Predictors of long-term outcomes in patients treated with riociguat for chronic thromboembolic pulmonary hypertension: data from the CHEST-2 open-label, randomised, long-term extension trial

2016 ◽  
Vol 4 (5) ◽  
pp. 372-380 ◽  
Author(s):  
Gérald Simonneau ◽  
Andrea M D'Armini ◽  
Hossein-Ardeschir Ghofrani ◽  
Friedrich Grimminger ◽  
Pavel Jansa ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Long Term Outcomes ◽  
Open Label ◽  
Thromboembolic Pulmonary Hypertension ◽  
Extension Trial

scholarly journals Long-term outcomes after percutaneous transluminal pulmonary angioplasty for chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 16 (4) ◽  
pp. 27-32 ◽  
Author(s):  
Madina B Karabasheva ◽  
Nikolai M Danilov ◽  
Olesia V Sagaidak ◽  
Yurii G Matchin ◽  
Irina E Chazova
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Treatment Method ◽  
Classical Type ◽  
Chronic Obstructive ◽  
Clinical Effects ◽  
Long Term Outcomes ◽  
Thromboembolic Pulmonary Hypertension

Introduction. Chronic thromboembolic pulmonary hypertension is a precapillary form of pulmonary hypertension that develops due to thrombotic mass obstruction of the pulmonary arteries. Balloon pulmonary angioplasty (BPA) is a new, alternative treatment method for inoperable chronic thromboembolic pulmonary hypertension (CTEPH), which demonstrated good hemodynamic and clinical effects. In this article we studied the long-term outcomes results after BPA. Material and methods. The study included 22 patients with inoperable CTEPH who were treated by BPA 6 (5; 8) interventions per patient. The results of the procedure were evaluated 2 months and 18 (12; 18) months after the last BPA. Results. A significant decrease in all important hemodynamic parameters was noted. There no difference between the date immediately after the operation and the long term period. However, the division of patients into groups with and without disease progression allow us to identify factors affecting the effectiveness of BPA (weight, the number of BPA procedures per patient, the total number of treated segmental arteries, the presence of coronary heart disease and chronic obstructive pulmonary disease, not the appointment of a concomitant pathogenetic pulmonary arterial hypertension (PAH) therapy. Conclusion. BPA is an effective method of treating patients with inoperable CTEPH, which demonstrates a good, sustainable long-term result. Patients with classical type 4 pulmonary hypertension receiving PAH-specific therapy are best responders to BPA.

scholarly journals Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2)

2014 ◽  
Vol 45 (5) ◽  
pp. 1293-1302 ◽  
Author(s):  
Gérald Simonneau ◽  
Andrea M. D’Armini ◽  
Hossein-Ardeschir Ghofrani ◽  
Friedrich Grimminger ◽  
Marius M. Hoeper ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Risk Profile ◽  
World Health ◽  
Walking Distance ◽  
Open Label ◽  
End Points ◽  
Thromboembolic Pulmonary Hypertension

Riociguat is a soluble guanylate cyclase stimulator approved for the treatment of inoperable and persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH). In the 16-week CHEST-1 study, riociguat showed a favourable benefit–risk profile and improved several clinically relevant end-points in patients with CTEPH. The CHEST-2 open-label extension evaluated the long-term safety and efficacy of riociguat.Eligible patients from CHEST-1 received riociguat individually adjusted up to a maximum dose of 2.5 mg three times daily. The primary objective was the safety and tolerability of riociguat; exploratory efficacy end-points included 6-min walking distance (6MWD) and World Health Organization (WHO) functional class (FC).Overall, 237 patients entered CHEST-2 and 211 (89%) were ongoing at this interim analysis (March 2013). The safety profile of riociguat in CHEST-2 was similar to CHEST-1, with no new safety signals. Improvements in 6MWD and WHO FC observed in CHEST-1 persisted for up to 1 year in CHEST-2. In the observed population at 1 year, mean±sd6MWD had changed by +51±62 m (n=172)versusCHEST-1 baseline (n=237), and WHO FC had improved/stabilised/worsened in 47/50/3% of patients (n=176)versusCHEST-1 baseline (n=236).Long-term riociguat had a favourable benefit–risk profile and apparently showed sustained benefits in exercise and functional capacity for up to 1 year.

scholarly journals Long-Term Outcomes After Percutaneous Transluminal Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension

