De-escalation of Oxygen Therapy and Medication in Patients With Chronic Thromboembolic Pulmonary Hypertension After Balloon Pulmonary Angioplasty.

BACKGROUND: There is no consensus on the adjustment of home oxygen therapy (HOT) and pulmonary hypertension (PH)-specific medications after balloon pulmonary angioplasty (BPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to examine the status of de-escalation and discontinuation of HOT and PH-specific medications post-BPA and clarify its effect on hemodynamics, biomarkers, and long-term outcomes. METHODS: From November 2012 to May 2018, 135 consecutive patients with CTEPH who underwent BPA at a single university hospital were enrolled (age, 63.5 ± 13.5 years; World Health Organization functional class (WHO-FC) II, III, IV; 34, 92, 9). RESULTS: The mean pulmonary arterial pressure decreased from 37.7 ± 11.3 to 20.4 ± 5.1 mm Hg 1 year post-BPA (P < 0.01). The proportion of patients who required HOT and combination medical therapy (≥ 2 PH-specific medications) decreased 1 year post-BPA (from 58.5% to 7.4% and from 40.0% to 10.4%, respectively). Baseline factors influencing the requirement of HOT and combination medical therapy post-BPA were almost identical (ie, lower exercise capacity and pulmonary diffusion capacity and worse hemodynamics). Regardless of their discontinuation, the improved hemodynamics, functional capacity (WHO-FC), and biomarkers (B-type natriuretic peptide and high-sensitivity troponin T) were almost maintained, and no adverse 1-year clinical outcomes (all-cause death and PH-related hospitalization) were observed. CONCLUSIONS: Most patients with CTEPH discontinued HOT and PH-specific combination medical therapy post-BPA, which was not associated with the deterioration of hemodynamics, functional capacity, or biomarkers. No long-term adverse outcomes were observed.

Survey results: status report on problems caused by sexual mismatch between sonographer and patient during echocardiography-a 2020 report of the Japanese Society of Echocardiography.

OBJECTIVES: Ultrasonography is an essential examination performed in various clinical fields. The number of clinical sonographers has been increasing. However, the working environments and conditions at each facility are different, leading to diverse problems. Among them, the emerging issue is the sexual disagreement between the sonographer and patient at the time of echocardiography. Since the patient must expose their breast during echocardiography, female patients may refuse to undergo the examination when conducted by a male sonographer. This study aimed to conduct a questionnaire survey to understand the measures for sonographer-patient gender mismatch at different facilities. METHODS: A questionnaire on the implementation of echocardiography by male sonographers for female patients was answered by representatives and specialist technicians of the Japanese Society of Echocardiography. RESULTS: Questionnaire responses were obtained from 50 facilities (59 participants). A total of 70% of the facilities restricted male sonographers from conducting echocardiography examinations for female patients. Among them, 81% of the facilities serviced female patients aged 60 years or younger. CONCLUSIONS: It has become clear that the gender selection of echocardiographic examiners varies from facility to facility, and providing a sufficient explanation before echocardiographic examination is necessary to avoid causing uncomfortable situations for female patients.

Balloon pulmonary angioplasty versus riociguat in inoperable chronic thromboembolic pulmonary hypertension (MR BPA): an open-label, randomised controlled trial.

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.

Clinical Significance of Right Ventricular Function in Pulmonary Hypertension.

Pulmonary hypertension (PH) is a progressive disease characterized by increased pulmonary vascular resistance that leads to right ventricular (RV) failure, a condition that determines its prognosis. This review focuses on the clinical value of the evaluation of RV function in PH. First, the pathophysiology of PH, including hemodynamics, RV function, and their interaction (known as ventriculoarterial coupling), are summarized. Next, non-invasive imaging modalities and the parameters of RV function, mainly assessed by echocardiography, are reviewed. Finally, the clinical impacts of RV function in PH are described. This review will compare the techniques that yield comprehensive information on RV function and their roles in the assessment of PH.

Clinical Significance of Guanylate Cyclase Stimulator, Riociguat, on Right Ventricular Functional Improvement in Patients with Pulmonary Hypertension.

