Additional Role of Unmodifiable Risk Factors in Pulmonary Arterial Hypertension Risk Stratification According to Current ESC/ERS Guidelines

Author(s):  
F. Dardi ◽  
A. Manes ◽  
M. Palazzini ◽  
E. Gotti ◽  
A. Rinaldi ◽  
...  
2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
E Zuffa ◽  
F Dardi ◽  
M Palazzini ◽  
E Gotti ◽  
A Rinaldi ◽  
...  

Abstract Background Current pulmonary hypertension (PH) guidelines stratify the risk of patients with pulmonary arterial hypertension (PAH) using a multiparametric approach. Anyway, the role of unmodifiable risk factors is not taken into account. Purpose The aim of this study was to evaluate the role of unmodifiable risk factors (age, gender, PAH aetiology) in PAH risk stratification using the recently proposed simplified risk table and to test if these factors influence the response to PAH-specific treatment. Methods All patients with PAH referred to a single centre were included from 2003 to 2017. We applied a simplified risk assessment strategy using the following criteria: WHO functional class, 6-min walking distance, right atrial pressure or brain natriuretic peptide plasma levels and cardiac index (CI) or mixed venous oxygen saturation (SvO2). The last 2 criteria were based on which parameter was available; if both were available the worst was chosen. Risk strata were defined as: Low risk= at least 3 low risk and no high-risk criteria; High risk= at least 2 high risk criteria including CI or SvO2; Intermediate risk= definitions of low or high risk not fulfilled. Then we performed multivariate Cox analysis to evaluate what are the independent predictors of survival (age, gender, PAH aetiology together with the recently proposed simplified PAH risk table) and we tested if these factors influence the response to PAH specific therapy comparing the % improvement of hemodynamic parameters from baseline to 3–4 months after starting treatment. Wilcoxon-Mann-Whitney test was used for comparisons. Results Six hundreds and twenty-one treatment-naïve patients were enrolled. Age [HR (95% CI) = 1.022 (1.014–1.030); p-value <0.001], male gender [HR (95% CI) = 1.881 (1.479–2.392); p-value <0.001] and connective tissue disease (CTD)-PAH aetiology [HR (95% CI)= 2.278 (1.733–2.995); p-value <0.001] were all independent predictors of prognosis in patients with PAH together with the recently validated simplified PAH risk table [HR (95% CI) = 2.161 (1.783–2.618); p-value <0.001] but they didn't significantly influence the response to PAH specific treatment as shown in the Figure. Figure 1 Conclusions Age, gender and CTD-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. Acknowledgement/Funding None


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Rotunno ◽  
F Dardi ◽  
A De Lorenzis ◽  
M Palazzini ◽  
E Zuffa ◽  
...  

Abstract Background Current pulmonary hypertension (PH) guidelines stratify the risk of patients with pulmonary arterial hypertension (PAH) using a multiparametric approach. A simplified risk table has been recently proposed and validated without including echocardiographic parameters. Purpose We evaluate the additional role of echocardiogram in PAH risk stratification using the recently proposed simplified risk table in patients with idiopathic/heritable (I/H) PAH and PAH associated with connective tissue disease (CTD) and congenital heart disease (CHD). Methods All patients with I/H-PAH, CTD-PAH and CHD-PAH referred to a single centre were included from 2003 to 2017. All patients were treated according to PH guidelines. The simplified risk assessment considered the following criteria: WHO functional class, 6-min walking distance, right atrial (RA) pressure or brain natriuretic peptide (BNP) plasma levels and cardiac index (CI) or mixed venous oxygen saturation (SvO2). For the last 2 criteria the worst parameter was chosen. Risk strata were defined as: Low risk= at least 3 low risk and no high-risk criteria; High risk= at least 2 high risk criteria including CI or SvO2; Intermediate risk= definitions of low or high risk not fulfilled. Then we performed a Cox analysis to evaluate the independent echocardiographic predictors of survival that were subsequently added to the simplified risk table to test their additional role in ameliorating risk stratification. Results 461 treatment-naïve patients were enrolled. Echocardiographic independent predictors of prognosis were the severity of tricuspid regurgitation [HR (95% CI) = 1.013 (1.006–1.021); p-value = 0.001], right atrial area [HR (95% CI) = 1.028 (1.012–1.045); p-value = 0.001] and the presence of pericardial effusion [HR (95% CI) = 1.533 (1.142–2.057); p-value= 0.004]. Only RA area significantly ameliorate the risk stratification power of the recently validated simplified PAH risk table (likelihood ratio chi2 increased from 63.8 to 68.1, likelihood ratio test = 0.039). Due to the significant correlation between RA area and both RA pressure (r=0.470; p&lt;0.001) and BNP (r=0.372; p=0.004), we elaborate a second risk table in which RA area (utilizing the cut-offs proposed by the current PH guidelines) was considered together within the criteria including RA pressure and BNP (the worst parameter of the 3 was considered). Considering the second risk table including RA area the risk stratification power significantly improved (likelihood ratio chi2 increased from 63.8 to 72.7, AIC/BIC decreased from 1956/1964 to 1947/1955). Conclusions Echocardiographic RA area significantly improve the risk discrimination power of the recently proposed simplified risk table for patients with PAH. Funding Acknowledgement Type of funding source: None


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