記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.jcin.2016.08.022.

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.ijcard.2015.06.075.

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.ijcard.2015.04.025.

記述言語：英語  

DOI： 10.1111/jce.16752

PubMed

記述言語：英語  

DOI： 10.1186/s12876-025-03965-1

PubMed

記述言語：英語  

DOI： 10.1111/jce.16626

PubMed

記述言語：英語  

DOI： 10.1111/pin.70017

PubMed

記述言語：英語  

DOI： 10.1016/j.hrthm.2025.04.020

PubMed

記述言語：英語  

DOI： 10.1016/j.jjcc.2025.04.002

PubMed

記述言語：英語  

DOI： 10.1007/s12928-025-01122-9

PubMed

記述言語：英語  

DOI： 10.1093/ehjcr/ytaf196

PubMed

記述言語：英語  

DOI： 10.1016/j.jjcc.2025.03.006

PubMed

記述言語：英語  

DOI： 10.1038/s41598-025-92933-6

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: The management of chronic thromboembolic pulmonary hypertension (CTEPH) has advanced significantly in recent years, thereby improving patient prognosis. However, the impact of cancer on the outcomes of patients with CTEPH under current treatment remains unclear. This study aimed to investigate the prevalence of cancer in patients with CTEPH and determine how comorbid cancer affects their prognosis and clinical course. METHODS: Data from an ongoing Japanese prospective cohort study were analyzed. Prevalence and primary cancer sites were evaluated. The association of a history of cancer with a composite endpoint, including all-cause death, lung transplantation, and worsening of CTEPH, as well as venous thromboembolism and bleeding events, was assessed. RESULTS: Of the 1,270 patients in the cohort, 134 (10.6%) had a history of cancer, with the most common primary sites being the breast in women and the prostate in men. The incidence of composite outcome and all-cause death was higher in those with a history of cancer (p < 0.001, log-rank test). In the Cox proportional hazard model, age- and sex-adjusted hazard ratios for the composite outcome and all-cause death were 2.69 (95% confidence interval, 1.48-4.89, p = 0.001) and 4.25 (95% confidence interval, 1.98-9.10, p < 0.001), respectively, for patients with a history of cancer. No significant differences in venous thromboembolism and bleeding events were observed between patients with and those without a history of cancer. CONCLUSIONS: A history of cancer, with a prevalence of 10.6%, is an independent risk factor for mortality in patients with CTEPH undergoing the currently recommended treatment.

DOI： 10.1016/j.healun.2024.10.022

PubMed

記述言語：英語  

DOI： 10.1038/s41440-024-02025-7

PubMed

記述言語：英語   出版者・発行元：Nature Publishing Group  

記述言語：英語  

DOI： 10.1038/s41598-025-87650-z

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Intensive care unit-acquired weakness (ICU-AW) is recognized as newly-acquired bilateral muscle weakness, which is a complication of critical illness in the ICU; however, there are no reports on the pathogenesis and early predictors of ICU-AW specifically associated with cardiogenic shock (CS). Therefore, this study aimed to investigate the clinical characteristics of ICU-AW in patients with CS requiring mechanical circulatory support (MCS). This study was a single-center, prospective, and observational study. Patients aged 16 years and older who underwent MCS for CS were included. ICU-AW was diagnosed based on Medical Research Council (MRC) score after awakening. The ICU-AW group included patients with the MRC score < 48 points, and the non-ICU-AW group included those with ≥ 48 points. Twenty-eight cases were enrolled on admission and MRC score was evaluated in 23 cases after awakening. Eleven patients were included in the non-ICU-AW group and 12 patients (52%) were in the ICU-AW group. The ICU-AW group showed a higher prevalence of extracorporeal membrane oxygenation and ventilator use. Creatine kinase, troponin T, interleukin (IL)-15 levels on admission were significantly higher, whereas hemoglobin and albumin levels were significantly lower in the ICU-AW group. A strong negative correlation was observed between the initial MRC scores and IL-15 levels. ICU-AW occurred 52% of patients with CS using MCS, indicating the significance of recognizing and managing this complication for those patients. In addition, IL-15 can be a potential biomarker for the early prediction of ICU-AW.

DOI： 10.1038/s41598-025-87381-1

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Advances in chronic thromboembolic pulmonary hypertension (CTEPH) treatment have improved prognosis, shifting focus towards symptom management. This study aimed to identify factors influencing the World Health Organization functional class (WHO-FC) in CTEPH patients. The CTEPH AC registry is a prospective, multicenter database from 35 Japanese institutions, analyzing data from August 2018 to July 2023. We examined factors associated with achieving WHO-FC I and WHO-FC changes over time in 1,270 patients. Significant factors for WHO-FC I achievement included male sex (odds ratio: 1.86, p = 0.019), age (0.98, p = 0.007), pulmonary vasodilator use (0.51, p = 0.001), post-balloon pulmonary angioplasty (BPA) (1.93, p = 0.010), lower mean pulmonary arterial pressure (0.94, p = 0.004), and lower pulmonary vascular resistance (PVR) (0.78, p = 0.006). Multivariate analysis showed that WHO-FC improvement correlated with male sex, baseline PVR, and BPA during follow-up. WHO-FC deterioration was associated with cancer, history of pulmonary endarterectomy and/or BPA at registration, bleeding risks, and thyroid disease or hormone therapy. BPA implementation is closely linked to symptomatic improvement and achieving WHO-FC I, while symptom worsening is often associated with patient-specific, difficult-to-control conditions.

DOI： 10.1007/s12928-025-01095-9

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

AIMS: Amyloid deposition in myocardial tissue is a definitive feature for diagnosing cardiac amyloidosis, though less invasive imaging modalities such as bone tracer cardiac scintigraphy and cardiac magnetic resonance imaging have been established as first steps for its diagnosis. This study aimed to develop a deep learning model to support the diagnosis of cardiac amyloidosis from haematoxylin/eosin (HE)-stained myocardial tissue. METHODS AND RESULTS: This single-centre retrospective observational study enrolled 166 patients who underwent myocardial biopsies between 2008 and 2022, including 76 patients diagnosed with cardiac amyloidosis and 90 with other diagnoses. A deep learning model was developed to output the probabilities of cardiac amyloidosis for all the small patches cutout from each myocardial specimen. The developed model highlighted the area in the stained images as highly suspicious, corresponding to where Dylon staining marked amyloid deposition, and discriminated the patches in the evaluation dataset with an area under the curve of 0.965. Provided that the diagnostic criterion for cardiac amyloidosis was defined as a median probability of cardiac amyloidosis >50% in all patches, the model achieved perfect performance in discriminating patients with cardiac amyloidosis from those without it, with an area under the curve of 1.0. CONCLUSION: A deep learning model was developed to diagnose cardiac amyloidosis from HE-stained myocardial tissue accurately. Although further prospective validation of this model using HE-stained myocardial tissues from multiple centres is needed, it may help minimize the risk of missing cardiac amyloidosis and maximize the utility of histological diagnosis in clinical practice.