Circulation ◽  
2016 ◽  
Vol 134 (24) ◽  
pp. 2030-2032 ◽  
Author(s):  
Takumi Inami ◽  
Masaharu Kataoka ◽  
Ryoji Yanagisawa ◽  
Haruhisa Ishiguro ◽  
Nobuhiko Shimura ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Long Term Outcomes ◽  
Thromboembolic Pulmonary Hypertension ◽  
Percutaneous Transluminal

Abstract 13941: Combined Assessment of Right Ventricular Function and Reverse Remodeling as Prognostic Marker after Administration of Medical Treatment for Pulmonary Hypertension

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Hiroyuki Sano ◽  
Hidekazu Tanaka ◽  
Yoshiki Motoji ◽  
Yuko Fukuda ◽  
Kumiko Dokuni ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Reverse Remodeling ◽  
Long Term Outcomes ◽  
Cox Models ◽  
Pulmonary Capillary ◽  
Thromboembolic Pulmonary Hypertension ◽  
Longitudinal Strains

Background: Right ventricular (RV) dysfunction plays an important role in disease progression and prognosis of patients with pulmonary hypertension (PH). However, the association of RV reverse remodeling with long-term outcomes remains unclear. Our objective was thus to test the hypothesis that mid-term RV reverse remodeling after adding PH-specific drugs was linked to long-term outcomes in patients with PH. Methods: We studied 45 PH patients with resting mean pulmonary artery pressure (mPAP) of 35±10mmHg. PH patients with chronic thromboembolic pulmonary hypertension and left-sided heart failure including pulmonary capillary wedge pressure of >15mmHg were excluded. Echocardiography was performed at enrollment and mid-term of 5.7±4.1 months after adding PH-specific drugs. RV function was calculated by averaging the three regional peak speckle-tracking longitudinal strains from RV free-wall (RV-free). RV remodeling was assessed in terms of the RV area, which was traced planimetrically at the end-systole (RVESA) from RV-focused apical 4-chamber views. Mid-term RV reverse remodeling was defined as a relative decrease in RVESA of at least 15%. Long-term event-free survival was then tracked for 5 years. Results: Patients with mid-term RV reverse remodeling experienced favorable long-term outcomes than those without mid-term RV reverse remodeling (log-rank p<0.01). The incremental benefit using sequential Cox models in the prediction of long-term outcomes showed that a model based on clinical variables including mPAP and pulmonary vascular resistance (PVR) was improved by addition of RV-free (p=0.03), and further improved by addition of the parameter of mid-term RV reverse-remodeling (p=0.01). Conclusion: Mid-term RV reverse remodeling after adding PH-specific drugs was associated with long-term outcomes, and this combined approach may well have clinical implications for better management of PH patients.

Pulmonary thromboendarterectomy is a curative resolution for chronic thromboembolic pulmonary hypertension associated with antiphospholipid syndrome: a retrospective cohort study

Lupus ◽  
2018 ◽  
Vol 27 (14) ◽  
pp. 2206-2214 ◽  
Author(s):  
C Li ◽  
J Zhao ◽  
S Liu ◽  
W Song ◽  
J Zhu ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Cohort Study ◽  
Cardiac Function ◽  
Retrospective Cohort Study ◽  
Retrospective Cohort ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Short Term ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and life-threatening condition with poor prognosis in patients with antiphospholipid syndrome (APS). Pulmonary thromboendarterectomy (PTE) is the optimal surgical option for CTEPH. Objectives This retrospective cohort study aimed to evaluate the efficacy and risk of PTE in patients with APS-associated CTEPH. Methods Consecutive patients with APS-associated CTEPH diagnosed between January 2012 and September 2017 at Peking Union Medical College Hospital were retrospectively evaluated. Demographics, clinical manifestations, antiphospholipid antibody (aPL) profiles, and pulmonary arterial hypertension–targeted medications were collected. Deterioration of cardiac function and death were chosen as the endpoints, in order to assess the effect of PTE on short-term and long-term prognoses (evaluated by the change of cardiac function after treatment and cardiac deterioration or death in the follow-up, respectively). Results A total of 20 patients with APS-associated CTEPH were enrolled, and eight patients underwent PTE. Chi-square test ( p = 0.01) and Kaplan–Meier curves (log rank test, p = 0.04) showed that there were statistically significant differences in both short-term and long-term prognoses between patients with and without PTE. Conclusion These results provide strong evidence that PTE is a curative resolution in patients with APS-associated CTEPH. Following a full specialized and multidisciplinary risk-benefit evaluation to limit the risk of thrombosis or bleeding and to manage possible thrombocytopenia, PTE is at least a temporal curative resolution for CTEPH complicated with APS.

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