scholarly journals Determinants of pulmonary vascular resistance reduction with upfront oral therapy in idiopathic pulmonary arterial hypertension: relevance in risk assessment

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
R Badagliacca ◽  
M D'Alto ◽  
S Ghio ◽  
A Greco ◽  
S Papa ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
Treatment Response ◽  
Oral Therapy ◽  
Low Risk ◽  
Risk Scores ◽  
Intermediate Risk ◽  
Risk Status ◽  
Pulmonary Arterial ◽  
Response Score

Abstract Background In pulmonary arterial hypertension (PAH) upfront oral therapy represents the standard of care for naive patients at low and intermediate risk. However little is known about associated changes in risk assessment and prediction of low risk status achievement. Purpose To evaluate determinants of PVR reduction in patients treated with upfront oral therapy and to create a score to predict PVR reduction after upfront oral treatment and compared its additive value on top of the European and REVEAL scoring system in predicting treatment response. Methods One-hundred-eighty-one consecutive naive PAH patients treated with upfront therapy at 11 italian centers were retrospectively evaluated. Evaluation included clinical, hemodynamic and simple echocardiographic parameters, together with European and REVEAL 2.0 risk scores. Results At the time of diagnosis, the majority of the patients was idiopathic PAH (80.6%), female (66.3%), at intermediate risk, 71.8% and 55.2%, respectively, according to the European (average method) and the REVEAL 2.0 risk scores. Ambrisentan-Tadalafil was the most frequent combination used (62%). The median PVR reduction obtained after 180 days (IQR 79–394) was −40.4% (IQR −25.8; −45.3). Age ≥60 years, male-sex, baseline mPAP 48 mmHg associated with low CI (<2.5 l/min/m2), and RV/LV ratio >1 associated with low TAPSE (<18 mm) emerged as independent predictors of poor PVR reduction, defined as the lower tertile of PVR changes (−25.8%). A treatment response score was created deriving weighted integers from the beta coefficient. At second evaluation 78 (43.1%) patients achieved or remained at European-derived low risk status, while 63 (34.8%) considering the REVEAL 2.0 score. Multivariate analysis for the prediction of treatment failure, defined as the absence of low-risk status at follow-up, demonstrated the incremental prognostic power of the models incorporating the treatment response score (≥3) on top of the European and REVEAL 2.0 scores, improving risk discrimination by 63.2% (IDI index 0.056) and 36.8% (IDI index 0.080), respectively. Conclusions A significant proportion of PAH patients treated with upfront oral combination are not able to achieve a low-risk status. The treatment response score helps clinicians in predicting treatment failure at the time of diagnosis. Funding Acknowledgement Type of funding source: None

Single-center prognostic validation of the risk assessment of the 2015 ESC/ERS guidelines in patients with pulmonary arterial hypertension in Japan

2020 ◽  
Vol 98 (9) ◽  
pp. 653-658 ◽  
Author(s):  
Ryo Imai ◽  
Shiro Adachi ◽  
Masahiro Yoshida ◽  
Shigetake Shimokata ◽  
Yoshihisa Nakano ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Risk Group ◽  
Low Risk ◽  
World Health ◽  
Right Atrial ◽  
Walking Distance ◽  
Pulmonary Arterial

The 2015 European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension include a multidimensional risk assessment for patients with pulmonary arterial hypertension (PAH). However, prognostic validations of this risk assessment are limited, especially outside Europe. Here, we validated the risk assessment strategy in PAH patients in our institution in Japan. Eighty consecutive PAH patients who underwent right heart catheterization between November 2006 and December 2018 were analyzed. Patients were classified as low, intermediate, or high risk by using a simplified version of the risk assessment that included seven variables: World Health Organization functional class, 6-min walking distance, peak oxygen consumption, brain natriuretic peptide, right atrial pressure, mixed venous oxygen saturation, and cardiac index. The high-risk group showed significantly higher mortality than the low- or intermediate-risk group at baseline (P < 0.001 for both comparisons), and the mortalities in the intermediate- and low-risk groups were both low (P = 0.989). At follow-up, patients who improved to or maintained a low-risk status showed better survival than those who did not (P = 0.041). Our data suggest that this risk assessment can predict higher mortality risk and long-term survival in PAH patients in Japan.

scholarly journals 121 Imaging of improved right ventricular function and risk status in pulmonary arterial hypertension

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Cristiano Miotti ◽  
Roberto Badagliacca ◽  
Carmine Dario Vizza
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Ventricular Function ◽  
Targeted Therapies ◽  
Right Ventricular Function ◽  
Low Risk ◽  
Risk Status ◽  
Pulmonary Arterial