DOI： 10.1093/ehjimp/qyae141

PubMed

記述言語：英語   出版者・発行元：一般社団法人 日本内科学会  

<p>A 67-year-old man on intense immunosuppressive therapy after heart transplantation for end-stage hypertrophic cardiomyopathy 7 years ago developed severe dyspnea and was admitted to our hospital. His serum SARS-CoV-2 antigen test was positive, and he was diagnosed with COVID-19-related pneumonia. He was started on ventilatory management for severe respiratory failure and remdesivir for COVID-19, with careful adjustment of immunosuppressive drugs. However, unexpectedly prolonged muscle weakness necessitated transfer to a rehabilitation facility. Although the COVID-19 pandemic has subsided, it is still considered a risk in post-transplant cases. Infection control is considered critical in heart transplant recipients, especially in those receiving intensified immunosuppressive drugs. </p>

DOI： 10.2169/internalmedicine.5254-25

PubMed

CiNii Research

記述言語：英語   出版者・発行元：Elsevier  

Purpose / This study aimed to assess the diagnostic performance of dynamic chest radiography (DCR) and investigate its added value to chest radiography (CR) in detecting pulmonary embolism (PE). / Methods / Of 775 patients who underwent CR and DCR in our hospital between June 2020 and August 2022, individuals who also underwent contrast-enhanced CT (CECT) of the chest within 72 h were included in this study. PE or non-PE diagnosis was confirmed by CECT and the subsequent clinical course. The enrolled patients were randomized into two groups. Six observers, including two thoracic radiologists, two cardiologists, and two radiology residents, interpreted each chest radiograph with and without DCR using a crossover design with a washout period. Diagnostic performance was compared between CR with and without DCR in the standing and supine positions. / Results / Sixty patients (15 PE, 45 non-PE) were retrospectively enrolled. The addition of DCR to CR significantly improved the sensitivity, specificity, accuracy, and area under the curve (AUC) in the standing (35.6–70.0 % [P < 0.0001], 84.8–93.3 % [P = 0.0010], 72.5–87.5 % [P < 0.0001], and 0.66–0.85 [P < 0.0001], respectively) and supine (33.3–65.6 % [P < 0.0001], 78.5–92.2 % [P < 0.0001], 67.2–85.6 % [P < 0.0001], and 0.62–0.80 [P = 0.0002], respectively) positions for PE detection. No significant differences were found between the AUC values of DCR with CR in the standing and supine positions (P = 0.11) or among radiologists, cardiologists, and radiology residents (P = 0.14–0.68). / Conclusions / Incorporating DCR with CR demonstrated moderate sensitivity, high specificity, and high accuracy in detecting PE, all of which were significantly higher than those achieved with CR alone, regardless of scan position, observer expertise, or experience.

CiNii Research

記述言語：英語   出版者・発行元：(一社)日本循環器学会  

記述言語：英語   出版者・発行元：一般社団法人 インターナショナル・ハート・ジャーナル刊行会  

<p>An 18-year-old man experienced refractory cardiogenic shock due to dilated cardiomyopathy of unknown aetiology, and was referred to our hospital to undergo a paracorporeal left ventricular assist device (LVAD) implantation. After surgery, the patient lost consciousness, with subsequent head computed tomography revealing low-density areas in the bilateral basal ganglia. Metabolic acidosis and hyperammonaemia also appeared. Metabolic evaluation and genetic tests led to a diagnosis of propionic acidemia. Following appropriate management of heart failure and propionic acidemia, his neurological and cardiac functions gradually recovered, and the paracorporeal LVAD was successfully explanted. Dilated cardiomyopathy and heart failure are rare manifestations of adult-onset propionic acidemia. This is the first reported case of cardiogenic shock due to propionic acidemia-associated cardiomyopathy successfully treated with a combination of mechanical circulatory support and medical therapy for heart failure and propionic acidemia.</p>

DOI： 10.1536/ihj.24-364

PubMed

CiNii Research

記述言語：英語  

DOI： 10.1186/s40959-024-00288-0

PubMed

記述言語：英語   出版者・発行元：International Journal of Cardiology  

Backgrounds: A soluble guanylyl cyclase stimulator vericiguat has been shown to reduce cardiovascular mortality or hospitalization for heart failure in patients with worsening heart failure in the VICTORIA study. However, little is known about the effects of vericiguat on biventricular structure and function. Methods and results: A retrospective analysis of 63 consecutive patients with heart failure with reduced ejection fraction (HFrEF) who were treated with vericiguat was performed. Clinical data and echocardiographic parameters were compared between baseline and follow-up after the initiation of vericiguat. The median follow-up duration was 266 days. Treatment with vericiguat significantly reduced the plasma BNP levels (log-transformed) compared to baseline (2.46 ± 0.51 vs. 2.14 ± 0.58, p < 0.0001). Left ventricular end-diastolic volume index and left ventricular end-systolic volume index were significantly reduced (LVEDVI, 113.5 ± 46.3 vs. 103.6 ± 51.0, p = 0.0056; LVESVI, 82.0 ± 41.9 vs. 72.8 ± 44.7, p = 0.0077; respectively). The tricuspid annular plane systolic excursion to pulmonary artery systolic pressure (TAPSE/PASP) ratio, an indicator of right ventricle-pulmonary artery (RV-PA) coupling, increased significantly after the treatment (0.56 ± 0.29 vs. 0.92 ± 1.09, p < 0.0001). Univariate and multivariate analyses showed that the treatment effects of vericiguat on BNP levels, LV reverse remodeling, and RV-PA coupling were not correlated with the achievement of the quadruple therapy with beta-blockers, renin-angiotensin system inhibitors, mineralocorticoid inhibitors, and sodium-glucose cotransporter-2 inhibitors, nor with worsening heart failure (WHF). Conclusion: Treatment with vericiguat improved adverse LV remodeling and RV-PA uncoupling in HFrEF patients. These effects were independent of WHF and achieving the quadruple therapy. Patients with HFrEF may benefit from early initiation of vericiguat to prevent biventricular adverse remodeling.

DOI： 10.1016/j.ijcard.2024.132441

Web of Science

Scopus

PubMed

記述言語：英語  

DOI： 10.1038/s41598-024-75328-x

PubMed

記述言語：英語   出版者・発行元：(一社)インターナショナルハートジャーナル刊行会  

症例は18歳男性で、精神運動発達遅滞の既往があった。労作時呼吸困難を主訴に近医を受診し、急性非代償性心不全と診断された。心不全はうっ血および低灌流所見を認めるwet-cold型であった。強心剤と利尿剤が投与されたが症状が改善せず、心原性ショックを呈したため、当院に搬送された。搬送時の血圧は84/70mmHg、心拍数は134bpmであった。胸部X線で肺うっ血を認め、心胸比は69%であった。心エコー検査で左室拡張末期径は69mmで、左室駆出率は16%であった。心臓カテーテル検査で肺動脈楔入圧は28mmHg、心係数は1.8L/min/m2であった。心内膜心筋生検で心筋症を認めた。難治性心原性ショックに対して植込み型補助人工心臓(LVAD)の植込み術を施行した。4日後に意識消失し、代謝性アシドーシスと高アンモニア血症を認めた。タンデムマス法でプロピオニルカルニチン(C3)の増加を認め、遺伝学的検査でPCCA遺伝子変異が検出された。プロピオン酸血症と診断し、栄養療法とカルベジロールを含む薬物療法を開始した。心機能は徐々に改善し、118日後にLVADから離脱した。左室駆出率は45%に増加し、173日後に退院となった。

