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

2021 ◽  
Vol 3 (1) ◽  
pp. 10-11
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, n = 117; MONO, n = 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 Initial combination therapy of ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) in the modified intention-to-treat population of the AMBITION study: post hoc analysis

2020 ◽  
Vol 79 (5) ◽  
pp. 626-634 ◽  
Author(s):  
Masataka Kuwana ◽  
Christiana Blair ◽  
Tomohiko Takahashi ◽  
Jonathan Langley ◽  
John G Coghlan
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Risk Group ◽  
Low Risk ◽  
Clinical Failure ◽  
Intention To Treat ◽  
Initial Combination Therapy ◽  
Pulmonary Arterial ◽  
Post Hoc ◽  
Initial Combination

ObjectivesTo 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.MethodsThis 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.ResultsThe analysis included 216 patients (COMB, n=117; MONO, n=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).ConclusionsThe 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.Trial registration numberNCT01178073.

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 Increased Levels of Runt-Related Transcription Factor 2 Are Associated With Poor Survival of Patients With Idiopathic Pulmonary Arterial Hypertension

2020 ◽  
Vol 14 (4) ◽  
pp. 155798832094545
Author(s):  
Xuntao Yuan ◽  
Zuogang Wang ◽  
Lan Wang ◽  
Qinhua Zhao ◽  
Sugang Gong ◽  
...  
Keyword(s):  
Transcription Factor ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Proportional Hazards ◽  
Independent Predictor ◽  
Cox Proportional Hazards ◽  
Clinical Severity ◽  
Related Transcription Factor ◽  
Pulmonary Arterial ◽  
Circulating Levels

Runt-related transcription factor 2 (RUNX2) plays a pivotal role in the pathogenesis of pulmonary arterial hypertension (PAH); yet, whether circulating levels of RUNX2 can predict survival of patients with idiopathic PAH (IPAH) is still unclear. The present study aimed to investigate the correlation between circulating levels of RUNX2 and survival of patients with IPAH. Blood samples were collected from 46 incident patients with IPAH and 30 healthy controls in Shanghai Pulmonary Hospital. Levels of RUNX2 were measured using ELISA. Linear regression and cox proportional hazards analysis were performed to assess the prognostic value of RUNX2 levels in predicting survival using the Kaplan–Meier method. Nonsurvivors had significantly shorter 6MWD, higher levels of NT-proBNP, increased mRAP, mPAP, mPAWP, PVR, and decreased CO as well as CI, compared with survivors ( p < .05). Plasma levels of RUNX2 were significantly higher in nonsurvival and survival patients with IPAH compared with controls ( p ≤ .001), and higher in nonsurvivors than in survivors ( p = .001). RUNX2 levels served as an independent predictor of survival in these patients ( p < .001). RUNX2 levels ≥41.5 ng/ml had a sensitivity of 80.0% and a specificity of 74.2% by ROC analysis. Patients with a RUNX2 level <41.5 ng/ml and/or mRAP <3.5 mmHg had a significantly better prognosis than those with a higher RUNX2 level in all subjects as well as in male or female patients ( p < .05). The level of circulating RUNX2 is an independent predictor for survival and it is correlated with the clinical severity of IPAH.

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 Warfarin in Systemic Sclerosis-associated and Idiopathic Pulmonary Arterial Hypertension. A Bayesian Approach to Evaluating Treatment for Uncommon Disease

2012 ◽  
Vol 39 (2) ◽  
pp. 276-285 ◽  
Author(s):  
SINDHU R. JOHNSON ◽  
JOHN T. GRANTON ◽  
GEORGE A. TOMLINSON ◽  
HADDAS A. GROSBEIN ◽  
THAOLAN LE ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Proportional Hazards ◽  
Credible Interval ◽  
Cox Proportional Hazards ◽  
Expert Elicitation ◽  
Matched Cohort ◽  
Posterior Median ◽  
Pulmonary Arterial

Objective.Warfarin is recommended in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) and idiopathic PAH (IPAH) to improve survival. There is no evidence to support this in SSc-PAH and the evidence in IPAH is conflicting. We evaluated the ability of warfarin to improve survival using 2 large SSc-PAH and IPAH cohorts.Methods.The effect of warfarin on all-cause mortality was evaluated. Bayesian propensity scores (PS) were used to adjust for baseline differences between patients exposed and not exposed to warfarin, and to assemble a matched cohort. Bayesian Cox proportional hazards models were constructed using informative priors based on international PAH expert elicitation.Results.Review of 1138 charts identified 275 patients with SSc-PAH (n = 78; 28% treated with warfarin) and 155 patients with IPAH (n = 91; 59% treated with warfarin). Baseline differences in PAH severity and medications were resolved using PS matching. In the matched cohort of 98 patients with SSc-PAH (49 treated with warfarin), the posterior median hazard ratio (HR) was 1.06 [95% credible interval (CrI) 0.70, 1.63]. In the matched cohort of 66 patients with IPAH (33 treated with warfarin), the posterior median HR was 1.07 (95% CrI 0.57, 1.98). The probability that warfarin improves median survival by 6 months or more is 23.5% in SSc-PAH and 27.7% in IPAH. Conversely, there is a > 70% probability that warfarin provides no significant benefit or is harmful.Conclusion.There is a low probability that warfarin improves survival in SSc-PAH and IPAH. Given the availability of other PAH therapies with demonstrable benefits, there is little reason to use warfarin to improve survival for these patients.

scholarly journals Change in R wave in lead V1 predicts survival of patients with pulmonary arterial hypertension