Abstract Aims Right ventricular function adaptation to afterload as assessed by the ratio of tricuspid annular plane excursion (TAPSE) to systolic pulmonary artery pressure (PASP) has been shown to be of prognostic relevance in pulmonary arterial hypertension (PAH). To test the hypothesis that improvement in TAPSE/PASP under targeted therapies is associated with the likelihood to achieve a better prognostic low-risk status in PAH. Methods and results The study retrospectively enrolled 677 PAH patients (55% idiopathic) with follow-up clinical, right heart catheterization, and echocardiographic evaluations within 12 months [interquartile range (IQR): 180–344 days] after initiation of targeted therapies from 2005 to 2017 in 11 Italian centres. European guidelines-derived and United States Registry to Evaluate Early and Long-Term PAH Disease Management registry REVEAL 2.0 risk scores were assessed at baseline and follow-up. The patients improved their functional class and 6-min walk distance, but a minority of them achieved or maintained the low risk status as assessed either with the European or the REVEAL 2.0 score (30% and 27%, respectively). The TAPSE/PASP ratio increased curvilinearly in proportion to decreased pulmonary vascular resistance (PVR) by more than 50%. Patients at low risk were, respectively, 4.93 and 3.37 times more likely to have TAPSE/PASP ≥0.35 mm/mmHg than those at intermediate or high risk, according to the ESC/ERS [odds ratio: (OR) 4.93, confidence interval (CI): 3.40–7.14; P = 0.0001] and the REVEAL 2.0 score (OR: 3.37, CI: 2.32–4.90; P = 0.0001). Conclusions Improvement of TAPSE/PASP under targeted therapies in PAH is associated with a low risk status, marked reduction in PVR, and improved outcome.

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.

Kombinationstherapie mit Ambrisentan und Tadalafil bei CTD-PAH: viel hilft viel?

2021 ◽  
pp. 1-2
Author(s):  
Panagiota Xanthouli
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Proportional Hazards ◽  
Risk Group ◽  
Cox Proportional Hazards ◽  
Low Risk ◽  
Intermediate Risk ◽  
Clinical Failure ◽  
Pulmonary Arterial ◽  
Baseline Characteristics

<b>Objectives:</b> To evaluate initial combination therapy with ambrisentan plus tadalafil (COMB) compared with monotherapy of either agent (MONO), and the utility of baseline characteristics and risk stratification in predicting outcomes, in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) and the systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) subpopulation. <b>Methods:</b> This post hoc analysis of the Ambrisentan and Tadalafil in Patients with Pulmonary Arterial Hypertension (AMBITION) study included patients with CTD-PAH from the modified intention-to-treat population. Time to clinical failure (TtCF) was assessed by baseline characteristics, treatment assignment and risk group (low, intermediate and high) at baseline and week 16. TtCF was compared between groups using Kaplan-Meier curves and Cox proportional hazards regression modelling. <b>Results:</b> The analysis included 216 patients (COMB, <i>n</i> = 117; MONO, <i>n</i> = 99). The risk of clinical failure was lower with COMB versus MONO (risk reduction: CTD-PAH 51.7%, SSc-PAH 53.7%), particularly in patients with haemodynamic parameters characteristic of typical PAH without features of left heart disease and/or restrictive lung disease at baseline. The risk of clinical failure was lower with COMB versus MONO in the baseline low-risk group (HR not calculated due to no events in COMB), baseline intermediate-risk group (HR 0.519, 95% CI 0.297 to 0.905) and in the week 16 low-risk group (HR 0.069, 95% CI 0.009 to 0.548). <b>Conclusions:</b> The benefit of COMB over MONO was demonstrated in patients with CTD-PAH, particularly in those with typical PAH haemodynamic characteristics at baseline. COMB is appropriate for patients categorised as low risk and intermediate risk at baseline and low risk at follow-up. <b>Trial registration number:</b> NCT01178073.

scholarly journals Risk assessment in patients with functional class II pulmonary arterial hypertension: Comparison of physician gestalt with ESC/ERS and the REVEAL 2.0 risk score

PLoS ONE ◽  
2020 ◽  
Vol 15 (11) ◽  
pp. e0241504
Author(s):  
Sandeep Sahay ◽  
Adriano R. Tonelli ◽  
Mona Selej ◽  
Zachary Watson ◽  
Raymond L. Benza
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Low Risk ◽  
Assessment Tools ◽  
Patient Risk ◽  
Objective Risk ◽  
Pulmonary Arterial

