scholarly journals COMPERA 2.0: A refined 4-strata risk assessment model for pulmonary arterial hypertension

2021 ◽  
pp. 2102311
Author(s):  
Marius M. Hoeper ◽  
Christine Pausch ◽  
Karen M. Olsson ◽  
Doerte Huscher ◽  
David Pittrow ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Assessment Model ◽  
Walking Distance ◽  
Intermediate Risk ◽  
Pulmonary Arterial ◽  
Changes In Risk

BackgroundRisk stratification plays an essential role in the management of patients with pulmonary arterial hypertension (PAH). The current European guidelines propose a 3-strata model to categorise risk as low, intermediate, or high, based on the expected 1-year mortality. However, with this model, most patients are categorised as intermediate risk. We investigated a modified approach based on 4 risk categories with intermediate risk subdivided into intermediate-low and intermediate-high risk.MethodsWe analysed data from COMPERA, a European pulmonary hypertension registry, and calculated risk at diagnosis and first follow-up based on functional class (FC), 6 min walking distance (6 MWD) and serum levels of brain natriuretic peptide (BNP) or N-terminal fragment of pro-BNP (NT-proBNP), using refined cut-off values. Survival was assessed with Kaplan-Meier analyses, log-rank testing, and Cox proportional hazards models.ResultsData from 1,655 patients with PAH were analysed. Using the 3-strata model, most patients were classified as intermediate risk (76.0% at baseline and 63.9% at first follow-up). The refined 4-strata risk model yielded a more nuanced separation and predicted long-term survival, especially at follow-up assessment. Changes in risk from baseline to follow-up were observed in 31.1% of the patients with the 3-strata model and in 49.2% with the 4-strata model. These changes, including those between the intermediate-low and intermediate-high strata, were associated with changes in long-term mortality risk.ConclusionsModified risk stratification using a 4-strata model based on refined cut-off levels for FC, 6MWD and BNP/NT-proBNP was more sensitive to prognostically relevant changes in risk than the original 3-strata model.

scholarly journals Outcomes of Atrioseptostomy with Stenting in Patients with Pulmonary Arterial Hypertension from a Large Single-Institution Cohort

Diagnostics ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. 725 ◽  
Author(s):  
Sergey V. Gorbachevsky ◽  
Anton A. Shmalts ◽  
Gulomjon M. Dadabaev ◽  
Nasirullo A. Nishonov ◽  
Manolis G. Pursanov ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Brain Natriuretic Peptide ◽  
Natriuretic Peptide ◽  
Risk Group ◽  
Functional Class ◽  
Walking Distance ◽  
Intermediate Risk ◽  
Pulmonary Arterial

The aim of this study was to analyze results of stenting atrioseptostomy in patients with pulmonary arterial hypertension and a different level of risk for one-year mortality that is not well described. Patients that underwent atrioseptostomy with stenting were retrospectively divided in two groups: “intermediate” (n = 55) or “high” risk (n = 13), according to the 2015 ESC/ESR guideline. Results of atrioseptostomy were assessed during hospital period and at follow-up. Patients from “intermediate” risk group demonstrated lower mortality rate (10/55, vs. 6/13) during the course of the study period, as well as higher freedom from lung transplantation or Potts shunt. At discharge, patients of both groups presented improvement in functional class and mobility. Patients from “intermediate” risk group showed longer 6-min walking distance, and lower levels of brain natriuretic peptide. At the latest follow-up, stable position and full patency of stents with right-to-left or bidirectional shunt at atrial level and absence of syncope was confirmed in patients of both groups. Patients from the “intermediate” risk group demonstrated higher functional class, better performance of walking test, and lower levels of brain natriuretic peptide. Stenting atrioseptostomy reliably secured interatrial communication and improved clinical condition in patients with idiopathic pulmonary arterial hypertension. Mid-term results were better in “intermediate” risk group.

scholarly journals Assessment of the REPLACE study composite endpoint in riociguat-treated patients in the PATENT study

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402097312
Author(s):  
Gérald Simonneau ◽  
Hossein-Ardeschir Ghofrani ◽  
Paul A. Corris ◽  
Stephan Rosenkranz ◽  
Ekkehard Grünig ◽  
...  
Keyword(s):  
Relative Risk ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Clinical Improvement ◽  
Composite Endpoint ◽  
World Health ◽  
Walking Distance ◽  
Pulmonary Arterial ◽  
Clinical Worsening

The goal of treatment in patients with pulmonary arterial hypertension is to achieve a low risk status, indicating a favorable long-term outcome. The REPLACE study investigated the efficacy of switching to riociguat in patients with pulmonary arterial hypertension and an insufficient response to phosphodiesterase-5 inhibitors. In this post hoc analysis, we applied the REPLACE composite endpoint of clinical improvement to the placebo-controlled PATENT-1 study of riociguat in pulmonary arterial hypertension and its long-term extension, PATENT-2. Clinical improvement was defined as ≥2 of the following in patients who completed the study without clinical worsening: ≥10% or ≥30 m improvement in 6-minute walking distance; World Health Organization functional class I or II; ≥30% decrease in N-terminal prohormone of brain natriuretic peptide. At PATENT-1 Week 12, patients treated with riociguat were more likely to achieve the composite endpoint vs. placebo ( P < 0.0001), with similar results in pretreated ( P = 0.0189) and treatment-naïve ( P < 0.0001) patients. Achievement of the composite endpoint at Week 12 was associated with a 45% reduction in relative risk of death and a 19% reduction in relative risk of clinical worsening in PATENT-2. Overall, these data suggest that use of the REPLACE composite endpoint in patients with pulmonary arterial hypertension is a valid assessment of response to treatment.

