scholarly journals PULMONARY VASOREACTIVITY TESTING WITH ADENOSINE PREDICTS LONG TERM HEMODYNAMIC RESPONSE TO PROSTACYCLIN MONOTHERAPY, AND PROSTACYCLIN AND PHOSPHODIESTERASE-5 INHIBITOR COMBINATION THERAPY IN PULMONARY ARTERIAL HYPERTENSION

2018 ◽  
Vol 71 (11) ◽  
pp. A1951
Author(s):  
Ramsey Wehbe ◽  
Jonathan Rich ◽  
Stuart Rich
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Hemodynamic Response ◽  
Phosphodiesterase 5 Inhibitor ◽  
Pulmonary Arterial ◽  
Phosphodiesterase 5 ◽  
Inhibitor Combination

Temporal trends in pulmonary arterial hypertension: Results from the COMPERA registry

2021 ◽  
pp. 2102024
Author(s):  
Marius M. Hoeper ◽  
Christine Pausch ◽  
Ekkehard Grünig ◽  
Gerd Staehler ◽  
Doerte Huscher ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Temporal Trends ◽  
Survival Rates ◽  
Term Survival ◽  
Pulmonary Arterial ◽  
Early Combination Therapy

BackgroundSince 2015, the European pulmonary hypertension guidelines recommend the use of combination therapy in most patients with pulmonary arterial hypertension (PAH). However, it is unclear to what extend this treatment strategy is adopted in clinical practice and if it is associated with improved long-term survival.MethodsWe analysed data from COMPERA, a large European pulmonary hypertension registry, to assess temporal trends in the use of combination therapy and survival of patients with newly diagnosed PAH between 2010 and 2019. For survival analyses, we look at annualized data and at cumulated data comparing the periods 2010–2014 and 2015–2019.ResultsA total of 2,531 patients were included. The use of early combination therapy (within 3 months after diagnosis) increased from 10.0% in patients diagnosed with PAH in 2010 to 25.0% in patients diagnosed with PAH in 2019. The proportion of patients receiving combination therapy 1 year after diagnosis increased from 27.7% to 46.3%. When comparing the 2010–2014 and 2015–2019 periods, 1-year survival estimates were similar (89.0% [95% CI, 87.2%, 90.9%] and 90.8% [95% CI, 89.3%, 92.4%]), respectively, whereas there was a slight but non-significant improvement in 3-year survival estimates (67.8% [95% CI, 65.0%, 70.8%] and 70.5% [95% CI, 67.8%, 73.4%]), respectively.ConclusionsThe use of combination therapy increased from 2010 to 2019, but most patients still received monotherapy. Survival rates at 1 year after diagnosis did not change over time. Future studies need to determine if the observed trend suggesting improved 3-year survival rates can be confirmed.

scholarly journals PATENT PLUS: a blinded, randomised and extension study of riociguat plus sildenafil in pulmonary arterial hypertension

2015 ◽  
Vol 45 (5) ◽  
pp. 1314-1322 ◽  
Author(s):  
Nazzareno Galiè ◽  
Katharina Müller ◽  
Andrea-Viviana Scalise ◽  
Ekkehard Grünig
Keyword(s):  
Blood Pressure ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Primary Outcome ◽  
Study Drug ◽  
Maximum Change ◽  
Pulmonary Arterial ◽  
Phosphodiesterase 5 ◽  
Supine Systolic Blood Pressure

PATENT PLUS evaluated the safety and efficacy of riociguat in combination with sildenafil in pulmonary arterial hypertension patients.Patients receiving sildenafil (20 mg three times daily) were randomised to placebo or riociguat (up to 2.5 mg three times daily) for 12 weeks. The primary outcome was maximum change in supine systolic blood pressure (SBP) from baseline within 4 h of dosing. Secondary objectives comprised additional blood pressure, heart rate and exploratory efficacy variables, and safety. Patients could enter a long-term extension (LTE), where all patients received riociguat plus sildenafil.There was no difference in maximum change in supine SBP from baseline within 4 h between the riociguat (n=12) (mean±sd baseline: –20.2±15.3 mmHg; week 12: –20.7±18.0 mmHg) and placebo groups (n=6) (–7.6±3.9 and –20.2±12.9 mmHg, respectively). Changes in standing SBP and supine or standing diastolic blood pressure were also not different. Combination therapy showed no favourable effects on exploratory clinical parameters, including haemodynamics and exercise capacity. In the LTE, there were high rates of discontinuation due to hypotension and three (18%) deaths (not considered study drug-related by the investigator).There were potentially unfavourable safety signals with sildenafil plus riociguat and no evidence of a positive benefit/risk ratio. Concomitant use of riociguat with phosphodiesterase-5 inhibitors is therefore contraindicated.

