Key result
Simplified four-variable ESC/ERS risk table effectively stratifies 1-year mortality across pulmonary arterial hypertension subgroups.
Why the study?
The 2015 ESC/ERS pulmonary arterial hypertension risk stratification strategy had been validated in overall PAH cohorts, but its validity across specific clinical subgroups (idiopathic/heritable, connective tissue disease-associated, and congenital heart disease-associated PAH) using a simplified risk table remained to be determined.
Does a simplified risk assessment table based on the 2015 ESC/ERS guidelines accurately stratify prognosis and survival in patients with I/H-PAH, CTD-PAH, and CHD-PAH?
Cohort
No
Does a simplified risk assessment table based on the 2015 ESC/ERS guidelines accurately stratify prognosis and survival in patients with I/H-PAH, CTD-PAH, and CHD-PAH?
Should not yet change PAH risk stratification practice; leaves open prospective validation of the simplified table.
Background: The 2015 ESC/ERS pulmonary hypertension (PH) guidelines propose a risk stratification strategy for patients with pulmonary arterial hypertension (PAH) based on a multiparametric approach. Low-, intermediate- and high-risk strata are defined by estimated 1-year mortality risks of <5%, 5–10% and >10%, respectively. This risk assessment has been recently validated in three cohort of PAH patients but not in all specific clinical subgroups of patients. Purpose: The aim of this study was to determine the validity of a simplified risk table in stratifying the prognosis of patients with idiopathic/heritable (I/H) PAH and PAH associated with connective tissue disease (CTD) and congenital heart disease (CHD). Methods: All consecutive patients with I/H-PAH, CTD-PAH and CHD-PAH referred to a single centre were included in the study from 1995 to December 2017. All patients were treated according with current PH guidelines. A simplified version of the risk assessment strategy proposed by the ESC/ERS-PH guidelines was applied, using the following variables at baseline evaluation: WHO functional class, 6-min walking distance, right atrial pressure (RAP) and mixed venous oxygen saturation (SvO2). Risk strata were defined as follow: Low risk= at least 3 low risk determinants and no high-risk determinants; High risk= all four high risk determinants or 3 high-risk determinants including SvO2 or 2 high risk determinants including SvO2 and RAP; Intermediate risk= when the above definitions of low or high risk are not fulfilled. Kaplan Meier curves and Log-rank test were used for survival analysis.
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Dardi et al. (2018) conducted a cohort in Pulmonary arterial hypertension (idiopathic/heritable, connective tissue disease-associated, and congenital heart disease-associated). Simplified ESC/ERS risk assessment table (using WHO functional class, 6-minute walking distance, right atrial pressure, and mixed venous oxygen saturation) vs. Low, intermediate, and high risk strata (defined by estimated 1-year mortality risks of <5%, 5-10%, and >10%) was evaluated on Survival / prognosis stratification. A simplified 4-variable ESC/ERS risk table was evaluated to stratify 1-year mortality (<5%, 5-10%, >10%) in PAH subgroups, though specific outcome results were omitted from the abstract.
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