BACKGROUND: Riociguat is a soluble guanylate cyclase stimulator that improves hemodynamics in patients with pulmonary hypertension (PH). Accumulating evidence implicates the additional effect of riociguat on the increase in cardiac output. However, its mechanisms have not been fully understood. This study aimed to investigate whether riociguat could ameliorate right ventricular (RV) contraction as well as hemodynamics. METHODS: We studied 45 patients with pulmonary arterial hypertension (14) or chronic thromboembolic pulmonary hypertension (31) and evaluated hemodynamics, using right-sided heart catheterization, before and after the administration of riociguat. RV function was assessed by echocardiography, including speckle-tracking echocardiography. RESULTS: Riociguat significantly improved the WHO functional class and reduced the mean pulmonary arterial pressure and vascular resistance. In addition, the cardiac index increased. RV remodeling was ameliorated after riociguat administration as assessed by the echocardiographic parameters, such as RV diameter and RV area index. RV function, including RV fractional area change and RV global longitudinal strain, also significantly improved, and their improvement was even observed in patients with mild PH after pulmonary endarterectomy or balloon pulmonary angioplasty. Furthermore, covariance analysis revealed that RV global longitudinal strain and RV fractional area change improved after riociguat administration, even with the same mean pulmonary arterial pressure, implicating the improvement of RV contractile function by riociguat, regardless of RV loading. CONCLUSIONS: Riociguat not only improved the hemodynamics of patients with PH but also ameliorated the echocardiographic parameters with RV function. RV strain could detect the subtle improvement in mild PH, and riociguat may have a benefit even after intervention, as assessed by speckle-tracking echocardiography.

Appropriate device selection for transcatheter atrial septal defect closure using three-dimensional transesophageal echocardiography.

Detail morphological evaluation for ASD is essential to achieve successful transcatheter closure. Three-dimensional transesophageal echocardiography (3D-TEE) is emerging, but few studies have comprehensively verified the usefulness of 3D-TEE. We divided 329 patients who underwent transcatheter ASD closure at our university hospital into 157 in the Conventional group evaluated with 2-dimensional transesophageal echocardiography and balloon sizing (BS), and 172 in the 3D-TEE group evaluated with 3D-TEE additionally. We assessed usefulness of 3D-TEE and consider appropriate device selection procedure. Overall, the percentage with re-sizing of device tended to be lower in the 3D-TEE group than in the Conventional group (10.1% vs 6.0%, p = 0.187). Among preprocedural modalities, the device size was mainly decided based on the BS diameter. A logistic regression analysis demonstrated that large atrial septum aneurysms (ASA) were associated with a ≥ 2 mm discrepancy of the BS diameter from the preprocedural 3D-TEE diameter (p < 0.05). Compared to the Amplatzer Septal Occluder, the differences in device size and the preprocedural ASD measurement were greater when using the Occlutech Figulla Flex II Occluder (FFII). Particularly, among the patients implanted with FFIIs, the discrepancies of the device size from the 3D-TEE measurement were greater in patients with large ASA than those with small ASA. Preprocedural 3D-TEE is useful to select the appropriate device size. Particularly, it is necessary to select a much larger device than that derived from the preprocedural 3D-TEE measurement when using FFII in patients with a septal aneurysm.

Preoperative right ventricular strain predicts sustained right ventricular dysfunction after balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension.

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.

Regional Right Ventricular Abnormalities Implicate Distinct Pathophysiological Conditions in Patients With Chronic Thromboembolic Pulmonary Hypertension.

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.

Characteristics of Chronic Obstructive Pulmonary Disease Patients with Pulmonary Hypertension Assessed by Echocardiography in a Three-Year Observational Cohort Study.