記述言語：英語  

DOI： 10.1253/circj.CJ-24-0402

PubMed

記述言語：英語  

DOI： 10.1186/s40792-024-02014-5

PubMed

記述言語：英語  

DOI： 10.1210/clinem/dgae098

PubMed

記述言語：英語   出版者・発行元：JACC: Asia  

Background: The contemporary outcome of balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) are unclear. Objectives: This study aimed to clarify the characteristics and outcomes of CTEPH patients treated with BPA and PEA in Japan. Methods: Among 1,270 participants enrolled between 2018 and 2023 in the CTEPH AC (Chronic Thromboembolic Pulmonary Hypertension Anticoagulant) registry, a Japanese nationwide CTEPH registry, 369 treatment-naive patients (BPA strategy: n = 313; PEA strategy: n = 56) and 690 on-treatment patients (BPA strategy: n = 561; PEA strategy: n = 129) were classified according to the presence of prior reperfusion therapy. Morbidity and mortality events (all-cause death, rescue mechanical reperfusion therapy, and/or initiation of parenteral pulmonary vasodilators), pulmonary hemodynamics, exercise tolerance, and relevant laboratory test results were evaluated. Results: The BPA strategy was chosen in older patients than the PEA strategy (mean age, BPA vs PEA: 66.5 ± 12.6 years vs 62.5 ± 11.8 years; P = 0.028). Median follow-up period was 615 (Q1-Q3: 311-997) days in treatment-naive patients and 1,136 (Q1-Q3: 684-1,300) days in on-treatment patients. BPA strategy had as acceptable morbidity and mortality as PEA strategy (5-year morbidity and mortality event rate, BPA vs PEA: 10.2% [95% CI: 5.2%-19.5%] vs 16.1% [95% CI: 4.3%-50.6%] in treatment-naive patients; 9.7% [95% CI: 6.7%-13.8%] vs 6.9% [95% CI: 2.7%-17.3%] in on-treatment patients), with greater improvement of renal function; glomerular filtration rate in propensity score-matched population (difference between change: 4.9 [95% CI: 0.5-9.3] mL/min/1.73 m2; P = 0.030). Conclusions: BPA strategy was more frequently chosen in older patients compared with PEA strategy and showed acceptable outcomes for efficacy with greater advantage for improvement in renal function. (Multicenter registry of chronic thromboembolic pulmonary hypertension in Japan; UMIN000033784)

DOI： 10.1016/j.jacasi.2024.05.007

Scopus

PubMed

出版者・発行元：医歯薬出版  

DOI： 10.32118/ayu290020178

CiNii Research

記述言語：英語  

DOI： 10.1126/sciadv.adi1621

PubMed

記述言語：英語   出版者・発行元：Pharmaceuticals  

Pulmonary arterial hypertension (PAH) remains a significant challenge in cardiology, necessitating advancements in treatment strategies. This study explores the safety and efficacy of transitioning patients from beraprost to selexipag, a novel selective prostacyclin receptor agonist, within a Japanese cohort. Employing a multicenter, open-label, prospective design, 25 PAH patients inadequately managed on beraprost were switched to selexipag. Key inclusion criteria included ongoing beraprost therapy for ≥3 months, a diagnosis of PAH confirmed by mean pulmonary artery pressure (mPAP) ≥ 25 mmHg, and current treatment with endothelin receptor antagonists and/or phosphodiesterase type 5 inhibitors. Outcomes assessed were changes in hemodynamic parameters (mPAP, cardiac index, pulmonary vascular resistance) and the 6 min walk distance (6-MWD) over 3–6 months. The study found no statistically significant changes in these parameters post-switch. However, a subset of patients, defined as responders, demonstrated improvements in all measured hemodynamic parameters, suggesting a potential benefit in carefully selected patients. The transition was generally well-tolerated with no serious adverse events reported. This investigation underscores the importance of personalized treatment strategies in PAH, highlighting that certain patients may benefit from switching to selexipag, particularly those previously on higher doses of beraprost. Further research is needed to elucidate the predictors of positive response to selexipag and optimize treatment regimens for this complex condition.

DOI： 10.3390/ph17050555

Web of Science

Scopus

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Pulmonary arterial hypertension (PAH) remains a significant challenge in cardiology, necessitating advancements in treatment strategies. This study explores the safety and efficacy of transitioning patients from beraprost to selexipag, a novel selective prostacyclin receptor agonist, within a Japanese cohort. Employing a multicenter, open-label, prospective design, 25 PAH patients inadequately managed on beraprost were switched to selexipag. Key inclusion criteria included ongoing beraprost therapy for ≥3 months, a diagnosis of PAH confirmed by mean pulmonary artery pressure (mPAP) ≥ 25 mmHg, and current treatment with endothelin receptor antagonists and/or phosphodiesterase type 5 inhibitors. Outcomes assessed were changes in hemodynamic parameters (mPAP, cardiac index, pulmonary vascular resistance) and the 6 min walk distance (6-MWD) over 3-6 months. The study found no statistically significant changes in these parameters post-switch. However, a subset of patients, defined as responders, demonstrated improvements in all measured hemodynamic parameters, suggesting a potential benefit in carefully selected patients. The transition was generally well-tolerated with no serious adverse events reported. This investigation underscores the importance of personalized treatment strategies in PAH, highlighting that certain patients may benefit from switching to selexipag, particularly those previously on higher doses of beraprost. Further research is needed to elucidate the predictors of positive response to selexipag and optimize treatment regimens for this complex condition.

記述言語：英語   出版者・発行元：Circulation Journal  

The 87th Annual Meeting of the Japanese Circulation Society (JCS2023) was held in March 2023 in Fukuoka, Japan, marking the first in-person gathering after the COVID-19 pandemic. With the theme of “New Challenge With Next Generation” the conference emphasized the development of future cardiovascular leaders and technologies such as artificial intelligence (AI). Notable sessions included the Mikamo Lecture on heart failure and the Mashimo Lecture on AI in medicine. Various hands-on sessions and participatory events were well received, promoting learning and networking. Post-event surveys showed high satisfaction among participants, with positive feedback on face-to-face interactions and the overall experience. JCS2023, attended by 17,852 participants, concluded successfully, marking a significant milestone in post-pandemic meetings, and advancing cardiovascular medicine.

DOI： 10.1253/circj.CJ-24-0127

Web of Science

Scopus

PubMed

記述言語：英語   出版者・発行元：Pulmonary Circulation  

Advances in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) over the past decade changed the disease landscape, yet global insight on clinical practices remains limited. The CTEPH global cross-sectional scientific survey (CLARITY) aimed to gather information on the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the treatment and management of CTEPH patients. The survey was circulated to hospital-based medical specialists through Scientific Societies and other medical organizations from September 2021 to May 2022. The majority of the 212 respondents involved in the treatment of CTEPH were from centers performing up to 50 pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA) procedures per year. Variation was observed in the reported proportion of patients deemed eligible for PEA/BPA, as well as those that underwent the procedures, including multimodal treatment and subsequent follow-up practices. Prescription of pulmonary arterial hypertension-specific therapy was reported for a variable proportion of patients in the preoperative setting and in most nonoperable patients. Reported use of vitamin K antagonists and direct oral anticoagulants was similar (86% vs. 82%) but driven by different factors. This study presents heterogeneity in treatment approaches for CTEPH, which may be attributed to center-specific experience and region-specific barriers to care, highlighting the need for new clinical and cohort studies, comprehensive clinical guidelines, and continued education.

DOI： 10.1002/pul2.12406

Web of Science

Scopus

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   出版者・発行元：Japanese Journal of Radiology  

Dynamic chest radiography (DCR) is a novel functional radiographic imaging technique that can be used to visualize pulmonary perfusion without using contrast media. Although it has many advantages and clinical utility, most radiologists are unfamiliar with this technique because of its novelty. This review aims to (1) explain the basic principles of lung perfusion assessment using DCR, (2) discuss the advantages of DCR over other imaging modalities, and (3) review multiple specific clinical applications of DCR for pulmonary vascular diseases and compare them with other imaging modalities.