2018 ◽  
Vol 8 (2) ◽  
pp. 204589401877649 ◽  
Author(s):  
Shinji Sato ◽  
Aiko Ogawa ◽  
Hiromi Matsubara
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Rank Test ◽  
Characteristic Analysis ◽  
Kaplan Meier ◽  
Pulmonary Arterial ◽  
Analysis Survival ◽  
Treatment Escalation

Clinical guidelines for pulmonary hypertension recommend evaluating treatment response through various methods; however, electrocardiography (ECG) is not included as one of the methods of choice. We aimed to identify ECG parameters that correlated with prognosis in patients with pulmonary arterial hypertension (PAH). A total of 112 consecutive patients with PAH were enrolled in this study. Among them, 83 with treatment escalation were studied for further analysis. Survival analyses were conducted using the Kaplan–Meier method with the log-rank test. Cox proportional hazards regression modeling was used to identify predictors of survival. Receiver operating characteristic analysis was used to determine cut-off values for selected variables. ECG parameters were changed from baseline to three months after treatment. Patients in whom the R wave amplitude in lead V1 decreased by ≥1 mm (0.1 mV) within three months demonstrated significantly better survival ( P = 0.017). Our results suggest that evaluation of ECG parameters can contribute to assessments of survival in patients with PAH.

scholarly journals Effect of riociguat on pulmonary arterial compliance in the PATENT and CHEST studies

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402096383
Author(s):  
Thenappan Thenappan ◽  
Nadine Al-Naamani ◽  
Stefano Ghio ◽  
Hossein-Ardeschir Ghofrani ◽  
Paul M. Hassoun ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Proportional Hazards ◽  
Arterial Compliance ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Cox Proportional Hazards ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Pulmonary arterial compliance is a measure of the pulsatile afterload of the right ventricle. Lower pulmonary arterial compliance is associated with reduced right ventricular function and worse prognosis in pulmonary hypertension. The effect of pulmonary vasodilators on pulmonary arterial compliance has not been evaluated in detail in pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. In this post hoc analysis of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension in the PATENT and CHEST studies, we evaluated the change in pulmonary arterial compliance with riociguat versus placebo. Association of pulmonary arterial compliance with clinical outcomes was assessed using Kaplan–Meier and Cox proportional hazards analyses. Compared with placebo, riociguat significantly improved pulmonary arterial compliance in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Pulmonary arterial compliance at baseline was associated with survival and clinical worsening-free survival in pulmonary arterial hypertension but only with clinical worsening-free survival in chronic thromboembolic pulmonary hypertension. In patients with pulmonary arterial hypertension, pulmonary arterial compliance at follow-up ≥1.6 mL/mmHg was associated with better outcomes than pulmonary arterial compliance <1.6 mL/mmHg. In patients with chronic thromboembolic pulmonary hypertension, pulmonary arterial compliance at follow-up did not predict outcomes. Cox proportional hazards analyses showed no association between change in pulmonary arterial compliance and outcomes in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. In conclusion, riociguat improved pulmonary arterial compliance in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Pulmonary arterial compliance at baseline or follow-up, rather than change in pulmonary arterial compliance, is of prognostic importance for outcomes.

Abstract 16357: Application of the H2FPEF-score in Pulmonary Arterial Hypertension: Insights From the Amsterdam UMC PAH-cohort

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Azar Kianzad ◽  
Frances S De Man ◽  
Lucas R Celant ◽  
anton vonk noordegraaf ◽  
M M Handoko ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Proportional Hazards ◽  
Venous Blood ◽  
Right Heart Catheterization ◽  
Cox Proportional Hazards ◽  
Heart Catheterization ◽  
P Value ◽  
Mixed Venous Blood ◽  
Pulmonary Arterial

Introduction: Newly diagnosed idiopathic pulmonary arterial hypertension (PAH) patients increasingly have features of patients with heart failure with preserved ejection fraction (HFpEF). These so-called “atypical” PAH patients can be identified using the H2FPEF-score (Reddy, Circulation 2018). This patient group is often excluded from clinical trials, and it is therefore unclear whether they respond similarly to PAH-specific medication. Aim: To assess baseline characteristics and treatment response of patients diagnosed with PAH stratified by the H2FPEF-score. Methods: A retrospective analysis of 253 treatment naïve PAH-patients who underwent diagnostic right heart catheterization at baseline. Groups were compared with ANOVA, post-hoc pairwise t-tests with Bonferroni correction and multivariate Cox proportional hazards analysis. Results: This cohort included 58 (23%) patients with a low (≤1), 159 (63%) patients with an intermediate (2-5) and 36 (14%) patients with a high (≥6) H2FPEF-score. Patients with a high H2FPEF-score were older and more often male, used more diuretics, had more comorbidities and had more often PAH with severely impaired diffusion capacity compared to patients with a low H2FPEF-score. In addition, patients with a high H2FPEF-score had a significantly lower cardiac index, mixed venous blood gas, oxygen saturation and 6-minute walk distance (6MWD) compared to patients with a low H2FPEF-score. A high H2FPEF-score was associated with worse survival despite correcting for confounders (HR 2.99, 95% CI 1.53-5.84, Fig.1 A). Despite potential survival bias, at 1-year follow-up 6MWD (Fig.1 B) significantly improved in patients with a low and intermediate H2FPEF-score, but did not change in patients with a high H2FPEF-score (interaction p-value< 0.001). Conclusions: A high H2FPEF-score is associated with worse survival in PAH. Also, no improvement in 6MWD was observed in patients with a high H2FPEF-score only.

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 (&lt;2.5 l/min/m2), and RV/LV ratio &gt;1 associated with low TAPSE (&lt;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

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