scholarly journals Clinical and Hemodynamic Characterization Super Responders to IV Prostacyclin

2020 ◽  
Author(s):  
Adrienne K Conger ◽  
Steven J Halliday ◽  
Meredith E Pugh ◽  
Ivan M Robbins ◽  
Anna R Hemnes
Keyword(s):  
Right Ventricular ◽  
Ventricular Function ◽  
Right Ventricular Function ◽  
Walk Distance ◽  
Free Survival ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
Minute Walk Distance ◽  
Minute Walk

Abstract BackgroundParenteral prostacyclins are the only therapy proven to extend survival in pulmonary arterial hypertension (PAH), yet at the bedside clinicians have no tools to predict which patients are most likely to benefit from this medication class. MethodsWe retrospectively analyzed all PAH patients treated with IV epoprostenol therapy at our center from 1/1/1996 to 12/31/2016. We analyzed survival in patients and defined the 90 th percentile of survival. Patients were divided into those who survived past this point (super responders) and those who had had an event prior to this time point after initiation of iv epoprostenol (usual responders).ResultsThe median survival after IV epoprostenol initiation was 4.32 years, and the 90 th percentile of event-free survival was 11.09 years. Fourteen patients met criteria for super responder and 45 had a survival <90 th percentile, comprising the usual responder group. Super responders tended to be younger, have longer six-minute walk distances and higher mean pulmonary arterial pressure (p<0.05 for all). In follow up, super responders continued to have a higher six-minute walk distance and were more likely to have achieved normal or only mildly impaired right ventricular function, though no differences in hemodynamics were observed.ConclusionsThere may be a super responder phenotype that can be defined in patients with PAH by >90 th percentile of survival. Super responders were more likely that usual responders to be younger and were more likely to have achieved favorable right ventricular function at follow up, however, differences in hemodynamics were not observed.

scholarly journals Clinical and Hemodynamic Characterization Super Responders to IV Prostacyclin in Pulmonary Arterial Hypertension

2021 ◽  
Author(s):  
Adrienne K Conger ◽  
Steven J Halliday ◽  
Meredith E Pugh ◽  
Ivan M Robbins ◽  
Anna R Hemnes
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Ventricular Function ◽  
Right Ventricular Function ◽  
Free Survival ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
Minute Walk

Abstract Background: Parenteral prostacyclins are the only therapy proven to extend survival in pulmonary arterial hypertension (PAH), yet at the bedside clinicians have no tools to predict which patients are most likely to benefit from this medication class. Methods: We retrospectively analyzed all PAH patients treated with IV epoprostenol therapy at our center from 1/1/1996 to 12/31/2016. We analyzed survival in patients and defined the 90 th percentile of survival. Patients were divided into those who survived past this point (super responders) and those who had had an event prior to this time point after initiation of iv epoprostenol (usual responders). Results: The median survival after IV epoprostenol initiation was 4.32 years, and the 90 th percentile of event-free survival was 11.09 years. Fourteen patients met criteria for super responder and 45 had a survival <90 th percentile, comprising the usual responder group. Super responders tended to be younger, have longer six-minute walk distances and higher mean pulmonary arterial pressure (p<0.05 for all). In follow up, super responders continued to have a higher six-minute walk distance and were more likely to have achieved normal or only mildly impaired right ventricular function, though no differences in hemodynamics were observed. Conclusions: There may be a super responder phenotype that can be defined in patients with PAH by >90 th percentile of survival. Super responders were more likely that usual responders to be younger and were more likely to have achieved favorable right ventricular function at follow up, however, differences in hemodynamics were not observed.

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 Early experience with selexipag for the treatment of adults with pulmonary arterial hypertension associated with congenital heart disease

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Van Dissel ◽  
M Post ◽  
G.T Sieswerda ◽  
H.W Vliegen ◽  
A.P.J Van Dijk ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Side Effects ◽  
Congenital Heart ◽  
Funding Source ◽  
Walk Distance ◽  
Titration Phase ◽  
Pulmonary Arterial ◽  
Minute Walk Distance ◽  
Minute Walk

