4975Efficacy of pulmonary hypertension-targeted drugs for severe chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T Inami ◽  
M Kataoka ◽  
J Itoh ◽  
K Takeuchi ◽  
H Kikuchi ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Small Vessel Disease ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Targeted Drugs ◽  
Significant Difference ◽  
Balloon Pulmonary Angioplasty ◽  
Severe Group ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
High Pulmonary Vascular Resistance

Abstract Background High pulmonary vascular resistance (PVR) is a risk factor for reperfusion pulmonary edema by balloon pulmonary angioplasty (BPA). It has not been clarified whether improvement of hemodynamics by pulmonary hypertension (PH)-targeted drugs before BPA session is effective clinically. Severe chronic Thromboembolic pulmonary hypertension (CTEPH) patients have small vessel disease like pathological findings of idiopathic pulmonary arterial hypertension. Objectives The purpose of this study was to investigate the efficacy of PH-targeted drugs for patients with CTEPH before BPA. Methods This study included consecutive 147 CTEPH patients treated with PH-targeted drugs before BPA. All patients were divided into 2 groups such as severe group (PVR >12.5 wood units) and non-severe group (PVR <12.5 wood units). PVR were compared between baseline and after treated with PH-targeted drugs. All data were expressed by median [25thpercentile-75thpercentile]. Results The median age and male were 66 [53–74] years old and 42. Numbers of patients treated with phosphodiesterase V inhibitors, endothelin receptor antagonists and riociguat were 84 (57%), 92 (63%), and 43 (29%), respectively. PVR significantly decreased after treated with PH-targeted drugs in two groups (severe group: 16.3 [13.7–20.0] to 10.0 [7.2–13.3] wu, P<0.0001; non-severe group: 6.8 [4.9–8.9] to 5.7 [3.5] to 7.8, P<0.001). Changes of PVR in severe group were significantly larger than in non-severe group (−6.9 [−8.5 to −3.2] vs. −0.7 [−2.4 to 0.7], P<0.0001). There was no significant difference in efficacy of BPA and numbers of dilated vessel between two groups (severe vs. non-severe group after BPA; PVR: 2.7 [1.9–3.5] vs. 2.1 [1.7–3.1] wu, P>0.05; numbers of dilated vessel: 18 [14–21] vs. 16 [10–21], P>0.05) Conclusions PH-targeted drugs may improve hemodynamics for severe CTEPH more effectively.

Usefulness of peripheral-pressure-directed balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
Y Nagamatsu ◽  
T Inami ◽  
C Nakata ◽  
K Takeuchi ◽  
H Kikuchi ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Oxygen Consumption ◽  
Arterial Pressure ◽  
Pulmonary Arterial Pressure ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Peak Oxygen Consumption ◽  
Pressure Wire ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Abstract Background Balloon pulmonary angioplasty (BPA) in expert center may be considered in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are technically non-operable for pulmonary endarterectomy. BPA based on objective quantitative methods has not been established. The endpoint at BPA sessions has not been clarified yet. Objectives The purpose of this study was to investigate the usefulness of BPA according to peripheral pressure measured by pressure wire or catheter. Methods This study included consecutive 143 CTEPH who underwent cardiopulmonary exercise test with right heart catheterization after BPA. All patients were divided into 2 groups such as angio-guided (n=47) group or pressure-guidedgroup (n=96). In pressure-guided group, the pulmonary arterial pressure (PAP) proximal and distal to the target lesion and the ratio of the 2 pressures were measured by the pressure wire or catheter. the endpoint was determined to when the pressure ratio of distal to proximal pressures was &gt;0.7 to 0.8 finally. The dilation was stopped when the distal mean PAP after each dilation reached 35 mm Hg and when the baseline mean PAP was &gt;35 mm Hg. Changes of hemodynamic parameters such as PAP and pulmonary vascular resistance (PVR) from baseline, and mean PAP and PVR at peak oxygen consumption, and pulmonary arterial pressure – cardiac output (PA-CO) slope were compared between two groups. All data except changes of hemodynamics from baseline were expressed by median [25th percentile-75th percentile]. Changes of hemodynamics from baseline were expressed by average [95% confidence interval] Results The median age and male were 66 [56–72] years old and 35. There were no significant differences in number of sessions and dilated vessels between two groups (Angio-guided group vs. Pressure-guided group: 4 [3–6] vs. 4 [3–5], P&gt;0.05; 19 [12–22] vs. 17 [13–22], P&gt;0.05). Changes of PAP and PVR from baseline in pressure-guided group was significantly higher than in angio-guided groups (Angio-guided group vs. Pressure-guided group; ΔPAP: −19 [−20 to −17] vs. −20 [−21 to −20], P&gt;0.05; ΔPVR: −6.0 [−6.3 to −5.7] vs. −6.6 [−6.8 to −6.3], P&lt;0.01). Mean PAP and PVR at peak oxygen consumption (VO2) and PA-CO slope in pressure-guided group were significantly lower than in angio-guided group (Angio-guided group vs. Pressure-guided group; PAP at VO2: 43 [36–50] vs. 39 [34–43], P&lt;0.05; PVR at VO2: 2.7 [1.9–4.5] vs. 2.2 [1.6–2.9], P&lt;0.01; PA-CO slope: 3.8 [2.1–5.2] vs. 2.9 [1.9–4.0], P&lt;0.05). Conclusions Peripheral-pressure-directed BPA can improve hemodynamics at rest and exercise more effectively. Funding Acknowledgement Type of funding source: None