DOI： 10.1007/s11604-023-01483-2

Web of Science

Scopus

PubMed

記述言語：英語   出版者・発行元：Circulation  

DOI： 10.1161/CIRCULATIONAHA.123.067528

Web of Science

Scopus

PubMed

記述言語：英語  

DOI： 10.1161/JAHA.123.031219

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   出版者・発行元：Pulmonary Circulation  

Early recognition and diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is crucial for improving prognosis and reducing the disease burden. Established clinical practice guidelines describe interventions for the diagnosis and evaluation of CTEPH, yet limited insight remains into clinical practice variation and barriers to care. The CTEPH global cross-sectional scientific survey (CLARITY) was developed to gather insights into the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the recognition and diagnosis of CTEPH and the referral and evaluation of these patients. The survey was offered to hospital-based medical specialists through Scientific Societies and other medical organizations, from September 2021 to May 2022. Response data from 353 physicians showed that self-reported awareness of CTEPH increased over the past 10 years among 96% of respondents. Clinical practices in acute pulmonary embolism (PE) follow-up and CTEPH diagnosis differed among respondents. While 50% of respondents working in a nonexpert center reported to refer patients to an expert pulmonary hypertension/CTEPH center when CTEPH is suspected, 51% of these physicians did not report referral of patients with a confirmed diagnosis for further evaluation. Up to 50% of respondents involved in the evaluation of referred patients have concluded a different operability status than that indicated by the referring center. This study indicates that early diagnosis and timely treatment of CTEPH is challenged by suboptimal acute PE follow-up and patient referral practices. Nonadherence to guideline recommendations may be impacted by various barriers to care, which were shown to vary by geographical region.

DOI： 10.1002/pul2.12330

Web of Science

Scopus

PubMed

Web of Science

記述言語：英語  

DOI： 10.1038/s41598-023-44877-y

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

The prevalence of patients with hyperuricemia or gout is increasing worldwide. Hyperuricemia and gout are primarily attributed to genetic factors, along with lifestyle factors like consuming a purine-rich diet, alcohol and/or fructose intake, and physical activity. While numerous studies have reported various comorbidities linked to hyperuricemia or gout, the range of these associations is extensive. This review article focuses on the relationship between uric acid and thirteen specific domains: transporters, genetic factors, diet, lifestyle, gout, diabetes mellitus, metabolic syndrome, atherosclerosis, hypertension, kidney diseases, cardiovascular diseases, neurological diseases, and malignancies. The present article provides a comprehensive review of recent developments in these areas, compiled by experts from the Young Committee of the Japanese Society of Gout and Uric and Nucleic Acids. The consolidated summary serves to enhance the global comprehension of uric acid-related matters.

記述言語：英語   出版者・発行元：Biomolecules  

The prevalence of patients with hyperuricemia or gout is increasing worldwide. Hyperuricemia and gout are primarily attributed to genetic factors, along with lifestyle factors like consuming a purine-rich diet, alcohol and/or fructose intake, and physical activity. While numerous studies have reported various comorbidities linked to hyperuricemia or gout, the range of these associations is extensive. This review article focuses on the relationship between uric acid and thirteen specific domains: transporters, genetic factors, diet, lifestyle, gout, diabetes mellitus, metabolic syndrome, atherosclerosis, hypertension, kidney diseases, cardiovascular diseases, neurological diseases, and malignancies. The present article provides a comprehensive review of recent developments in these areas, compiled by experts from the Young Committee of the Japanese Society of Gout and Uric and Nucleic Acids. The consolidated summary serves to enhance the global comprehension of uric acid-related matters.

DOI： 10.3390/biom13101519

Web of Science

Scopus

PubMed

記述言語：英語  

DOI： 10.1161/CIRCHEARTFAILURE.122.010347

PubMed

記述言語：英語   出版者・発行元：Japan Radiological Society  

Dynamic chest radiography (DCR) is a novel functional radiographic imaging technique that can be used to visualize pulmonary perfusion without using contrast media. Although it has many advantages and clinical utility, most radiologists are unfamiliar with this technique because of its novelty. This review aims to (1) explain the basic principles of lung perfusion assessment using DCR, (2) discuss the advantages of DCR over other imaging modalities, and (3) review multiple specific clinical applications of DCR for pulmonary vascular diseases and compare them with other imaging modalities.

CiNii Research

記述言語：英語   出版者・発行元：Journal of Thrombosis and Haemostasis  

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) requires lifelong anticoagulation. Long-term outcomes of CTEPH under current anticoagulants are unclear. Objectives: The CTEPH AC registry is a prospective, nationwide cohort study comparing the safety and effectiveness of direct oral anticoagulants (DOACs) and warfarin for CTEPH. Patients/Methods: Patients with CTEPH, both tre atment-naïve and on treatment, were eligible for the registry. Inclusion criteria were patients aged ≥20 years and those who were diagnosed with CTEPH according to standard guidelines. Exclusion criteria were not specified. The primary efficacy outcome was a composite morbidity, and mortality outcome comprised all-cause death, rescue reperfusion therapy, initiation of parenteral pulmonary vasodilators, and worsened 6-minute walk distance and WHO functional class. The safety outcome was clinically relevant bleeding, including major bleeding. Results: Nine hundred twenty-seven patients on oral anticoagulants at baseline were analyzed: 481 (52%) used DOACs and 446 (48%) used warfarin. The 1-, 2-, and 3-year rates of composite morbidity and mortality outcome were comparable between the DOAC and warfarin groups (2.6%, 3.1%, and 4.2% vs 3.0%, 4.8%, and 5.9%, respectively; P = .52). The 1-, 2-, and 3-year rates of clinically relevant bleeding were significantly lower in DOACs than in the warfarin group (0.8%, 2.4%, and 2.4% vs 2.5%, 4.8%, and 6.4%, respectively; P = 0.036). Multivariable Cox proportional-hazards regression models revealed lower risk of clinically relevant bleeding in the DOAC group than the warfarin group (hazard ratio: 0.35; 95% CI: 0.13-0.91; P = .032). Conclusion: This registry demonstrated that under current standard of care, morbidity and mortality events were effectively prevented regardless of anticoagulants, while the clinically relevant bleeding rate was lower when using DOACs compared with warfarin.

DOI： 10.1016/j.jtha.2023.03.036

Web of Science

Scopus

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   出版者・発行元：Interventional Cardiology Clinics  

DOI： 10.1016/j.iccl.2023.02.003

Scopus

PubMed

記述言語：英語  

DOI： 10.3390/nu15122731

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Pulmonary regurgitation (PR) is a risk factor for sudden cardiac death in adult patients with repaired tetralogy of Fallot (TOF). However, transthoracic echocardiography (TTE) cannot fully visualize the pulmonary valve (PV) and PR. We investigated whether intracardiac echocardiography (ICE) could visualize the PV and PR better than TTE. Thirty adult patients with TOF (mean age 33 ± 15 years) scheduled for cardiac catheterization underwent ICE. The visualization of PV and the severity of PR were classified into three grades. ICE depicted the PV better than TTE (ICE vs. TTE: not visualized, partially visualized, and fully visualized: n = 1 [3%], n = 13 [43%], and n = 16 [53%] vs. n = 14 [47%], n = 13 [43%], and n = 3 [10%], p < 0.001). Especially in patients after pulmonary valve replacement (PVR), the PV was more fully visualized by ICE. The assessment of PR by TTE underestimated the severity of PR in comparison to cardiac magnetic resonance imaging (MRI) (severe PR: 8 [28%] vs. 22 [76%], p = 0.004), while there was no discrepancy between the results of ICE and MRI (21 [72%] vs. 22 [76%], p = 1.000). In comparison to TTE, ICE can safely provide better visualization of the PV and PR in adults with TOF, especially in patients who have undergone PVR.

