Search

Background: Treatment options for patients with chronic thromboembolic pulmonary hypertension ineligible for pulmonary endarterectomy (inoperable CTEPH) include balloon pulmonary angioplasty (BPA) and riociguat. However, these two treatment options have not been compared prospectively. We aimed to compare the safety and efficacy of BPA and riociguat in patients with inoperable CTEPH., Methods: This open-label, randomised controlled trial was conducted at four high-volume CTEPH centres in Japan. Patients aged 20-80 years with inoperable CTEPH (mean pulmonary arterial pressure ≥25 to <60 mm Hg and pulmonary artery wedge pressure ≤15 mm Hg) and WHO functional class II or III were randomly assigned (1:1) to BPA or riociguat via a computer program located at the registration centre using a minimisation method with biased-coin assignment. In the BPA group, the aim was for BPA to be completed within 4 months of the initial date of the first procedure. BPA was repeated until mean pulmonary arterial pressure decreased to less than 25 mm Hg. The frequency of BPA procedures depended on the difficulty and number of the lesions. In the riociguat group, 1·0 mg riociguat was administered orally thrice daily. When the systolic blood pressure was maintained at 95 mm Hg or higher, the dose was increased by 0·5 mg every 2 weeks up to a maximum of 2·5 mg thrice daily; dose adjustment was completed within 4 months of the date of the first dose. The primary endpoint was change in mean pulmonary arterial pressure from baseline to 12 months, measured in the full analysis set (patients who were enrolled and randomly assigned to one of the study treatments, and had at least one assessment after randomisation). BPA-related complications and indices related to clinical worsening were recorded throughout the study period. Adverse events were recorded throughout the study period and evaluated in the safety analysis set (patients who were enrolled and randomely assigned to one of the study treatments, and had received part of or all the study treatments). This trial is registered in the Japan Registry of Clinical Trials (jRCT; jRCTs031180239) and is completed., Findings: Between Jan 8, 2016, and Oct 31, 2019, 61 patients with inoperable CTEPH were enrolled and randomly assigned to BPA (n=32) or riociguat (n=29). Patients in the BPA group underwent an average of 4·7 (SD 1·6) BPA procedures. In the riociguat group, the mean maintenance dose was 7·0 (SD 1·0) mg/day at 12 months. At 12 months, mean pulmonary arterial pressure had improved by -16·3 (SE 1·6) mm Hg in the BPA group and -7·0 (1·5) mm Hg in the riociguat group (group difference -9·3 mm Hg [95% CI -12·7 to -5·9]; p<0·0001). A case of clinical worsening of pulmonary hypertension occurred in the riociguat group, whereas none occurred in the BPA group. The most common adverse event was haemosputum, haemoptysis, or pulmonary haemorrhage, affecting 14 patients (44%) in the BPA group and one (4%) in the riociguat group. In 147 BPA procedures done in 31 patients, BPA-related complications were observed in 17 procedures (12%) in eight patients (26%)., Interpretation: Compared with riociguat, BPA was associated with a greater improvement in mean pulmonary arterial pressure in patients with inoperable CTEPH at 12 months, although procedure-related complications were reported. These findings support BPA as a reasonable option for inoperable CTEPH in centres with experienced BPA operators, with attention to procedure-related complications., Funding: Bayer Yakuhin., Translation: For the Japanese translation of the abstract see Supplementary Materials section., Competing Interests: Declaration of interests TK received lecture fees from Bayer Yakuhin, Daiichi Sankyo, Abbott Medical Japan, Acist Japan, Kaneka Medix; and contributes to a course