scholarly journals Epidemiology and Management of Chronic Thromboembolic Pulmonary Hypertension in Greece. Real-World Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

2021 ◽  
Vol 10 (19) ◽  
pp. 4547
Author(s):  
Eftychia Demerouti ◽  
Panagiotis Karyofyllis ◽  
Vassilios Voudris ◽  
Maria Boutsikou ◽  
George Anastasiadis ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
World Health ◽  
Real World Data ◽  
Significant Difference ◽  
Thromboembolic Pulmonary Hypertension ◽  
History Of ◽  
Pulmonary Arterial ◽  
Health Organization

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare disease with poor prognosis if left untreated, characterized by pulmonary vascular bed obstruction due to unresolving thromboembolic material. The Hellenic pulmonary hypertension registry (HOPE) was launched in Greece in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. In total, 98 patients with CTEPH were enrolled from January 2015 until November 2019. Of these patients, 55.1% represented incident population, 50% were classified in the World Health Organization functional class II and 49% had a history of acute pulmonary embolism. The median values of pulmonary vascular resistance (PVR) and cardiac index were 7.4 (4.8) WU and 2.4 (1.0) L/min/m2, respectively, the mean diffusing capacity for carbon monoxide was 74.8 ± 20.6%, the median 6-minute walk distance was 347 (220) meters and the median value of N Terminal-pro brain natriuretic peptide was 506.0 (1450.0) pg/mL. In total, 60.2% of the patients were under pulmonary arterial hypertension-targeted therapy at the time of enrolment; specifically, riociguat was received by 35.7% of the patients and combination therapy was the preferred strategy for 16% of the patients. In total, 74 patients were evaluated for pulmonary endarterectomy (PEA), 34 (45.9%) were assessed as operable but only 23 of those (31.1%) finally underwent PEA. The remaining 40 patients were ineligible for PEA according to the operability assessment and 13 (17.6%) of them underwent balloon pulmonary angioplasty. The age of the non-operable patients was significantly higher than the operable patients (p < 0.001), while there was no significant difference with regard to the history of coagulopathies between the operable and non-operable patients (p = 0.33).

scholarly journals Multicentre randomised controlled trial of balloon pulmonary angioplasty and riociguat in patients with chronic thromboembolic pulmonary hypertension: protocol for the MR BPA study

BMJ Open ◽  
2020 ◽  
Vol 10 (2) ◽  
pp. e028831 ◽  
Author(s):  
Takashi Kawakami ◽  
Hiromi Matsubara ◽  
Kohtaro Abe ◽  
Masaharu Kataoka ◽  
Shun Kohsaka ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Randomised Controlled Trial ◽  
Soluble Guanylate Cyclase ◽  
Pulmonary Arterial Pressure ◽  
Controlled Trial ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Randomised Controlled