Background: Pulmonary hypertension (PH) is a major comorbidity of chronic obstructive pulmonary disease (COPD). However, the association of PH detected by echocardiography and COPD-related outcome in longitudinal follow-up has not been elucidated. In this study, we aimed to investigate the relationship between clinical characteristics of COPD patients with PH detected by echocardiography and various outcome parameters such as COPD exacerbation and health status over a three-year observation period. Methods: In this observational study, we analyzed patients with COPD who underwent chest computed tomography and echocardiography at baseline (n = 183). Results: The prevalence of PH was 21.9% (40 patients). The median estimated systolic pulmonary artery pressure in patients with PH was 38.8 mmHg. COPD patients with PH were older, had a lower body mass index, scored worse in the COPD Assessment Test and St. George's Respiratory Questionnaire, and exhibited a lower diffusing capacity of the lung for carbon monoxide in comparison to patients without PH. In computed tomography images, the percentages of low-attenuation areas (LAA%) and interstitial abnormalities were higher in COPD patients with PH than in those without PH. Higher values for LAA% (LAA ≥ 30%) and interstitial abnormalities independently increased the risk of PH. The ratio of main pulmonary diameter to aortic artery diameter was significantly correlated with estimated systolic pulmonary artery pressure. In the follow-up analysis, the frequency of exacerbations in three years was significantly higher in patients with PH compared to patients without PH. Conclusion: In this study, we identified the clinical characteristics of COPD patients with PH detected by echocardiography. The presence of PH assessed by echocardiography was related to future COPD exacerbations and closely related to radiographical emphysema.

Significance of echocardiographic evaluation for transcatheter aortic valve implantation.

Transcatheter aortic valve implantation (TAVI) is widely accepted as an alternative to surgical aortic valve replacement (SAVR) for the treatment of severe aortic stenosis (AS). Existing scientific evidence demonstrates that TAVI is superior to SAVR, and it is expected that indications for the clinical applications of TAVI will be expanded in the future. Echocardiography plays a key role in perioperative assessment of patients undergoing TAVI. Preprocedural echocardiographic evaluation is important to determine the severity of AS in addition to patients' anatomical suitability for TAVI. Furthermore, echocardiography is essential for intraoperative guidance, assessment of complications, postoperative evaluation, and prognostic prediction. Inaccurate echocardiographic measurements and evaluation can lead to less-than-optimal/inappropriate treatment strategies in patients with AS. Therefore, a thorough understanding of the limitations of echocardiographic evaluation is important. This review summarizes the role of echocardiographic evaluation in patients undergoing TAVI.

Multiple papillary fibroelastomas attached to left ventricular side and aortic side of the aortic valve: A report of new case and literature review.

The aortic valve (AV) is the most commonly affected site in multiple papillary fibroelastomas, but the frequency of embolism caused by the attachment side of the AV has not been elucidated. According to the review of the previous literature, 16 cases have been found attached to the AV. Of these, 6 of these have been found to be attached on the aortic side and 4 on the left ventricular side, 1 was bilateral, and 5 cases were unknown. Of the cases found on the aortic side, embolism occurred in 3 of them, and of the left ventricular side cases, embolism occurred in 2 of them. The frequency of embolism is equivalent even if papillary fibroelastoma attached to either side of the AV.

Long-term outcomes in 3 cases of quadricuspid aortic valve, using a new classification system: A case series and literature review.

Quadricuspid aortic valve has been classified based on leaflet size. However, no association is seen between classification and severity of aortic regurgitation (AR). Bicuspid aortic valve is classified according to the number of cusps, with significantly higher prevalence of AR in cases with a raphe. We classified cases according to raphe number. In 1 patient with no raphe, AR severity did not change into the eighth decade. However, AR severity worsened in patients with a raphe, in 1 case requiring aortic valve replacement in the fifth decade. Unequal shear stress may lead to leaflet fibrosis and progressive AR.

Shortening Hospital Stay Is Feasible and Safe in Patients With Chronic Thromboembolic Pulmonary Hypertension Treated With Balloon Pulmonary Angioplasty.

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.

Incidence, Clinical Characteristics, and Long-term Outcome of the Dilated Phase of Hypertrophic Cardiomyopathy.