financially supported by Nippon Shinyaku. HM received a grant from Bayer Yakuhin; and personal fees from Actelion Pharmaceuticals Japan, AYUMI Pharmaceutical, Medical Review, Kaneka Medix, GlaxoSmithKline, Daiichi Sankyo, Toray Industries, Nippon Shinyaku, Bayer Yakuhin, Pfizer, Sumitomo Dainippon Pharma, Mochida Pharmaceutical, Janssen Pharmaceutical, and TOA EIYO. KA received research funding from Mochida Pharmaceutical, Actelion Pharmaceuticals Japan, and Daiichi Sankyo. SK received grants from Daiichi Sankyo and Novartis Pharma; and personal fees from Bristol-Myers Squibb. KHo received research funding from Konica Minolta. YT received grants from Actelion Pharmaceuticals and Nippon Shinyaku. HS received a grant from Bayer Yakuhin; and personal fees from Bayer Yakuhin, Nippon Shinyaku, Daiichi Sankyo, and Actelion Pharmaceuticals Japan. AO received a grant from Bayer Yakuhin; personal fees from Actelion Pharmaceuticals Japan, Nippon Shinyaku, Merck, and Bayer Yakuhin; and contributed to collaborative research with Nippon Shinyaku. MJ received a grant from Bayer Yakuhin. KHi received grants from Actelion Pharmaceuticals and Nippon Shinyaku. HT received grants from Daiichi Sankyo, Mitsubishi Tanabe Pharma, Boehringer Ingelheim Japan, IQVIA Solutions Japan, Omron Healthcare, and MEDINET; and personal fees from Daiichi Sankyo, Mitsubishi Tanabe Pharma, Boehringer Ingelheim Japan, AstraZeneca, Ono Pharmaceutical, Otsuka Pharmaceutical, Teijin Pharma, Novartis Pharma, Bayer Yakuhin, Pfizer, Bristol-Myers Squibb, Kowa Company, and Nippon Rinsho. YS received personal fees from Mochida Pharmaceutical, Kowa Company, and Eisai. TS, YY, MK, MM, and KF declare no competing interests., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Objective: To evaluate whether the change in computed tomography pulmonary angiography (CTPA) metrics after balloon pulmonary angioplasty (BPA) can predict treatment effect in chronic thromboembolic pulmonary hypertension (CTEPH) patients., Methods: This study included 82 CTEPH patients who underwent both CTPA and right heart catheterization (RHC) before and at the scheduled time of 6 months after BPA. The diameters of the main pulmonary artery (dPA), ascending aorta (dAA), right atrium (dRA), right ventricular free wall thickness (dRVW), and right and left ventricles (dRV, dLV) were measured on CTPA. The correlation of the New York Heart Association functional class (NYHA FC), 6-minute walking distance (6MWD), brain natriuretic peptide (BNP) level, and calculated CT metrics with a decrease in mean pulmonary artery pressure (ΔmPAP) using RHC (used as the reference for BPA effect) was investigated. Using multiple regression analysis, independent variables were also identified., Results: In univariate analysis, clinical indicators (NYHA FC, 6MWD, and BNP level) improved significantly after BPA and were significantly correlated with ΔmPAP (p < 0.01). In the univariate analysis of CTPA parameters, dPA, dRA, dPA/dAA ratio, dRVW, and dRV/dLV ratio decreased significantly and were significantly correlated with ΔmPAP (p < 0.01). Multivariate analysis demonstrated that decreased dPA (p = 0.001) and decreased dRA (p = 0.039) on CTPA were independent predictive factors of ΔmPAP., Conclusions: Decreased dPA and dRA on CTPA could predict a decrease in mPAP after BPA, thus potentially eliminating unnecessary invasive catheterization., Key Points: • The reduction in mean pulmonary artery pressure after balloon pulmonary angioplasty in CTEPH patients was significantly correlated with the clinical indices improvement and CTPA parameter decrease. • The decreased diameter of the main pulmonary artery and the decreased diameter of the right atrium on CTPA were independent predictors of mean pulmonary artery pressure reduction., (© 2021. European Society of Radiology.)