scholarly journals Riociguat for the treatment of pulmonary arterial hypertension: a long-term extension study (PATENT-2)

2015 ◽  
Vol 45 (5) ◽  
pp. 1303-1313 ◽  
Author(s):  
Lewis J. Rubin ◽  
Nazzareno Galiè ◽  
Friedrich Grimminger ◽  
Ekkehard Grünig ◽  
Marc Humbert ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Primary Objective ◽  
Functional Class ◽  
World Health ◽  
Walking Distance ◽  
Open Label ◽  
Pulmonary Arterial

Riociguat is a soluble, guanylate cyclase stimulator, approved for pulmonary arterial hypertension. In the 12-week PATENT-1 study, riociguat was well tolerated and improved several clinically relevant end-points in patients with pulmonary arterial hypertension who were treatment naïve or had been pretreated with endothelin-receptor antagonists or prostanoids. The PATENT-2 open-label extension evaluated the long-term safety and efficacy of riociguat.Eligible patients from the PATENT-1 study received riociguat individually adjusted up to a maximum dose of 2.5 mg three times daily. The primary objective was to assess the safety and tolerability of riociguat; exploratory efficacy assessments included 6-min walking distance and World Health Organization (WHO) functional class.Overall, 396 patients entered the PATENT-2 study and 324 (82%) were ongoing at this interim analysis (March 2013). The safety profile of riociguat in PATENT-2 was similar to that observed in PATENT-1, with cases of haemoptysis and pulmonary haemorrhage also being observed in PATENT-2. Improvements in the patients', 6-min walking distance and WHO functional class observed in PATENT-1 persisted for up to 1 year in PATENT-2. In the observed population at the 1-year time point, mean±sd 6-min walking distance had changed by 51±74 m and WHO functional class had improved in 33%, stabilised in 61% and worsened in 6% of the patients versus the PATENT-1 baseline.Long-term riociguat was well tolerated in patients with pulmonary arterial hypertension, and led to sustained improvements in exercise capacity and functional capacity for up to 1 year.

LONG-TERM EFFICACY OF SITAXSENTAN IN PATIENTS WITH SEVERE PULMONARY ARTERIAL HYPERTENSION: A SIX-YEAR FOLLOW-UP

CHEST Journal ◽  
2009 ◽  
Vol 136 (4) ◽  
pp. 56S
Author(s):  
Maria-Dolores Hernandez de Diego ◽  
Fernan-Alejandra Ayala-Ugalde ◽  
Tomas Pulido-Zamudio ◽  
Luis-Efren Santos-Martinez ◽  
Hector Peña ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Term Efficacy ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

scholarly journals 150 Incremental value of cardiopulmonary exercise testing in intermediate-risk pulmonary arterial hypertension

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Roberto Badagliacca ◽  
Franz Rischard ◽  
Francesco Lo Giudice ◽  
Luke Howard ◽  
Silvia Papa ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Exercise Testing ◽  
Prognostic Model ◽  
Cardiopulmonary Exercise Testing ◽  
Intermediate Risk ◽  
Cardiopulmonary Exercise ◽  
Pulmonary Arterial

Abstract Aims Risk assessment in pulmonary arterial hypertension (PAH) is essential for prognostication. However, the majority of patients end-up in an intermediate risk status despite targeted-therapy, offering insufficient guidance in clinical practice. The added value of cardiopulmonary exercise testing (CPET) in this setting remains undefined. Methods and results Two independent cohorts with idiopathic PAH at intermediate risk were used to develop (n = 124) and externally validate (n = 143) the prognostic model. Risk assessment was based on the simplified version of the ESC/ERS guidelines score. The same definition of clinical worsening (CW) was used for both cohorts. Discrimination and calibration were assessed. Seventy-four derivation cohort patients experienced CW (51.2%) during a median of 34 months. Stroke volume index (SVI) and 6-min walk-distance (6MWD) were independent predictors of CW. With addition of CPET variables, SVI and VO2 peak independently improved the power of the prognostic model, determined by the integrated discrimination integral (IDI) index. ROC-derived cut-off values for SVI and VO2 peak were 34 and 14 ml/kg/min, respectively. Forty-eight validation cohort patients experienced CW (33.5%) during a median of 27 months follow-up. Different combinations of cut-off values of SVI and VO2 peak defined three meaningful groups showing good discrimination and calibration. The event-free survival rates at 1, 2, and 3 years were, respectively, 96%, 89%, and 89% for high SVI/high VO2 peak combination; 85%, 73%, and 61% for high SVI/low VO2 peak; and 80%, 70%, and 56% for low SVI/low VO2 peak. Conclusions Combinations of VO2 peak and SVI during follow-up is important in the prognostication of intermediate-risk prevalent patients with idiopathic PAH.

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