Some patients with hypertrophic cardiomyopathy (HCM) develop systolic dysfunction, called the dilated phase of HCM (d-HCM), which is associated with increased morbidity and mortality. We conducted a retrospective study using an HCM database to clarify the incidence, clinical characteristics, and long-term outcomes of d-HCM. We analyzed an HCM cohort consisting of 434 patients (273 with apical HCM and 161 with non-apical HCM; 18 had obstructive HCM, 16 had dilated HCM, and 127 had other HCM) diagnosed by echocardiography in our hospital between 1991 and 2010. The follow-up period was 8.4 ± 6.7 years. The mean age at final follow-up was 67 ± 14 years, and 304 patients (70%) were men. The mean age of the 16 d-HCM patients at the initial visit was 45 ± 17 years, the age at final follow-up was 59 ± 18 years, and 13 were men. Thirteen d-HCM patients developed atrial fibrillation and six patients developed ischemic stroke. Twelve d-HCM patients were implanted with cardiac devices: one pacemaker, nine implantable cardioverter-defibrillators, and two cardiac resynchronization therapy with defibrillator. Five patients died of progressive heart failure at the age of 61 ± 23 years. The age at the initial visit and final follow-up were lower and the NYHA class, brain natriuretic peptide levels, and left ventricular function at initial evaluation were worse in the d-HCM group. Univariate analysis demonstrated that a lower age at the initial visit was associated with d-HCM (hazard ratio 0.955/1 year increase; 95% CI 0.920-0.991, P = 0.015). In our HCM cohort, the incidence of d-HCM was 4%. A high prevalence of atrial fibrillation and cerebral infarction and poor prognosis were noted in this group, despite patients undergoing medication and device implantation.

Prevalence, Clinical Profile, and In-Hospital Outcomes of Sleep-Disordered Breathing in Patients Undergoing Transcatheter Aortic Valve Implantation in Japan.

Background: The prevalence, patient profile, and outcomes of sleep-disordered breathing (SDB) in aortic stenosis (AS) remain unknown, especially in East Asia. Methods and Results: One hundred and eighty-one AS patients undergoing transcatheter aortic valve implantation (TAVI) were enrolled. Sixty-one patients (33.7%) had SDB, and lower stroke volume index was an independent determinant of SDB. Incidence of in-hospital stroke after TAVI was higher in the SDB group. Conclusions: SDB is associated with left ventricular systolic dysfunction in Japanese AS patients referred for TAVI. SDB was highly associated with the incidence of stroke as a procedural complication.

Impact of preprocedural echocardiographic parameters on increased stroke volume after transcatheter aortic valve replacement.

Aims: Increased stroke volume (SV) is a prognosticator of severe aortic stenosis (AS) after transcatheter aortic valve replacement (TAVR). This study aimed to investigate preprocedural echocardiographic predictors of increased SV after TAVR. Methods and results: Clinical and echocardiographic data were retrospectively analysed in 129 patients with severe AS who underwent TAVR (2013-2015). We compared the echocardiographic data and cardiac events between the decreased SV group (n=28) and the increased SV group (n=101). Univariate and multivariate analyses were used to assess the predictors of increasing SV. AS severity significantly diminished, left and right ventricular function improved, and SV index (SVi) increased after TAVR: aortic valve area index (0.46±0.13 vs. 1.18±0.33 cm2, p<0.001); aortic regurgitation (AR) grade (1.85±0.55 vs. 1.60±0.54, p<0.001); left ventricular ejection fraction (59.9±12.7 vs. 64.1±12.0%, p<0.001); right ventricular fractional area change (RVFAC) (48.8±11.9 vs. 53.3±14.0%, p<0.001); SV index (SVi) (46.7±11.0 vs. 52.8±12.0 ml/m2, p<0.001). Kaplan-Meier survival estimates suggested that the SVi increase was associated with the decreased cardiovascular events one year after TAVR (hazard ratio 4.08, 95% confidence interval [CI]: 1.32-12.7, p=0.02). On multivariate analysis, preprocedural AR grade (odds ratio [OR] 7.00, 95% CI: 2.76-17.8, p<0.001) and preprocedural RVFAC (OR 1.05, 95% CI: 1.01-1.10, p=0.011) correlated with the SV increase. Conclusions: Preprocedurally, greater AR and higher RVFAC could predict an increased SVi and thus the occurrence of fewer cardiac events. Preserved preprocedural RV systolic function is crucial for an increased SV after TAVR.

Delayed hemopericardium due to non-penetrating chest trauma: a report of new case and literature review.

To our knowledge, only 15 cases of delayed traumatic hemopericardium resulting from non-penetrating chest trauma have been reported. We present the case of a 63-year-old man with delayed hemopericardium, 2 months after striking the anterior chest on a mailbox when he fell down three steps during a postal delivery. Our case and review of the previously reported cases suggest that some cases might show quite slow progression of blood accumulation. Therefore, careful observation of patients who have experienced blunt trauma of the anterior chest is necessary.