The efficacy of extensive balloon pulmonary angioplasty (BPA) beyond hemodynamic improvement in chronic thromboembolic pulmonary hypertension (CTEPH) patients has been verified. However, the relationship between extensive BPA in CTEPH patients after partial hemodynamic improvement and exercise tolerance or quality of life (QOL) remains unclear. We prospectively enrolled 22 CTEPH patients (66±10 years, females: 59%) when their mean pulmonary artery pressure initially decreased to <30 mmHg during BPA sessions. Hemodynamic and echocardiographic data, cardiopulmonary exercise testing, and QOL scores using the 36-item short form questionnaire (SF-36) were evaluated at enrollment (entry), just after the final BPA session (finish), and at the 6-month follow-up (follow-up). We analyzed whether extensive BPA improves exercise capacity and QOL scores over time. Moreover, the clinical characteristics leading to improvement were elucidated. The peak oxygen uptake (VO2) showed significant improvement at entry, finish, and follow-up (17.3±5.5, 18.4±5.9, and 18.9±5.3 mL/kg/min, respectively; P<0.001). Regarding the QOL, the physical component summary (PCS) scores significantly improved (32±11, 38±13, and 43±13, respectively; P<0.001), but the mental component summary scores remained unchanged. Linear regression analysis revealed that age and a low peak VO2 at entry were predictors of improvement in peak VO2, while low PCS scores and low TAPSE at entry were predictors of improvement in PCS scores. In conclusion, extensive BPA led to improved exercise tolerance and physical QOL scores, even in CTEPH patients with partially improved hemodynamics., Competing Interests: The authors have declared that no competing interests exist.

Aims: Balloon pulmonary angioplasty (BPA) improves hemodynamics and exercise tolerance in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, its diagnostic and predictive values remain unclear. We investigated the diagnostic and predictive values of BPA by assessing the mechanism of right ventricular (RV) dysfunction., Methods and Results: Hemodynamic improvement was maintained over 6 months in 99 patients with CTEPH who underwent BPA. Notably, 57 of 99 patients showed normalization of pulmonary vascular resistance (PVR) after BPA. The RV mid free wall longitudinal strain (RVMFS) was inversely correlated with the 6-min walk distance (r = -.35, P = .01) and serum levels of high-sensitivity cardiac troponin T (hs-cTNT) (r = -.39, P = .004) 6 months post-BPA in the PVR-normalized group. Among all variables analyzed, only the pre-BPA RVMFS was correlated with the post-BPA RVMFS (r = .40, P = .001), and the pre-BPA RVMFS (<-15.8%) was the strongest predictor of post-BPA normalization of RVMFS (area under the curve 0.80, P = .01, sensitivity 89%, and specificity 63%). The immediate post-BPA RVMFS showed worsening over 6 months after the procedure (-25.8% to -21.1%) in patients with high serum hs-cTNT levels (>0.0014 ng/mL). In contrast, we observed an improvement in these values in those with low serum hs-cTNT levels (-23.6% to -24.4%)., Conclusion: RVMFS of -15.8% may be a useful cutoff value to categorize the refractory and non-refractory stages of disease. Sustained serum hs-cTNT elevation post-BPA indicates subclinical RV myocardial injury, with resultant RVMFS deterioration and poor exercise tolerance., (© 2020 Wiley Periodicals LLC.)

Background Right ventricular (RV) dysfunction is a prognostic factor for cardiovascular disease. However, its mechanism and pathophysiology remain unknown. We investigated RV function using RV-specific 3-dimensional (3D)-speckle-tracking echocardiography (STE) in patients with chronic thromboembolic pulmonary hypertension. We also assessed regional wall motion abnormalities in the RV and chronological changes during balloon pulmonary angioplasty (BPA). Methods and Results Twenty-nine patients with chronic thromboembolic pulmonary hypertension who underwent BPA were enrolled and underwent right heart catheterization and echocardiography before, immediately after, and 6 months after BPA. Echocardiographic assessment of RV function included both 2-dimensional-STE and RV-specific 3D-STE. Before BPA, global area change ratio measured by 3D-STE was significantly associated with invasively measured mean pulmonary artery pressure and pulmonary vascular resistance ( r =0.671 and r =0.700, respectively). Dividing the RV into the inlet, apex, and outlet, inlet area change ratio showed strong correlation with mean pulmonary artery pressure and pulmonary vascular resistance before BPA ( r =0.573 and r =0.666, respectively). Only outlet area change ratio was significantly correlated with troponin T values at 6 months after BPA ( r =0.470), and its improvement after BPA was delayed compared with the inlet and apex regions. Patients with poor outlet area change ratio were associated with a delay in RV reverse remodeling after treatment. Conclusions RV-specific 3D-STE analysis revealed that 3D RV parameters were novel useful indicators for assessing RV function and hemodynamics in pulmonary hypertension and that each regional RV portion presents a unique response to hemodynamic changes during treatment, implicating that evaluation of RV regional functions might lead to a new guide for treatment strategies.