Web of Science

記述言語：英語  

DOI： 10.1016/j.eclinm.2023.101920

PubMed

記述言語：英語   出版者・発行元：Circulation Research  

Background: Mitochondrial DNA (mtDNA)-induced myocardial inflammation is intimately involved in cardiac remodeling. ZBP1 (Z-DNA binding protein 1) is a pattern recognition receptor positively regulating inflammation in response to mtDNA in inflammatory cells, fibroblasts, and endothelial cells. However, the role of ZBP1 in myocardial inflammation and cardiac remodeling remains unclear. The aim of this study was to elucidate the role of ZBP1 in mtDNA-induced inflammation in cardiomyocytes and failing hearts. Methods: mtDNA was administrated into isolated cardiomyocytes. Myocardial infarctionwas conducted in wild type and ZBP1 knockout mice. Results: We here found that, unlike in macrophages, ZBP1 knockdown unexpectedly exacerbated mtDNA-induced inflammation such as increases in IL (interleukin)-1β and IL-6, accompanied by increases in RIPK3 (receptor interacting protein kinase 3), phosphorylated NF-κB (nuclear factor-κB), and NLRP3 (nucleotide-binding domain and leucine-rich-repeat family pyrin domain containing 3) in cardiomyocytes. RIPK3 knockdown canceled further increases in phosphorylated NF-κB, NLRP3, IL-1β, and IL-6 by ZBP1 knockdown in cardiomyocytes in response to mtDNA. Furthermore, NF-κB knockdown suppressed such increases in NLRP3, IL-1β, and IL-6 by ZBP1 knockdown in response to mtDNA. CpG-oligodeoxynucleotide, a Toll-like receptor 9 stimulator, increased RIPK3, IL-1β, and IL-6 and ZBP1 knockdown exacerbated them. Dloop, a component of mtDNA, but not Tert and B2m, components of nuclear DNA, was increased in cytosolic fraction from noninfarcted region of mouse hearts after myocardial infarction compared with control hearts. Consistent with this change, ZBP1, RIPK3, phosphorylated NF-κB, NLRP3, IL-1β, and IL-6 were increased in failing hearts. ZBP1 knockout mice exacerbated left ventricular dilatation and dysfunction after myocardial infarction, accompanied by further increases in RIPK3, phosphorylated NF-κB, NLRP3, IL-1β, and IL-6. In histological analysis, ZBP1 knockout increased interstitial fibrosis and myocardial apoptosis in failing hearts. Conclusions: Our study reveals unexpected protective roles of ZBP1 against cardiac remodeling as an endogenous suppressor of mtDNA-induced myocardial inflammation.

DOI： 10.1161/CIRCRESAHA.122.322227

Web of Science

Scopus

PubMed

記述言語：英語   出版者・発行元：Pulmonary Circulation  

Pulmonary arterial hypertension (PAH), an intractable disease with a poor prognosis, is commonly treated using pulmonary vasodilators modulating the endothelin, cGMP, and prostacyclin pathway. Since the 2010s, drugs for treating pulmonary hypertension based on mechanisms other than pulmonary vasodilation have been actively developed. However, precision medicine is based on tailoring disease treatment to particular phenotypes by molecular-targeted drugs. Since interleukin-6 (IL-6) is involved in the development of PAH in animal models, and some patients with PAH have elevated IL-6 levels, the cytokine is expected to obtain potentials for therapeutic targeting. Accordingly, we identified a phenotype with elevated cytokine activity of the IL-6 family in the PAH population by combining case data extracted from the Japan Pulmonary Hypertension Registry with a comprehensive analysis of 48 cytokines using artificial intelligence clustering techniques. Including an IL-6 threshold ≥2.73 pg/mL as inclusion criteria for reducing the risk of insufficient efficacy, an investigator-initiated clinical study using satralizumab, a recycling anti-IL6 receptor monoclonal antibody, for patients with an immune-responsive phenotype is underway. This study is intended to test whether use of patient biomarker profile can identify a phenotype responsive to anti-IL6 therapy.

DOI： 10.1002/pul2.12251

Web of Science

Scopus

PubMed

Web of Science

記述言語：英語   出版者・発行元：Radiology  

Background: While current guidelines require lung ventilation-perfusion (V/Q) scanning as the first step to diagnose chronic pulmonary embolism in pulmonary hypertension (PH), its use may be limited by low availability and/or exposure to ionizing radiation. Purpose: To compare the performance of dynamic chest radiography (DCR) and lung V/Q scanning for detection of chronic thromboembolic PH (CTEPH). Materials and Methods: Patients with PH who underwent DCR and V/Q scanning in the supine position from December 2019 to July 2021 were retrospectively screened. The diagnosis of CTEPH was confirmed with right heart catheterization and invasive pulmonary angiography. Observer tests were conducted to evaluate the diagnostic accuracy of DCR and V/Q scanning. The lungs were divided into six areas (upper, middle, and lower for both) in the anteroposterior image, and the number of lung areas with thromboembolic perfusion defects was scored. Diagnostic performance was compared between DCR and V/Q scanning using the area under the receiver operating characteristic curve. Agreement between the interpretation of DCR and that of V/Q scanning was assessed using the Cohen kappa coefficient and percent agreement. Results: A total of 50 patients with PH were analyzed: 29 with CTEPH (mean age, 64 years ± 15 [SD]; 19 women) and 21 without CTEPH (mean age, 61 years ± 22; 14 women). The sensitivity, specificity, and accuracy of DCR were 97%, 86%, and 92%, respectively, and those of V/Q scanning were 100%, 86%, and 94%, respectively. Areas under the receiver operating characteristic curve for DCR and V/Q scanning were 0.92 (95% CI: 0.79, 0.97) and 0.93 (95% CI: 0.78, 0.98). Agreement between the consensus interpretation of DCR and that of V/Q scanning was substantial (κ = 0.79 [95% CI: 0.61, 0.96], percent agreement = 0.9 [95% CI: 0.79, 0.95]). Conclusion: Dynamic chest radiography had similar efficacy to ventilation-perfusion scanning in the detection of chronic thromboembolic pulmonary hypertension.

DOI： 10.1148/radiol.220908

Web of Science

Scopus

PubMed

記述言語：英語   出版者・発行元：Journal of Cardiovascular Development and Disease  

Pulmonary regurgitation (PR) is a risk factor for sudden cardiac death in adult patients with repaired tetralogy of Fallot (TOF). However, transthoracic echocardiography (TTE) cannot fully visualize the pulmonary valve (PV) and PR. We investigated whether intracardiac echocardiography (ICE) could visualize the PV and PR better than TTE. Thirty adult patients with TOF (mean age 33 ± 15 years) scheduled for cardiac catheterization underwent ICE. The visualization of PV and the severity of PR were classified into three grades. ICE depicted the PV better than TTE (ICE vs. TTE: not visualized, partially visualized, and fully visualized: n = 1 [3%], n = 13 [43%], and n = 16 [53%] vs. n = 14 [47%], n = 13 [43%], and n = 3 [10%], p < 0.001). Especially in patients after pulmonary valve replacement (PVR), the PV was more fully visualized by ICE. The assessment of PR by TTE underestimated the severity of PR in comparison to cardiac magnetic resonance imaging (MRI) (severe PR: 8 [28%] vs. 22 [76%], p = 0.004), while there was no discrepancy between the results of ICE and MRI (21 [72%] vs. 22 [76%], p = 1.000). In comparison to TTE, ICE can safely provide better visualization of the PV and PR in adults with TOF, especially in patients who have undergone PVR.

DOI： 10.3390/jcdd10010024

Web of Science

Scopus

PubMed

記述言語：英語   出版者・発行元：Pulmonary Circulation  

Treprostinil is a chemically stable analog of prostacyclin, and inhaled treprostinil was developed to deliver the effects directly to the pulmonary vasculature while minimizing systemic side effects. The objective of the study was to evaluate the efficacy on hemodynamics and exercise capacity, safety, and pharmacokinetics (PK) of inhaled treprostinil in Japanese patients with pulmonary arterial hypertension (PAH). Inhaled treprostinil was administered at three breaths (18 μg)/session four times daily, and the dose was gradually increased to a maximum of nine breaths (54 μg)/session. Endpoints included change in pulmonary vascular resistance index (PVRI) as primary, other efficacy parameters, safety, and PK. Seventeen PAH patients, the majority of whom (76.5%) had been receiving both an endothelin receptor antagonist (ERA) and a phosphodiesterase type-5 (PDE5) inhibitor/soluble guanylate cyclase (sGC) stimulator, received inhaled treprostinil. At Week 12, PVRI statistically decreased by −39.4 ± 25.5% (95% confidence interval: −52.6 to −26.3). The most frequently reported adverse events related to treprostinil were headache, cough, throat irritation, and hot flush. Regarding PK, there were no notable differences in the geometric mean Cmax and AUClast between Japanese and non-Japanese patients. Treatment with inhaled treprostinil using the dosing regimen approved in the United States resulted in significant improvement in hemodynamics, exercise capacity, and symptoms with a favorable tolerability and safety profile in Japanese patients. Inhaled treprostinil could be a valuable therapeutic option for Japanese patients with PAH, including those receiving a combination therapy with an ERA and a PDE5 inhibitor/sGC stimulator. Trial registration: JAPIC Clinical Trials Information [JapicCTI-194651].

