Response to treatment for pulmonary arterial hypertension (PAH) is not significantly affected by connective tissue disease-PAH etiology, gender, or age; however, these factors do have a significant impact on prognosis in combination with the simplified PAH risk table. This research was presented at the American College of Cardiology 68th Annual Scientific Session & Expo, held March 16-18, 2019 in New Orleans, Louisiana.

This study included 621 individuals with PAH who had not undergone treatment prior to study initiation. These participants were treated in a single center between 2003 and 2017. In order to assess risk in a simplified model, the following criteria were considered: 6-minute walking test, WHO functional class, right atrial pressure or levels of brain natriuretic peptide plasma, and either mixed venous oxygen saturation (SvO2) or cardiac index. For the SvO2 or cardiac index criteria, either the only available one or worst available one was chosen.

High risk was defined as having at least 2 high-risk criteria, including SvO2 or cardiac index; low risk was defined as no high-risk criteria and at least 3 low-risk criteria; medium risk was reserved for those cases that fit neither of the previous definitions.

Independent survival predictors were identified using Cox analysis (for gender, age, PAH etiology + simplified risk table). These factors’ influence on response to treatment was examined by comparing hemodynamic parametric improvement between baseline and the 3- to 4-month mark using the Wilcoxon-Mann-Whitney test.

Independent predictors of prognosis were etiology (hazard ratio [HR] 2.278; 95% CI, 1.733-2.995), simplified risk table (HR 2.161; 95% CI, 1.783-2.618), being male (HR 1.881; 95% CI, 1.479-2.392), and age (HR 1.022; 95% CI, 1.014-1.030); P <.001 for all. However, etiology, age, and gender did not have a significant effect on treatment response.

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The study researchers conclude that “[age], gender and [connective tissue disease-PAH] aetiology significantly influence prognosis together with the recently validated simplified PAH risk table but don’t significantly influence the response to PAH-specific treatment.”


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: American College of Cardiology 68th Annual Scientific Session & Expo; March 16-18, 2019; New Orleans, LA. Abstract 1142-491.