Introduction: Management 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 Analysis: This 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 Dissemination: This 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 Number: University Hospital Medical Information Network Clinical Trial Registry (UMIN000019549); Pre-results., Competing Interests: Competing interests: SK received honoraria for scientific lectures from Bayer Yakuhin Ltd. KF received scholarship grants from Bayer Yakuhin Ltd. and MSD K.K., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

In recent years, therapeutic options for patients with chronic thromboembolic pulmonary hypertension (CTEPH) have expanded with the development of catheter-based interventional therapy, namely balloon pulmonary angioplasty (BPA), also called percutaneous transluminal pulmonary angioplasty. For CTEPH patients with technically inoperable disease or with an unfavorable risk-to-benefit ratio for surgical pulmonary endarterectomy, BPA is an important alternative therapeutic strategy. One important treatment goal of BPA should be the relief of pulmonary hypertension. However, the indications for BPA in specialized Japanese centers currently go beyond the sole indication of relieving pulmonary hypertension. BPA is currently limited to specific institutes and experienced operators, which allows better management of its associated complications of reperfusion pulmonary edema and vascular injury using various strategies based on past experiences. This article discusses the latest indications and treatment goals of BPA and the current flow diagram for therapeutic decision-making in CTEPH, and summarizes the factors to be considered when performing BPA, from a Japanese perspective., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Balloon pulmonary angioplasty (BPA) improves pulmonary hemodynamics in chronic thromboembolic pulmonary hypertension (CTEPH) patients. However, whether it affects the severity of sleep apnea (SA) remains unknown. We investigated the effect of BPA on the severity of SA in CTEPH patients., Methods: We studied 13 patients with CTEPH who had an apnea hypopnea index (AHI) > 10 before BPA and underwent a second polygraph test 6 months after the last BPA session., Results: BPA decreased pulmonary vascular resistance, mean pulmonary artery pressure (PAP), and plasma B-type natriuretic peptide levels, and increased the 6-minute walking distance. BPA decreased the AHI (from 20.9 [13.9-35.7] to 16.3 [7.7-21.8] times/hour, P = 0.023) and hypopnea index (from 13.2 [8.4-22.5] to 6.4 [3.8-10.9] times/hour, P = 0.013), but not the obstructive, central, or mixed apnea index. The change in AHI correlated with that in mean PAP, but not with the change in body mass index or other parameters of hemodynamics., Conclusions: BPA-induced improvement in hemodynamics was associated with the attenuation of SA in patients with CTEPH and SA. Therefore, close attention should be paid to SA in CTEPH patients, and SA should be re-evaluated after BPA to avoid overestimating its severity., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Balloon pulmonary angioplasty (BPA) has emerged as a new treatment strategy for patients with chronic thromboembolic pulmonary hypertension (CTEPH). Improvements in hemodynamic parameters after BPA have been reported, but some patients continue to suffer from reduced exercise tolerance even after the normalization of hemodynamic parameters following BPA. As the amelioration of hemodynamic parameters is reportedly achieved via BPA, we hypothesized that the limiting factors for exercise tolerance in these patients are related to respiratory function. Therefore, we investigated the associations between respiratory function and exercise tolerance, and the mechanisms underlying respiratory dysfunction in patients after BPA. We analyzed 62 patients with CTEPH who underwent 1-year follow-up after BPA. Predictors for reduced exercise tolerance after BPA determined with six-minute walk test were sought from pulmonary hemodynamic and respiratory parameters using logistic regression analysis. After multivariate adjustments, high mean right atrium pressure (mRAP) and high alveolar-arterial oxygen gradient (A-aDO 2 ) were significant predictors for reduced exercise tolerance. Next, we analyzed factors associated with high A-aDO2. Among the pathophysiological causes of high A-aDO2, including ventilation, diffusing capacity, and low ventilation-perfusion ratio, only low ventilation-perfusion ratio caused by high intrapulmonary shunt fraction was associated with high A-aDO 2 . Impaired oxygenation due to residual high intrapulmonary shunt fraction was associated with reduced exercise tolerance in patients with CTEPH, after receiving BPA.