Abstract Background Recently, selexipag, a new orally available and selective prostacyclin receptor agonist, has become available for treatment of pulmonary arterial hypertension (PAH), but experience in patients with PAH associated with congenital heart disease (CHD) is limited to patients with closed defects. Purpose We present our early multi-centre experience using selexipag in the heterogeneous PAH-CHD population. Methods We prospectively evaluated adults with PAH-CHD from five PAH-CHD expert centres who were treated with selexipag. Patients were titrated to highest tolerable individualized dose (200 to 1,600 μg twice daily), after which patients entered the maintenance phase. Data on functional class (FC), 6-minute walk distance (6MWD), imaging and biochemical (N terminal pro-brain natriuretic peptide [NT-proBNP]) parameters were collected. Results Thirty-four patients (age 43±14 years, 56% female, 60% Eisenmenger syndrome, 22% Down syndrome, 60% dual PAH therapy) were started on selexipag. All patients experienced at least 2 side effects during the initial uptitration phase. Most side effects were manageable and diminished after reaching the maintenance dose, but eight patients discontinued treatment due to side effects during the titration phase. The most frequent side effects were consistent with the known side effects of prostacyclins, including headache, nausea, diarrhoea and jaw pain. Majority (68%) of patients reached lower maintenance doses of 200–600 μg. At 12 months, FC improved in three patients and remained unchanged in the others. 6-minute walk distance remained stable throughout follow-up (475 to 470 m; p=n.s.) in patients who remained on-treatment compared to patients who stopped selexipag (485 to 370 m). NT-proBNP levels remained stable in patients on-treatment (520 to 600 ng/L) but worsened in patients who stopped (700 to 1000 ng/L). One patient died during follow-up from end-stage heart failure. Conclusion There is a promising role for selexipag in the treatment of adults with PAH-CHD. However, based on our experience, the use is challenging due to complexity in dosing and side effect profiles, which limit patients' tolerability and acceptance during the titration phase. Funding Acknowledgement Type of funding source: Other. Main funding source(s): Actelion Pharmaceuticals

scholarly journals Right ventricular reverse remodeling assessed by echocardiography as a new goal-oriented treatment strategy in PAH patients

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
C Fauvel ◽  
O Raitiere ◽  
N Si-Belkacem ◽  
C Viacroze ◽  
E Artaud-Macari ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Cox Regression ◽  
Conditional Inference ◽  
Reverse Remodeling ◽  
Free Survival ◽  
Cox Regression Analysis ◽  
Mean Pulmonary Arterial Pressure ◽  
Conditional Inference Trees ◽  
Pulmonary Arterial

Abstract Background While in heart failure with reduced ejection fraction, left ventricular reverse remodeling assessed by transthoracic echocardiography (TTE) is associated with better prognosis, right ventricular reverse remodeling (RVRR) was less investigated in pulmonary arterial hypertension (PAH) Purpose We aimed to investigate whether RVVR assessed by echocardiography could help to stratify PAH patient's prognosis. Methods Between 2002 and 2019, all consecutive PAH patients were included, treated and followed in a single PAH center in accordance with the current ESC/ERS guidelines. In addition to regular risk stratification parameters, we measured several echocardiographic RV systolic function and size parameters, including tricuspid annular plane systolic excursion (TAPSE, mm) or RV-end diastolic area (cm2) from apical-4 chamber view both at baseline, 1-year of follow-up as well as their change. Primary composite outcome was three-year transplant-free survival and death from all cause from the 1-year evaluation. Conditional inference trees were used to determine which TTE parameters and cutoffs values were associated with primary outcome from hierarchy of multiple covariates in multivariable Cox regression analysis. Kaplan-Meier curves were then drawn and compared with log-rank test. Results 126 incident PAH patients were included (63% female, mean age 59±18 yo), mainly due to connectivite-tissue disease and idiopathic PAH (26% and 22% respectively). At baseline, mean pulmonary arterial pressure was 42 (33, 52) mmHg. At 1-y follow-up under pulmonary vasodilation therapy, NYHA (p&lt;0.01), NTproBNP (p&lt;0.01), mean pulmonary arterial pressure (p&lt;0.01) and cardiac index (p&lt;0.01) were significantly improved compared to baseline. Conditional inference trees showed that 1-year TAPSE gain &gt;1 mm and 1-year RV end-diastolic area decreased &gt;2 cm2 were associated with 3-year transplant-free survival in multivariable Cox regression analysis (HR=0.23, 95% CI [0.08–0.61] p=0,0035, HR=0.34, 95% CI [0.12–0.94], p=0.038). Simple score from 0 (absence of RVRR), 1 (partial RVRR) and 2 (complete RVRR), describing the number of TTE parameters reach at 1-year was then investigated. Patients with complete RVRR depicted better transplant-free survival than partial or absence of RVRR, log-rank p&lt;0.001 (figure). Conclusion Complete reverse remodeling from right ventricular size and function could represent a new goal-oriented treatment strategy in PAH patients. FUNDunding Acknowledgement Type of funding sources: None. RVRR survival curves

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