DOI： 10.1002/pul2.12198

Web of Science

Scopus

PubMed

記述言語：英語  

DOI： 10.25259/SNI_723_2022

PubMed

記述言語：英語  

DOI： 10.1093/jnen/nlac098

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   出版者・発行元：Magnetic Resonance Materials in Physics, Biology and Medicine  

Objective: We propose a deep learning-based fully automatic right ventricle (RV) segmentation technique that targets radially reconstructed long-axis (RLA) images of the center of the RV region in routine short axis (SA) cardiovascular magnetic resonance (CMR) images. Accordingly, the purpose of this study is to compare the accuracy of deep learning-based fully automatic segmentation of RLA images with the accuracy of conventional deep learning-based segmentation in SA orientation in terms of the measurements of RV strain parameters. Materials and methods: We compared the accuracies of the above-mentioned methods in RV segmentations and in measuring RV strain parameters by Dice similarity coefficients (DSCs) and correlation coefficients. Results: DSC of RV segmentation of the RLA method exhibited a higher value than those of the conventional SA methods (0.84 vs. 0.61). Correlation coefficient with respect to manual RV strain measurements in the fully automatic RLA were superior to those in SA measurements (0.5–0.7 vs. 0.1–0.2). Discussion: Our proposed RLA realizes accurate fully automatic extraction of the entire RV region from an available CMR cine image without any additional imaging. Our findings overcome the complexity of image analysis in CMR without the limitations of the RV visualization in echocardiography.

DOI： 10.1007/s10334-022-01017-3

Web of Science

Scopus

PubMed

記述言語：英語  

DOI： 10.1161/CIRCHEARTFAILURE.122.009366

PubMed

記述言語：英語   出版者・発行元：Journal of the Society for Cardiovascular Angiography and Interventions  

Balloon pulmonary angioplasty (BPA) is an evolving treatment modality for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are not candidates for pulmonary endarterectomy. Although several imaging modalities currently exist for evaluating CTEPH, their individual use, specifically in the clinical practice of BPA, has not been well described. In this article, we provide a preprocedural, intraprocedural, and postprocedural interventional imaging roadmap for safe and effective BPA performance in routine clinical practice. Preprocedural assessment includes transthoracic echocardiography for right ventricular assessment, ventilation/perfusion scan to identify pulmonary segments with the highest degree of hypoperfusion, cross-sectional chest imaging excluding alternative causes of mismatched defects and providing anatomic and perfusion imaging concurrently, and nonselective invasive pulmonary angiography for risk stratification of individual lesion subtypes. Intraprocedural assessment includes subselective segmental angiography (SSA) for delineating segmental and subsegmental branch anatomy, lesion identification, and vessel sizing. Intravascular ultrasound and optical coherence tomography serve as adjunctive intraprocedural tools for more accurate vessel sizing and lesion characterization when SSA alone is insufficient. Postprocedural considerations include chest radiography to monitor for immediate postprocedure complications and echocardiography for the interval assessment of the right ventricle on longer-term follow-up.

DOI： 10.1016/j.jscai.2022.100429

Scopus

PubMed

記述言語：英語  

DOI： 10.1097/FJC.0000000000001328

PubMed

記述言語：英語  

DOI： 10.1253/circrep.CR-22-0098

Web of Science

PubMed

記述言語：英語  

DOI： 10.1126/scisignal.abn8017

PubMed

記述言語：英語   出版者・発行元：(一社)日本循環器学会  

肺高血圧症患者レジストリ(JAPHR)のデータを用いて、門脈肺高血圧症(PoPH)患者における現在の治療法、その有効性、臨床転帰を評価した。2008～2021年に登録されたPoPH患者を対象とした。単剤療法の患者と併用療法の患者に分け、血行動態の変化、WHO機能分類、臨床転帰を比較した。21施設で治療を受けた患者62例(中央値61歳、女性59.7%)を解析した。このうち治療未経験者は25例、単剤療法は21例、併用療法は41例であった。追跡期間中央値は1466日であった。WHO機能分類I～IIの患者は54.9%から69.7%に増加し、29%の患者で機能分類が1以上改善した。追跡期間中の死亡例(21.0%)、肺高血圧症増悪例(9.7%)、肺動脈高血圧症に特異的な薬剤の副作用による中止例(9.7%)について、単剤療法群と併用療法群で有意差はなかった。3年生存率は88.5%(95%CI:76.0～94.7)、5年生存率は80.2%(同64.8～89.3)であった。平均肺動脈圧、肺血管抵抗、心係数は単剤療法群より併用療法群で有意に改善した。以上より、PoPH患者での併用療法は単剤療法と比較して副作用のリスクを増加させることなく血行動態を有意に改善した。

記述言語：英語   出版者・発行元：Pulmonary Circulation  

Vagal nerve stimulation (VNS) ameliorates pulmonary vascular remodeling and improves survival in a rat model of pulmonary hypertension (PH). However, the direct impact of VNS on right ventricular (RV) function, which is the key predictor of PH patients, remains unknown. We evaluated the effect of VNS among the three groups: pulmonary artery banding (PAB) with sham stimulation (SS), PAB with VNS, and control (no PAB). We stimulated the right cervical vagal nerve with an implantable pulse generator, initiated VNS 2 weeks after PAB, and stimulated for 2 weeks. Compared to SS, VNS increased cardiac index (VNS: 130 ± 10 vs. SS: 93 ± 7 ml/min/kg; p < 0.05) and end-systolic elastance assessed by RV pressure–volume analysis (VNS: 1.1 ± 0.1 vs. SS: 0.7 ± 0.1 mmHg/μl; p < 0.01), but decreased RV end-diastolic pressure (VNS: 4.5 ± 0.7 vs. SS: 7.7 ± 1.0 mmHg; p < 0.05). Furthermore, VNS significantly attenuated RV fibrosis and CD68-positive cell migration. In PAB rats, VNS improved RV function, and attenuated fibrosis, and migration of inflammatory cells. These results provide a rationale for VNS therapy as a novel approach for RV dysfunction in PH patients.

DOI： 10.1002/pul2.12154

Web of Science

Scopus

PubMed

Web of Science

記述言語：英語   出版者・発行元：The Lancet Respiratory Medicine  

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.