Balloon pulmonary angioplasty (BPA) has emerged as an effective treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Renal function has been identified as a prognostic marker in patients with pulmonary hypertension in previous studies. We, therefore, aimed to investigate the clinical parameters associated with improvements in renal function in patients with CTEPH. A total of 45 consecutive patients with inoperable CTEPH undergoing BPA (mean age 62.2 ± 15.1 years) were included in the study. We evaluated the patients' clinical characteristics at baseline and at 1-year post-BPA, and investigated the association between renal function and hemodynamic parameters, including right heart function. Hemodynamics and renal function showed sustained improvements at 1 year after BPA in 64.4% of patients. Improved estimated glomerular filtration rate (eGFR) was significantly correlated with increased cardiac index (r = 0.433, p = 0.003) and mixed venous oxygen saturation (SvO 2 ; r = 0.459, p = 0.002), and with decreased mean pulmonary arterial pressure (r = - 0.420, p = 0.004) and pulmonary vascular resistance (r = -- 0.465, p = 0.001). Multivariate analysis revealed that an increase in SvO 2 immediately after the final BPA was associated with improved eGFR after the 1st year (odds ratio 1.041; 95% confidence interval 1.004-1.078; P = 0.027). The cut-off value for predicting improved eGFR was an increase in SvO 2 after the final BPA of >125.4% over the baseline value (specificity 100%, sensitivity 24.1%). In conclusion, BPA improved symptoms, right heart function, hemodynamics, and renal function up to the chronic phase. Increasing SvO 2 by >125.4% above baseline in the acute phase is important for improving renal function at 1 year after BPA in CTEPH patients.

Background: There is no consensus on the length of hospital stay (LOHS) and post-interventional management after balloon pulmonary angioplasty (BPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). We examined temporal trends with respect to LOHS and requirement for intensive care for BPA and their relationship with the incidence of BPA-related complications., Methods: From November 2012 to September 2017, a total of 123 consecutive patients with CTEPH who underwent BPA were enrolled (age: 66.0 [54.0 to 74.0], World Health Organization [WHO] functional class II/III/IV; 27/88/8). Patients were divided for analysis into 3 groups according to the date of their first BPA: early-, middle-, and late-phase groups., Results: Mean pulmonary arterial pressure decreased from 36.0 (29.0 to 45.0) to 20.0 (16.0 to 22.0) mm Hg after BPA (P < 0.001). The LOHS was 41.0 (31.0 to 54.0) days in total including all sessions and 6.6 (6.0 to 7.9) days/session. Despite no significant differences in age, baseline hemodynamics, and laboratory data among the 3 groups, there was a significant reduction in LOHS (7.9 [7.0 to 9.5], 6.5 [6.1 to 7.3], 6.0 [5.3 to 6.5] days/session, P < 0.001) and use of intensive/high care unit (100%, 93%, 46%, P < 0.001). The reduction in LOHS and intensive/high care unit use did not affect the occurrence of BPA-related complications., Conclusions: Increasing experience with BPA was associated with a reduction in LOHS and the use of intensive/high care unit, but no change was noted in the rate of BPA-related complications. These findings suggest that the reduction in both LOHS and use of the intensive care unit for BPA is feasible and does not jeopardize the safety of the procedure., (Copyright © 2018 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Balloon pulmonary angioplasty (BPA) has been performed for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or residual pulmonary hypertension after pulmonary endarterectomy (PEA). We performed a systematic review to assess the efficacy and safety of BPA, especially compared to medical treatment or PEA., Methods: We reviewed all studies investigating pre- and post-treatment pulmonary hemodynamics, mortality, or complications from three electronic databases (PubMed, Cochrane Library, Japan Medical Abstracts Society) prior to February 2017. From 26 studies retrieved, we selected 13 studies (493 patients): the 10 most recent ones including complete data from each institution, one study of residual pulmonary hypertension, and two studies comparing BPA with medical treatment or PEA., Results: No randomized controlled or prospective controlled studies comparing BPA with medical treatment or PEA were reported. The early mortality of BPA ranged from 0% to 14.3%; lung injury occurred in 7.0% to 31.4% (average sessions, 2.5-6.6). Mean pulmonary arterial pressure decreased from 39.4-56 to 20.9-36 mm Hg, and the 6-min walk distance increased from 191-405 to 359-501 m. The 2-year mortality of 80 patients undergoing BPA was significantly lower compared to 68 patients receiving medical treatment (1.3% vs. 13.2%); the risk ratio was 0.14 (95% confidence interval: 0.03-0.76). No significant difference was observed in the 2-year mortality between BPA (n=97) and PEA (n=63) patients., Conclusions: This systematic review suggests that BPA improves hemodynamics, has acceptable early mortality, and may improve long-term survival compared with medical treatment in inoperable CTEPH patients., (Copyright © 2018 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.)