IntroductionManagement of inoperable chronic thromboembolic pulmonary hypertension (CTEPH) remains a clinical challenge. Currently, medical treatment involving pulmonary vasodilators (such as soluble guanylate-cyclase stimulators) is recommended, primarily for ameliorating symptoms. More recently, balloon pulmonary angioplasty (BPA) has been developed as alternative treatment for inoperable CTEPH. This study aimed to compare the efficacy and safety of BPA and riociguat (a soluble guanylate-cyclase stimulator) as treatments for inoperable CTEPH.Methods and analysisThis study is a multicentre randomised controlled trial. Subjects with inoperable CTEPH were randomised (1:1) into either a BPA or riociguat group, and observed for 12 months after initiation of treatment. The primary endpoint will be the change in mean pulmonary arterial pressure from baseline to 12 months after initiation of treatment. For primary analysis, we will estimate the least square means difference and 95% CI for the change of pulmonary arterial pressure between the groups at 12 months using the analysis of covariance adjusted for allocation factors.Ethics and disseminationThis study and its protocols were approved by the institutional review board of Keio University School of Medicine and each participating institution. Written informed consent was obtained from all participants. Results will be disseminated at medical conferences and in journal publications.Trial registration numberUniversity Hospital Medical Information Network Clinical Trial Registry (UMIN000019549); Pre-results.

scholarly journals Prognostic Value of Oxidative Stress Markers in Patients with Pulmonary Arterial or Chronic Thromboembolic Pulmonary Hypertension

2019 ◽  
Vol 2019 ◽  
pp. 1-10 ◽  
Author(s):  
Anna Smukowska-Gorynia ◽  
Piotr Rzymski ◽  
Justyna Marcinkowska ◽  
Barbara Poniedziałek ◽  
Anna Komosa ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Pulmonary Hypertension ◽  
Prognostic Value ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Clinical Deterioration ◽  
Oxidative Stress Markers ◽  
Stress Markers ◽  
Significant Difference ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Oxidative stress is regarded to play a crucial role in the pathophysiology of pulmonary arterial hypertension (PAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH). This study evaluated the prognostic value of serum oxidative stress markers (malondialdehyde (MDA), total antioxidant capacity (TAC), catalase activity (CAT), and superoxide activity (SOD)) in patients with PAH and CTEPH (n=45). During 13 months of follow-up (median 9 months), clinical deterioration occurred in 14 patients (including 2 deaths). On the Cox regression analysis, MDA, TAC, and CAT were associated with clinical deterioration (p=0.0068, HR=1.42, 95% CI: 1.10-1.82; p=0.0038, HR=0.033, 95% CI: 0.0032-0.33; and p=0.046, HR=0.20, 95% CI: 0.04-0.98, respectively). There was no significant difference in SOD (p=0.53, HR=0.97, 95% CI: 0.87-1.08). The cut-off value derived from ROC curve analysis was 3.79 μM (p=0.0048, AUC=0.76, 95% CI: 0.62-0.91) for MDA, 0.49 mM (p=0.027, AUC=0.71, 95% CI: 0.18-0.47) for TAC, and 1.34 U/L (p=0.029, AUC=0.71, 95% CI: 0.55-0.86) for CAT. MDA in the group with deterioration was higher (p=0.0041), while TAC as well as CAT were lower (p=0.027 and p=0.028, respectively) when compared to stable patients. Survival without clinical deterioration was significantly longer in patients with lower MDA (p=0.037, HR=0.37, 95% CI: 0.12-1.14, log-rank), higher TAC (p=0.0018, HR=0.19, 95% CI: 0.06-0.60, log-rank), and higher CAT (p=0.044, HR=0.31 95% CI: 0.11-0.88, log-rank). Markers of oxidative stress such as MDA, TAC, and CAT were associated with adverse clinical outcomes in patients with PAH and inoperable or residual CTEPH.

