PAH Prognosis Affected by Connective Tissue Disease-PAH Etiology

This article is part of MPR‘s coverage of the American Thoracic Society International Conference, taking place in Dallas, Texas. Our staff will report on medical research related to asthma and other respiratory conditions, conducted by experts in the field. Check back regularly for more news from ATS 2019.

DALLAS — In patients with pulmonary arterial hypertension (PAH), factors that affect prognosis but not treatment response included age, gender, and connective tissue disease-PAH etiology, and these factors may help stratify survival risk in these patients, according to study findings presented at the American Thoracic Society International Conference held May 17-22, in Dallas, Texas.

“Current pulmonary hypertension guidelines stratify the risk of patients with PAH using a multiparametric approach,” according to the researchers. “[T]he role of unmodifiable risk factors is not taken into account.” Therefore, the purpose of this study was to evaluate the role of unmodifiable risk factors (age, gender, PAH etiology) in PAH risk stratification using the recently proposed simplified risk table and to determine whether these factors affect the response to PAH-specific treatment.

Researchers enrolled a total of 621 treatment-naive patients with PAH who were referred to a single Italian center between 2003 and 2017. A simplified risk assessment tool that included World Health Organization functional class, 6-minute walking distance, right atrial pressure or brain natriuretic peptide plasma levels, and cardiac index (CI) or mixed venous oxygen saturation (SvO2) were applied to the cohort. Of the criteria applied, the worst parameter of the last 2 criteria (if available) was chosen. Patients were categorized as being low risk (≥3 low-risk and no high-risk criteria), high risk (≥2 high-risk criteria, including CI or SvO2), or intermediate risk (low- or high-risk criteria not fulfilled).

A multivariate Cox analysis was performed to determine the independent predictors of prognosis, specifically survival. Independent predictors investigated in the study included age, gender, and PAH etiology in combination with a recently proposed simplified PAH risk tool. The researchers also examined whether these predictors influenced the response to PAH-specific therapy. The percent improvement of hemodynamic parameters was compared from baseline to 3- to 4-month follow-up.

Independent predictors of prognosis in the cohort included age [hazard ratio [HR], 1.022; 95% CI, 1.014-1.030; P <.001), male sex (HR, 1.881; 95% CI, 1.479-2.392; P <.001), and connective tissue disease-PAH etiology (HR, 2.278; 95% CI, 1.733-2.995; P <.001) combined with the simplified PAH risk assessment (HR, 2.161; 95% CI, 1.783-2.618; P <.001). However, these predictors did not appear to affect the response to PAH-specific treatment.

Study limitations included the inclusion of patients from a single center in Italy and the lack of a randomized control group.

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Reference

Dardi F, Manes A, Palazzini M, et al. Additional role of unmodifiable risk factors in pulmonary arterial hypertension risk stratification according to current ESC/ERS guidelines. Presented at: the American Thoracic Society International Conference; May 20, 2019; Dallas, TX. Abstract A3650/P995.

This article originally appeared on Pulmonology Advisor