Chronic thromboembolic pulmonary hypertension (CTEPH) has a poor prognosis because of the associated progressive right heart failure. Accurate evaluation of right ventricular (RV) function would thus be useful to predict prognosis. However, the significance of RV diastolic function remains unclear. We aimed to identify which echocardiographic measures are most accurate, and potentially useful, in assessing RV diastolic function in patients with CTEPH, and to study the effects of balloon pulmonary angioplasty (BPA) on them. We enrolled 53 CTEPH patients who underwent BPA. Echocardiographic parameters, including two-dimensional speckle-tracking echocardiography, were compared to the hemodynamic parameters measured by right heart catheterization before and after BPA. RV strain rate during early diastole (SR&#95;E), tricuspid e' and right atrial area (RAA) were ameliorated after BPA, concomitant with a decrease in the time constant of the RV pressure curve during diastole (tau), indicating the improvement of RV diastolic function. Among them, SR&#95;E had the strongest correlation with tau (r = - 0.39, p < 0.001). Furthermore, the receiver operating characteristic analyses revealed that E/SR&#95;E (AUC 0.704) and inferior vena cava diameter (AUC 0.726) had a stronger association with higher mean right atrial pressure than RAA (AUC 0.632). In contrast, RAA had a stronger correlation with 6 min-walk distances than SR&#95;E (r = - 0.39, p < 0.001 vs. r = 0.30, p = 0.005). Taken together, echocardiographic assessment of RV diastolic function might be associated with hemodynamics as well as exercise tolerance in patients with CTEPH, indicating its benefits in evaluating the therapeutic effects of BPA.

We performed a 2-stage procedure combining balloon pulmonary angioplasty and pulmonary endarterectomy for a high-risk chronic thromboembolic pulmonary hypertension patient with high pulmonary vascular resistance. First, balloon pulmonary angioplasty was performed for distal lesions to improve hemodynamics and decrease the surgical risk. Subsequently, pulmonary endarterectomy was performed for proximal lesions, and the hemodynamics and symptoms improved dramatically. Our strategy was therefore found to be useful for treating a high-risk chronic thromboembolic pulmonary hypertension patient., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: For treatment of chronic thromboembolic pulmonary hypertension (CTEPH), the evaluation of segmental pulmonary perfusion is important. There are no previous reports about lung subtraction iodine mapping (LSIM) computed tomography (CT) for evaluation of segmental pulmonary perfusion in patients with CTEPH, using lung perfusion SPECT/CT (LPS) as the reference., Methods: 50 patients (age, 60.7±16.7years) with known or suspected CTEPH were enrolled in this study. Non-contrast chest CT and CT pulmonary angiography (CTPA) were performed on a 320-detector row CT system. Then, based on a non-rigid registration followed by subtraction of non-contrast images from contrast-enhanced images, color-coded LSIM images were generated. LPS was performed using a SPECT/CT system within a period of 2months, and served as the reference standard. LSIM and CTPA images were evaluated in a blinded manner for the detection of pulmonary perfusion defects on a segment-by-segment basis., Results: The sensitivity, specificity, accuracy, and positive and negative predictive values of LSIM for the detection of segmental perfusion defects were 95% (734/773), 84% (107/127), 93% (841/900), 97% (734/754) and 73% (107/146), respectively, while the corresponding values for CTPA were 65% (505/773), 61% (78/127), 65% (583/900), 91% (505/554) and 23% (78/346). Generalized estimating equations analyses revealed a significantly better performance of LSIM than that of CTPA regarding the sensitivity, accuracy, and positive and negative predictive values (all P<0.0001)., Conclusions: LSIM is a feasible technique for segment-based evaluation of pulmonary perfusion in patients with CTEPH, and it provides a significantly higher diagnostic accuracy compared with CTPA., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: The acute favourable effect of balloon pulmonary angioplasty (BPA) has been proven in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, data on its effect 6 months after therapy (from now on referred to as mid-term) and influence on the right ventricle and myocardial damage are sparse. To evaluate factors that influence improvement in cardiac output (CO) and subclinical myocardial damage, we examined hemodynamics and serum high-sensitivity troponin T (hs-TnT) levels before, 1 week after, and 6 months after BPA., Methods: In a retrospective study, we reviewed 67 consecutive patients from November 2012 to January 2016 with CTEPH who had undergone BPA at Keio University Hospital., Results: Six months after BPA, the mean right atrium pressure, mean pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR), B-type natriuretic peptide (BNP), and hs-TnT levels decreased; CO and 6-minute walking distance increased. Changes in CO and hs-TnT levels varied compared with other hemodynamic parameters and BNP levels. The CO-increase group (n = 42) had higher mean PAP and PVR, and lower CO at baseline than the CO-decrease/stable group (n = 25). The hs-TnT-decrease group (n = 36) had higher mean right atrium pressure, PAP, PVR, and BNP levels, and lower CO at baseline than the hs-TnT-increase/stable group (n = 31)., Conclusions: Six months after BPA, hemodynamics and exercise capacity improved and hs-TnT levels decreased. Improvements in CO and hs-TnT levels were more prominent in CTEPH patients with impaired baseline hemodynamics, suggesting that BPA has a favourable mid-term effect on hemodynamics and subclinical myocardial damage in patients with CTEPH, especially in those with impaired hemodynamics., (Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

The aim was to investigate the effect of balloon pulmonary angioplasty (BPA) on right ventricular (RV) function in chronic thromboembolic pulmonary hypertension. Twenty-six patients with chronic thromboembolic pulmonary hypertension were enrolled and were divided into 2 groups, group H with high (>30 mm Hg) mean pulmonary arterial pressure and group L with low (25 to 30 mm Hg) mean pulmonary arterial pressure. RV function was assessed using 2-dimensional speckle-tracking echocardiography as well as 3-dimensional echocardiography, and RV dyssynchrony was assessed by the RV strain curves. Exercise capacity was evaluated by the 6-minute walk distance. RV dilatation was significantly reduced after BPA. In group H, RV ejection fraction, RV free wall longitudinal strain and RV dyssynchrony were all impaired before BPA and were ameliorated after BPA. In group L, RV ejection fraction as well as RV dyssynchrony were impaired without the reduction of RV free wall longitudinal strain and were improved after BPA, indicating that RV dysfunction may be attributable to the RV dyssynchrony in group L. Furthermore, RV dyssynchrony at baseline was the only parameter that was correlated with improvement in the 6-minute walk distance after BPA. RV dyssynchrony may affect RV function and could be the useful parameter for clinical outcome after BPA., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Balloon pulmonary angioplasty (BPA) is an alternative therapy for patients with chronic thromboembolic pulmonary hypertension who are ineligible for standard therapy, pulmonary endarterectomy. Although there are several classifications of vascular lesions, these classifications are based on the features of the specimen removed during pulmonary endarterectomy. Because organized thrombi are not removed during balloon pulmonary angioplasty, we attempted to establish a new classification of vascular lesions based on pulmonary angiographic images. We evaluated the success and complication rate of BPA in accordance with the location and morphology of thromboembolic lesions., Methods and Results: We reviewed 500 consecutive procedures (1936 lesions) of BPA in 97 patients with chronic thromboembolic pulmonary hypertension and investigated the outcomes of BPA based on the lesion distribution and the angiographic characteristics of the thromboembolic lesions, as follows: type A, ring-like stenosis lesion; type B, web lesion; type C, subtotal lesion; type D, total occlusion lesion, and type E, tortuous lesion. The success rate was higher, and the complication rate was lower in ring-like stenosis and web lesions. The total occlusion lesions had the lowest success rate. Tortuous lesions were associated with a high complication rate and should be treated only by operators with extensive experience with BPA., Conclusions: We modified the previous angiographic classification and established a new classification for each vascular lesion. We clarified that the outcome and complication rate of the BPA are highly dependent on the lesion characteristics., (© 2016 American Heart Association, Inc.)