Background Accurate and regular risk assessment is important for evaluation and treatment of pulmonary arterial hypertension (PAH) patients, including those with functional class (FC) II symptoms, a population considered at low risk for disease progression. Risk assessment methods include subjective and objective evaluations. Multiparametric assessments include tools based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines (COMPERA and FPHR methods, respectively) and the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL; REVEAL 2.0 tool). To better understand risk status determination in FC II patients, we compared physician-reported risk assessments with objective multiparameter assessment tools. Methods This retrospective chart analysis included PAH patients with FC II symptoms receiving monotherapy or dual therapy. Physicians were surveyed (via telephone) to obtain an assessment of patient risk using their typical methodology, which might have been informed by objective risk assessment. Patient risk was then calculated independently using COMPERA, FPHR and REVEAL 2.0 tools. Factors associated with incongruent risk assessment were identified. Results Of the 153 patients, 41%, 46%, and 13% were classified as low, intermediate, and high risk, respectively, by physicians. Concordance between physician gestalt and objective methods ranged from 43%–54%. Among patients considered as low risk by physician gestalt, 4%–28% were categorized as high risk using objective methods. The most common physician factor associated with incongruent risk assessment was less frequent echocardiography during follow-up (every 7–12 months vs. every 3 months; p = 0.01). Conclusions More than half of FC II PAH patients were classified as intermediate/high risk using objective multiparameter assessments. Incorporating objective risk-assessment algorithms into clinical practice may better inform risk assessment and treatment strategies.

scholarly journals A pragmatic approach to risk assessment in pulmonary arterial hypertension using the 2015 European Society of Cardiology/European Respiratory Society guidelines

Open Heart ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. e001725
Author(s):  
Fabio Dardi ◽  
Alessandra Manes ◽  
Daniele Guarino ◽  
Elisa Zuffa ◽  
Alessandro De Lorenzis ◽  
...  
Keyword(s):  
Risk Assessment ◽  
High Risk ◽  
Risk Groups ◽  
Low Risk ◽  
Intermediate Risk ◽  
Pragmatic Approach ◽  
European Respiratory Society ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology

ObjectiveTo optimise treatment of patients with pulmonary arterial hypertension (PAH), the 2015 European Society of Cardiology/European Respiratory Society guidelines recommend using risk stratification, with the aim of patients achieving low-risk status. Previous analyses of registries made progress in using risk stratification approaches, however, the focus is often on patients with a low-risk prognosis, whereas most PAH patients are in intermediate-risk or high-risk categories. Using only six parameters with high prognostic relevance, we aimed to demonstrate a pragmatic approach to individual patient risk assessment to discriminate between patients at low risk, intermediate risk and high risk of death.MethodsRisk assessment was performed combining six parameters in four criteria: (1) WHO functional class, (2) 6 min walk distance, (3) N-terminal pro-brain natriuretic peptide (BNP)/BNP plasma levels or right atrial pressure and (4) cardiac index or mixed venous oxygen saturation. Assessments were made at baseline and at first follow-up after 3–4 months.Results725 PAH treatment-naive patients were analysed. Survival estimates between risk groups were statistically significant at baseline and first follow-up (p<0.001), even when the analysis was performed within PAH etiological subgroups. Similar results were observed in 208 previously treated PAH patients. Furthermore, patients who remained at or improved to low risk had a significantly better estimated survival compared with patients who remained at or worsened to intermediate risk or high risk (p≤0.005).ConclusionThe simplified risk-assessment method can discriminate idiopathic, connective-tissue-disease-associated and congenital-heart-disease-associated PAH patients into meaningful high-risk, intermediate-risk and low-risk groups at baseline and first follow-up. This pragmatic approach reinforces targeting a low-risk profile for PAH patients.

P1803Refining the 2015 European guidelines risk assessment tool for pulmonary arterial hypertension in adult congenital heart disease

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A C Van Dissel ◽  
A P J Van Dijk ◽  
A L Duijnhouwer ◽  
B J M Mulder ◽  
B J Bouma
Keyword(s):  
Risk Assessment ◽  
High Risk ◽  
Congenital Heart ◽  
Assessment Tool ◽  
Risk Groups ◽  
Low Risk ◽  
Risk Assessment Tool ◽  
Intermediate Risk ◽  
European Guidelines ◽  
Pulmonary Arterial

