Risk stratification and prognostic factors in patients with pulmonary arterial hypertension and comorbidities a cross-sectional cohort study with survival follow-up

Abstract

Background: The objective of this study was to analyze prognostic factors and risk stratification in patients with pulmonary arterial hypertension (PAH) and comorbidities. Methods: Patients with invasively diagnosed PAH were included in the analysis. Comorbidities were clinically diagnosed as proposed in the 6th World Symposium of pulmonary hypertension. Uni-and multivariate analysis were employed for identification of factors predicting survival and time to first clinical worsening (TTCW). Risk stratification was based on parameters from ESC/ERS-guidelines 2015. Results: In total 142 patients were enrolled in the study, 90 of them were diagnosed as PAH without and 52 with comorbidities. All patients received targeted PAH therapy and were followed for 3.3 ± 2.4 years. In PAH patients without comorbidities survival and TTCW were significantly associated with reduced 6-min walking distance (6MWD), elevated N-terminal pro brain natriuretic peptide (NT-proBNP), WHO-functional class (WHO-FC) and right atrial (RA) area. In the multivariate analysis, 6MWD was an independent predictor for survival (p = 0.002) and WHO-FC for TTCW (p = 0.001). In patients with PAH and comorbidities these parameters had no significant association with survival and TTCW. Average risk score was significantly associated with survival (p = 0.001) and TTCW (p = 0.013) in PAH but not in PAH with comorbidities (both p > 0.05; figure 1).

The impact of cardiovascular comorbidities associated with risk for left heart disease on idiopathic pulmonary arterial hypertension: Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

“…In this real‐life nationwide cohort, almost one to five patients (19.5%) with IPAH have equal or more than three CVCs associated with increased risk for left heart disease, which is in accordance with European cohorts. 11 Compared to IPAH patients with fewer CVCs, patients with ≥3 CVCs have a different phenotype in terms of sex distribution and age and different clinical and hemodynamic characteristics (worse functional status, lower mPAP, and PVR).…”

“…As stated in a previous study, this could be related to an earlier diagnosis due to an easier patient access to cardiovascular, especially echocardiographic, screening in Greece. 5 An observational study by Xanthouli et al 11 in 90 patients with a “typical” PAH phenotype and 52 patients with PAH and cardiac or pulmonary comorbidities concluded that risk stratification based on ESC/ERS‐guidelines could only be confirmed in patients without comorbidities, but not in patients with PAH and comorbidities, suggesting that in patients with IPAH, comorbidities confound the overall clinical profile; therefore, conventional risk models may not adequately capture differences in prognosis. As a consequence, an adapted clinical score may be necessary to be applied in PAH patients with CVCs.…”

“…A strength of our study over previous studies is that we included only patients classified in the IPAH group to achieve greater homogeneity at the expense of a more limited sample size. 11 In addition, we added atrial fibrillation as a fifth factor that is associated with left heart disease, similarly to Rosenkranz et al 13 Due to the limited sample size, the multivariable model used up to four variables in the total cohort. Additionally, information regarding only the initial assessment at first visit is presented.…”

Real-World Analysis of Treatment Patterns Among Hospitalized Patients with Pulmonary Arterial Hypertension

“…In addition, while the REVEAL 2.0 risk algorithm does include hospitalization as a variable, other algorithms do not, and the proportion of clinicians who rely primarily on guidelines and non-REVEAL risk profiles is currently unknown. Comorbidity may also cause reluctance among clinicians to initiate combination therapy, and contraindications among concomitant medications as well as polypharmacy per se may also curtail treatment choices [29,35,36]. Thus, the considerable comorbidity we observed is notable, especially given that several of the observed specific PAH medications have contraindications for comorbidities prevalent in the sample (e.g., hypertension, renal dysfunction, liver disorders, chronic obstructive pulmonary disease) [37][38][39][40][41][42][43][44].…”

Prediction of Prognostic Hemodynamic Indices in Pulmonary Hypertension Using Non-Invasive Parameters

“…The planned study should also take into account the different prognostic significance of non-invasive parameters in PAH patients with and without comorbidities. The necessity to use different prognostic parameters in patients with PAH and cardio-pulmonary comorbidities was demonstrated for the first time by Xanthouli et al in a recently published paper [38], and this issue was not known at the time of designing our study.…”