DOI： 10.1016/S2213-2600(22)00171-0

Web of Science

Scopus

PubMed

記述言語：英語  

DOI： 10.1136/bmjresp-2022-001337

PubMed

記述言語：英語   出版者・発行元：Radiology: Cardiothoracic Imaging  

DOI： 10.1148/ryct.220086

Web of Science

Scopus

PubMed

記述言語：英語  

DOI： 10.1016/j.jacbts.2022.03.012

PubMed

記述言語：英語   出版者・発行元：European Respiratory Journal  

Background Treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) remain limited. Selexipag, an oral selective IP prostacyclin receptor agonist approved for pulmonary arterial hypertension, is a potential treatment option for CTEPH. Methods In this multicentre, randomised, double-blind, placebo-controlled study, 78 Japanese patients with inoperable CTEPH or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy and/or balloon pulmonary angioplasty were randomly assigned to receive placebo or selexipag. The primary end-point was the change in pulmonary vascular resistance (PVR) from baseline to week 20. Secondary end-points were changes in other haemodynamic parameters: 6-min walk distance (6MWD), Borg dyspnoea scale score, World Health Organization (WHO) functional class, EuroQol five-dimension five-level tool and N-terminal pro-brain natriuretic peptide. Results The change in PVR was -98.2±111.3 dyn s cm-5 and -4.6±163.6 dyn s cm-5 in the selexipag and placebo groups, respectively (mean difference -93.5 dyn s cm-5; 95% CI -156.8 to -30.3; p=0.006). The changes in cardiac index (p<0.001) and Borg dyspnoea scale score (p=0.036) were also significantly improved over placebo. 6MWD and WHO functional class were not significantly improved. The common adverse events in the selexipag group corresponded to those generally observed following administration of a prostacyclin analogue. Conclusion Selexipag significantly improved PVR and other haemodynamic variables in patients with CTEPH, although exercise capacity remained unchanged. Further large-scale investigation is necessary to prove the role of selexipag in CTEPH.

DOI： 10.1183/13993003.01694-2021

Web of Science

Scopus

PubMed

記述言語：英語   出版者・発行元：BMJ Open  

Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of prior pulmonary thromboembolism (PE), caused by incomplete clot dissolution after PE. In patients with CTEPH, lifelong anticoagulation is mandatory to prevent recurrence of PE and secondary in situ thrombus formation. Warfarin, a vitamin K antagonist, is commonly used for anticoagulation in CTEPH based on historical experience and evidence. The anticoagulant activity of warfarin is affected by food and drug interactions, requiring regular monitoring of prothrombin time. The lability of anticoagulant effect often results in haemorrhagic and thromboembolic complications. Thus, lifelong warfarin is a handicap in terms of safety and convenience. Currently, the use of direct oral anticoagulants (DOACs) in CTEPH has increased with the advent of four DOACs. The safety of DOACs is superior to warfarin, with less intracranial bleeding in patients with non-valvular atrial fibrillation and venous thromboembolism. Edoxaban, the latest DOAC, also has proven efficacy and safety for those diseases in two large clinical trials; the ENGAGE-AF trial and HOKUSAI-VTE trial. The present trial seeks to evaluate whether edoxaban is non-inferior to warfarin in preventing worsening of CTEPH. Methods and analysis The KABUKI trial (is an investigator-initiated, multicentre, phase 3, randomised, single-blind, parallel-group, warfarin-controlled, non-inferiority trial to evaluate the efficacy and safety of edoxaban versus warfarin (vitamin K Antagonist) in subjects with chronic thromBoembolic pUlmonary hypertension taking warfarin (vitamin K antagonIst) at baseline) is designed to prove the non-inferiority of edoxaban to warfarin in terms of efficacy and safety in patients with CTEPH. Ethics and dissemination This study is approved by the Institutional Review Board of each participating institution. The findings will be published in a peer-reviewed journal, including positive, negative and inconclusive results. Trial registration number NCT04730037. Protocol version This paper was written per the study protocol V.4.0, dated 29 January 2021.

DOI： 10.1136/bmjopen-2022-061225

Web of Science

Scopus

PubMed

記述言語：英語  

DOI： 10.1016/j.explore.2021.12.001

PubMed

記述言語：英語   出版者・発行元：European Heart Journal Cardiovascular Imaging  

DOI： 10.1093/ehjci/jeac027

Web of Science

Scopus

PubMed

記述言語：英語   出版者・発行元：Life  

Background: In patients with chronic kidney disease (CKD) on hemodialysis, comorbid pulmonary hypertension (PH) aggravates exercise tolerance and eventually worsens the prognosis. The treatment strategy for pre-capillary PH, including combined pre-and post-capillary PH (Cpc-PH), has not been established. Objectives: This study aimed to evaluate the impact of pulmonary vasodilators on exercise tolerance and pulmonary hemodynamics in patients with CKD on hemodialysis. Methods and Results: The medical records of 393 patients with suspected PH who underwent right heart catheterization were reviewed. Of these, seven patients had isolated pre-capillary PH and end-stage CKD on hemodialysis. Pulmonary vasodilators decreased pulmonary vascular resistance from 5.9 Wood units (interquartile range (IQR), 5.5–7.6) at baseline to 3.1 Wood units (IQR, 2.6–3.3) post-treatment (p = 0.02) as well as increased pulmonary capillary wedge pressure from 10 mmHg (IQR, 7–11) to 11 mmHg (IQR, 8–16) (p = 0.04). Pulmonary vasodilators increased the World Health Organization functional class I or II from 0% to 100% (p = 0.0002) and the 6 min walk distance from 273 m (IQR, 185–365) to 490 m (IQR, 470–550) (p = 0.03). Conclusions: Pulmonary vasodilators for PH in patients with CKD on hemodialysis decrease pulmonary vascular resistance and eventually improve exercise tolerance. Pulmonary vasodilators may help hemodialysis patients with pre-capillary PH, although careful management considering the risk of pulmonary edema is required.

DOI： 10.3390/life12060780

Web of Science

Scopus

PubMed

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語  

DOI： 10.1007/s10557-020-07123-5

PubMed

記述言語：英語   出版者・発行元：Heart and Lung  

Background: Balloon pulmonary angioplasty (BPA) improved pulmonary arterial compliance (CPA) and exercise tolerance in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Objectives: To investigate whether CPA is a useful index to indicate exercise tolerance improvement by BPA in CTEPH patients. Methods: The correlation between changes in CPA and improvements in 6-minute walk distance (6MWD) by BPA was retrospectively analyzed in 70 patients (Analysis 1), and it was sequentially analyzed in 46 symptomatic patients who achieved mean pulmonary arterial pressure (mPAP)<30mmHg (Analysis 2). Results: We enrolled 70 patients (female/male:57/13, mean age:59 years) who underwent a total of 352 BPA sessions which significantly increased CPA (1.5±0.8 vs. 3.0±1.0 mL/mmHg) and decreased pulmonary vascular resistance (PVR) (8.0 ± 3.9 vs. 3.6 ± 1.7 wood units). The correlation coefficient between improvement in 6MWD and changes in PVR and CPA were r=0.21 (p=0.09) and r=0.14 (p=0.26) (Analysis 1). In Analysis 2, those were r=0.32 (p=0.06) and r=0.38 (p=0.02), respectively. Conclusions: CPA can be a useful index to indicate the improvement in exercise tolerance by BPA in symptomatic patients with lower mPAP.

DOI： 10.1016/j.hrtlng.2021.11.003

Web of Science

Scopus

PubMed

記述言語：英語   出版者・発行元：International Heart Journal  

The AMBITION study (NCT01178073) provided the first long-term clinical evidence for initial combination therapy with ambrisentan and tadalafil in patients with pulmonary arterial hypertension (PAH). Nevertheless, predictors of treatment response were not assessed. To identify predictors for response to initial combination therapy, we examined pdata from 302 patients with PAH (World Health Organization Functional Class II or III) who received initial combination therapy from the modified intention-to-treat population of the AMBITION study (n = 605). A responder was defined as not having undergone a clinical failure event. Univariate and multivariate analyses were performed. Multivariate logistic regression with interactive backward selection was used to assess the independent association of potential predictors with response. Treatment responders were younger, more often female, and less likely to have comorbidities or a requirement for oxygen therapy, compared with nonresponders. At multivariate analysis, female sex (odds ratio [OR] 2.67; 95% confidence interval [CI] 1.29, 5.52; P = 0.0081), longer 6-minute walk distance (OR 1.01; 95% CI 1.00, 1.01; P = 0.0039), lower baseline log N-terminal-prohormone of brain natriuretic peptide (OR 0.70; 95% CI 0.52, 0.94; P = 0.0190), and aldosterone antagonist use (OR 2.54; 95% CI 1.03, 6.26; P = 0.0436) independently predicted response to initial combination therapy. Besides demographic factors, the absence of comorbidities and less severe disease state, and the use of aldosterone antagonist therapy identified patients with PAH most likely to respond to initial combination therapy with ambrisentan and tadalafil. Further study to evaluate the role of aldosterone antagonist therapy in PAH is warranted.