Abstract Background Current European guidelines advocate a goal-oriented treatment approach in pulmonary arterial hypertension (PAH), based on a comprehensive risk assessment. However, this instrument has been based predominantly on patients with idiopathic PAH and its accuracy has not been well established for PAH associated with congenital heart disease (CHD)–a patient population known to be distinctly different for other PAH aetiologies. Purpose To investigate the discriminatory ability of the guidelines risk assessment tool and explore the benefit of including other cut-offs or variables in PAH-CHD. Methods and results Data from 112 PAH-CHD patients (age 42.1±16 years, 70% Eisenmenger, 38% Down syndrome) seen between 2004 and 2016 at two specialized adult PAH-CHD expert centres were prospectively collected. Patients were classified as “Low”, “Intermediate”, or “High” risk following the strategy proposed by Kylhammar (Eur Heart J, 2017) based on N-terminal pro-brain natriuretic peptide (NT-proBNP), 6-minute walk distance, functional class and imaging parameters and analysed by Kaplan-Meier method, truncated at 5 years. At baseline, 25% (28) of patients were classified as “Low risk”, 69% (77) as “Intermediate risk” and 6% (7) as “High risk”. Although survival was better (P=0.012) for patients with higher proportions of “Low risk” variables, this method did not discriminate well between the three risk groups (Figure 1A, P=0.371). One-year survival estimates corresponded moderately to those proposed by the guidelines, 96.4% in the “Low risk” (vs. >95%), 94.8% in the “Intermediate risk” (vs. 90–95%), and 85.7% in the “High risk” (vs. <90%) baseline cohorts, respectively. Analysis of different cut-off values for NT-proBNP (i.e., “Low”, “Intermediate”, “High” as <500, 500–1440 and >1400 ng/l, respectively) and use of tricuspid annular plane systolic excursion (TAPSE) measurements (“Low”, “Intermediate”, “High” as <1.6, 2.6–2.7 and >2.7 cm, respectively) as imaging parameter instead of right atrial area improved discrimination between the risk groups (Figure 1B). With these adjustments to the risk assessment tool, survival differed between all three risk groups (P<0.001). Figure 1 Conclusion Our preliminary findings suggest that an updated version of the European guidelines risk assessment tool–with different cut-off values for NT-proBNP and use of TAPSE–discriminates more accurately in the PAH-CHD population. Further analysis will be performed to estimate the prognostic benefit of reaching a “Low risk” profile, as this is the recommended treatment goal.

Risk assessment and treatment goals in patients presenting with pulmonary arterial hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2560-2562
Author(s):  
Marius M. Hoeper
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Right Ventricular Function ◽  
Functional Class ◽  
Low Risk ◽  
World Health ◽  
Pulmonary Arterial ◽  
Health Organization

Risk stratification in patients with pulmonary arterial hypertension is based on a multidimensional approach that utilizes clinical features, World Health Organization functional class, exercise capacity, biomarkers, and evaluation of right ventricular function by imaging and/or invasive measurements. Risk assessment at the time of diagnosis determines the initial treatment strategy, while risk assessment during follow-up is performed to identify disease progression and the need for treatment modifications. A low-risk category is characterized by non-progressive disease, absence of signs of right heart failure and syncope, World Health Organization functional class I or II with normal or near normal exercise capacity, normal or near normal plasma levels of B-type natriuretic peptide, and well-preserved right ventricular function as determined by echocardiography, cardiovascular magnetic resonance imaging, or right heart catheterization. Achieving and maintaining a low-risk status is a major objective in the treatment of patients with pulmonary arterial hypertension.

scholarly journals Risk assessment in pulmonary arterial hypertension

2016 ◽  
Vol 25 (142) ◽  
pp. 390-398 ◽  
Author(s):  
Amresh Raina ◽  
Marc Humbert
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Assessment Tools ◽  
Response To Treatment ◽  
Risk Scores ◽  
Patient Assessment ◽  
Pulmonary Arterial ◽  
Multiple Variables

Regular patient assessment is essential for the management of chronic diseases, such as pulmonary arterial hypertension (PAH). Comprehensive patient assessment and risk stratification in PAH are important to guide treatment decisions and to monitor disease progression as well as patients' response to treatment. Approaches for assessing risk in PAH patients include the use of risk variables, as recommended in the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines, and the application of risk equations and scores, such as the French registry risk equation and the REVEAL registry risk score. Risk stratification and risk scores are both useful predictors of survival on a population basis, and provide an estimate for individual patients' risk. The 2015 ESC/ERS PH guidelines recommend regular assessment of multiple variables at an expert centre. The respective merits and limitations of different risk assessment methods in PAH are discussed in this article, as well as some considerations that can be taken into account in the future development of risk assessment tools.

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