scholarly journals Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension

2017 ◽  
Vol 26 (143) ◽  
pp. 160119 ◽  
Author(s):  
Irene Lang ◽  
Bernhard C. Meyer ◽  
Takeshi Ogo ◽  
Hiromi Matsubara ◽  
Marcin Kurzyna ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Fibrous Tissue ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Term Survival ◽  
Percutaneous Balloon ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Vessel Dissection

Chronic thromboembolic pulmonary hypertension (CTEPH) is thought to result from incomplete resolution of pulmonary thromboemboli that undergo organisation into fibrous tissue within pulmonary arterial branches, filling pulmonary arterial lumina with collagenous obstructions. The treatment of choice is pulmonary endarterectomy (PEA) in CTEPH centres, which has low post-operative mortality and good long-term survival. For patients ineligible for PEA or who have recurrent or persistent pulmonary hypertension after surgery, medical treatment with riociguat is beneficial. In addition, percutaneous balloon pulmonary angioplasty (BPA) is an emerging option, and promises haemodynamic and functional benefits for inoperable patients. In contrast to conventional angioplasty, BPA with undersized balloons over guide wires exclusively breaks intraluminal webs and bands, without dissecting medial vessel layers, and repeat sessions are generally required. Observational studies report that BPA improves haemodynamics, symptoms and functional capacity in patients with CTEPH, but controlled trials with long-term follow-up are needed. Complications include haemoptysis, wire injury, vessel dissection, vessel rupture, reperfusion pulmonary oedema, pulmonary parenchymal bleeding and haemorrhagic pleural effusions. This review summarises the available evidence for BPA, patient selection, recent technical refinements and periprocedural imaging, and discusses the potential future role of BPA in the management of CTEPH.

scholarly journals Balloon Pulmonary Angioplasty in Technically Operable and Technically Inoperable Chronic Thromboembolic Pulmonary Hypertension

2021 ◽  
Vol 10 (5) ◽  
pp. 1038
Author(s):  
Szymon Darocha ◽  
Aleksander Araszkiewicz ◽  
Marcin Kurzyna ◽  
Marta Banaszkiewicz ◽  
Stanisław Jankiewicz ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Rank Test ◽  
Log Rank Test ◽  
Significant Difference ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Minute Walk ◽  
Long Term Studies

Background: In this study, we aimed to assess the efficacy and safety of balloon pulmonary angioplasty (BPA) in patients with technically inoperable distal-type chronic thromboembolic pulmonary hypertension (d-CTEPH) and technically operable proximal-type disease (p-CTEPH) by analyzing the results of BPA treatment in two collaborating CTEPH referral centers. Methods and results: We assessed hemodynamic results, functional efficacy, complication and survival rate after BPA treatment in 70 CTEPH patients (median age 64 years; (interquartile range (IQR): 52–73 years)), of whom 16 (median age 73 years; (QR 62–82 years)) were in the p-CTEPH subgroup. Altogether, 377 BPA procedures were performed, resulting in significant (p < 0.001) improvement in mean pulmonary artery pressure (mPAP 48.6 ± 10 vs. 31.3 ± 8.6 mmHg), pulmonary vascular resistance (694 ± 296 vs. 333 ± 162 dynes*s*cm−5), six-minute walk test (365 ± 142 vs. 433 ± 120 metres) and N-terminal pro B-type natriuretic peptide (1307 (510–3294) vs. 206 (83–531) pg/mL). The rate of improvement did not differ between the sub-groups. Lung injury episodes and severe hemoptysis were similarly infrequent in d-CTEPH and p-CTEPH (6.4% vs. 5%; p = 0.55 and 1.0% vs. 2.5; p = 0.24, respectively). There was no significant difference between the sub-groups regarding survival (p = 0.53 by log-rank test). Conclusion: BPA may be beneficial in patients with p-CTEPH who cannot undergo pulmonary endarterectomy (PEA). Larger long-term studies are needed to better define the efficacy, safety, and optimal BPA procedural standards in this population.

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