scholarly journals High Right Ventricular Afterload during Exercise in Patients with Pulmonary Arterial Hypertension

2021 ◽  
Vol 10 (9) ◽  
pp. 2024
Author(s):  
Mari Nishizaki ◽  
Aiko Ogawa ◽  
Hiromi Matsubara
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Exercise Training ◽  
Arterial Hypertension ◽  
Hemodynamic Response ◽  
Pulmonary Vasculature ◽  
Pulmonary Hemodynamics ◽  
Pulmonary Hemodynamic ◽  
Pulmonary Arterial ◽  
Response To Exercise

The right ventricle (RV) is more sensitive to an increase in afterload than the left ventricle (LV), and RV afterload during exercise increases more easily than LV afterload. Pulmonary arterial hypertension (PAH)-specific therapy has improved pulmonary hemodynamics at rest; however, the pulmonary hemodynamic response to exercise is still abnormal in most patients with PAH. In these patients, RV afterload during exercise could be higher, resulting in a greater increase in RV wall stress. Recently, an increasing number of studies have indicated the short-term efficacy of exercise training. However, considering the potential risk of promoting myocardial maladaptive remodeling, even low-intensity repetitive exercise training could lead to long-term clinical deterioration. Further studies investigating the long-term effects on the RV and pulmonary vasculature are warranted. Although the indications for exercise training for patients with PAH have been expanding, exercise training may be associated with various risks. Training programs along with risk stratification based on the pulmonary hemodynamic response to exercise may enhance the safety of patients with PAH.

Mortality trends in pulmonary arterial hypertension in canada: a temporal analysis of survival per ESC/ERS Guideline Era

2021 ◽  
pp. 2101552
Author(s):  
Jason G.E. Zelt ◽  
Jordan Sugarman ◽  
Jason Weatherald ◽  
Arun C. R. Partridge ◽  
Jiaming (Calvin) Liang ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Temporal Analysis ◽  
Mortality Trends ◽  
European Respiratory Society ◽  
Term Survival ◽  
Long Term Survival ◽  
Pulmonary Arterial

IntroductionThe evolution in pulmonary arterial hypertension (PAH) management has been summarized in three iterations of the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. No study has assessed whether changes in management, as reflected in the changing guidelines, has translated to improved long-term survival in PAH.MethodsMixed retrospective/prospective analysis of treatment naïve, incident PAH patients (n=392) diagnosed at three major centers in Canada from 2009–2021. Patients were divided into two groups based on their diagnosis date and in accordance with three ESC/ERS guideline iterations: 2009 and 2015. Overall survival was assessed based on date of diagnosis and initial treatment strategy (i.e. mono versus combination).ResultsIn Canada, there was a shift towards more aggressive upfront management with combination therapy after the publication of the 2015 guidelines (10.4% and 30.8% in 2009–2015 patients, and 36.0% and 57.4% in 2016–2021 patients, for baseline and 2-year follow-up respectively). A key factor associated with combination therapy after 2015 was higher pulmonary vascular resistance (p=0.009). The 1, 3 and 5 year survival rates in Canada were 89.2%, 75.6%. and 56.0%, respectively. Despite changes in management, there was no improvement in long-term survival before and after publication of the 2015 ESC/ERS guideline (p=0.53).ConclusionsThere was an increase in the use of initial and sequential combination therapy in Canada after publication of the 2015 ESC/ERS guidelines, which was not associated with improved long-term survival. These data highlight the continued difficulties of managing this aggressive pulmonary disease in an era without a cure.

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