinical worsening events (HR 2.670, <i>P</i> = 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 (<i>P</i> &lt; 0.001).</p> Conclusion <p>An HFA-PEFF score ≥ 5 was associated with all-cause mortality and clinical worsening events in CTD-PAH patients.</p> Trial registration <p>NCT05980728. Connective&#xa0;Tissue&#xa0;Disease&#xa0;Patients&#xa0;With&#xa0;Pulmonary&#xa0;Hypertension. Oct 17, 2023.</p>

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The prognostic value of HFA-PEFF score in connective tissue disease-associated PAH: evidence from a cohort study

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年4月5日

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  • Jiayi Dai,
  • Li Ma,
  • Yixin Zhang,
  • Linwei Shan,
  • Dongyu Li,
  • Lin Li,
  • Qi Hu,
  • Zhangdi Zhou,
  • Xiaoxuan Sun,
  • Qiang Wang,
  • Lei Zhou

摘要

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.