DOI： 10.1536/ihj.21-497

Web of Science

Scopus

PubMed

記述言語：英語   出版者・発行元：(一社)インターナショナルハートジャーナル刊行会  

AMBITION試験(アンブリセンタン、タダラフィル初回併用療法の有用性を評価した多施設共同無作為化二重盲検試験)の事後解析を実施し、初回併用療法に対する反応性の予測因子について検討した。modified ITT集団のうち初回併用療法が実施された肺動脈性肺高血圧症患者302例を対象とした。患者をレスポンダー群234例(女性181例、平均54.4±14.0歳)、非レスポンダー群68例(女性42例、平均61.0±12.2歳)に分けて検討した。その結果、レスポンダーでは、非レスポンダーと比較して若年で女性が多く併存疾患や酸素療法を要する患者が少ないことが示された。多変量解析の結果、女性(オッズ比[OR]2.67、P=0.0081)、6分間歩行距離(OR1.01、P=0.0039)、ベースライン時点でのlog NT-pro BNPの低下(OR 0.70、P=0.0190)、アルドステロン拮抗薬使用(OR2.54、P=0.0436)が初回併用療法に対する反応の独立した予測因子であった。以上のように、人口統計学的要因のほか、併存疾患が認められず、病勢が比較的軽度であること、アルドステロン拮抗薬使用が、アンブリセンタン、タダラフィル初回併用療法に最も反応しやすい肺動脈性肺高血圧症患者の特定に繋がることが示された。

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1097/MNM.0000000000000673.

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.hrtlng.2017.01.004.

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.ejrad.2016.12.015.

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.hrtlng.2015.06.005.

開催年月日： 2017年5月

記述言語：英語   会議種別：口頭発表（招待・特別）  

開催地：ワシントンDC   国名：アメリカ合衆国  

開催年月日： 2017年4月

記述言語：英語   会議種別：口頭発表（招待・特別）  

開催地：ジャカルタ   国名：インドネシア共和国  

開催年月日： 2017年3月

記述言語：英語   会議種別：シンポジウム・ワークショップ パネル（公募）  

開催地：金沢   国名：日本国  

開催年月日： 2024年3月

記述言語：英語  

開催地：神戸   国名：日本国  

開催年月日： 2022年3月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（公募）  

国名：日本国  

開催年月日： 2018年10月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（公募）  

開催地：東京   国名：日本国  

開催年月日： 2018年7月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（公募）  

開催地：横浜   国名：日本国  

開催年月日： 2018年3月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（公募）  

国名：日本国  

開催年月日： 2017年5月

記述言語：日本語   会議種別：口頭発表（招待・特別）  

開催地：札幌   国名：日本国  

開催年月日： 2017年3月

記述言語：日本語  

開催地：金沢   国名：日本国  

開催年月日： 2017年3月

記述言語：日本語  

開催地：金沢   国名：日本国  

開催年月日： 2016年11月

記述言語：英語  

開催地：New Orleans   国名：アメリカ合衆国  

開催年月日： 2016年11月

記述言語：英語  

開催地：New Orleans   国名：アメリカ合衆国  

開催年月日： 2016年10月

記述言語：日本語   会議種別：口頭発表（招待・特別）  

開催地：札幌   国名：日本国  

開催年月日： 2016年10月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（公募）  

開催地：東京   国名：日本国  

開催年月日： 2016年9月 - 2016年10月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（公募）  

開催地：東京   国名：日本国  

開催年月日： 2016年9月 - 2016年10月

記述言語：日本語   会議種別：口頭発表（招待・特別）  

開催地：東京   国名：日本国  

開催年月日： 2016年9月 - 2016年10月

記述言語：日本語   会議種別：口頭発表（一般）  

開催地：東京   国名：日本国  

開催年月日： 2016年9月 - 2016年10月

記述言語：日本語   会議種別：口頭発表（招待・特別）  

開催地：東京   国名：日本国  

開催年月日： 2016年9月 - 2016年10月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（公募）  

開催地：東京   国名：日本国  

開催年月日： 2016年9月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（公募）  

開催地：東京   国名：日本国  

開催年月日： 2016年9月

記述言語：英語  

開催地：Roma   国名：イタリア共和国  

開催年月日： 2016年8月 - 2016年9月

記述言語：英語  

開催地：Roma   国名：イタリア共和国  

開催年月日： 2016年7月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（公募）  

開催地：東京   国名：日本国  

開催年月日： 2016年5月

記述言語：英語   会議種別：シンポジウム・ワークショップ パネル（公募）  

開催地：Sendai   国名：日本国  

開催年月日： 2016年5月

記述言語：英語   会議種別：口頭発表（招待・特別）  

開催地：Sendai   国名：日本国  

開催年月日： 2016年5月

記述言語：英語  

開催地：San Francisco   国名：アメリカ合衆国  

記述言語：英語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：英語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：英語  

記述言語：英語  

記述言語：英語  

記述言語：英語  

記述言語：英語  

記述言語：英語  

記述言語：日本語  

記述言語：英語  

記述言語：英語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：英語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：英語  

記述言語：日本語  

記述言語：日本語  

記述言語：英語  

記述言語：日本語  

記述言語：日本語  

記述言語：英語  

記述言語：英語  

記述言語：日本語  

記述言語：英語  

記述言語：英語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語  

記述言語：日本語   出版者・発行元：(株)日本臨床社  

記述言語：英語   出版者・発行元：(公社)日本医学放射線学会  

記述言語：英語   掲載種別：記事・総説・解説・論説等（学術雑誌）  

記述言語：英語  

記述言語：日本語   出版者・発行元：(有)科学評論社  

記述言語：日本語   出版者・発行元：(株)文光堂  

記述言語：英語   掲載種別：記事・総説・解説・論説等（学術雑誌）  

種別：査読等 

外国語雑誌　査読論文数：15

国内会議録　査読論文数：20

種別：大会・シンポジウム等 

種別：査読等 

外国語雑誌　査読論文数：16

日本語雑誌　査読論文数：0

国際会議録　査読論文数：0

国内会議録　査読論文数：30

種別：大会・シンポジウム等 

種別：査読等 

外国語雑誌　査読論文数：10

日本語雑誌　査読論文数：0

国際会議録　査読論文数：0

国内会議録　査読論文数：30

種別：大会・シンポジウム等 

種別：大会・シンポジウム等 

種別：査読等 

外国語雑誌　査読論文数：16

種別：大会・シンポジウム等 

種別：大会・シンポジウム等 

種別：査読等 

外国語雑誌　査読論文数：8

日本語雑誌　査読論文数：0

国際会議録　査読論文数：0

国内会議録　査読論文数：10

種別：大会・シンポジウム等 

種別：大会・シンポジウム等 

種別：査読等 

外国語雑誌　査読論文数：9

日本語雑誌　査読論文数：0

国際会議録　査読論文数：0

国内会議録　査読論文数：10

種別：大会・シンポジウム等 

1年を通した小クラスでの症例ベースの講義.

1年を通した小クラスでの症例ベースの講義.

1年を通した小クラスでの症例ベースの講義.

1年を通した小クラスでの症例ベースの講義.