BACKGROUND:To provide evidence from randomized controlled trials (RCTs) for large-vessel vasculitis (LVV), including Takayasu arteritis (TAK) and giant cell arteritis (GCA), to inform the forthcoming 2026 Japanese Circulation Society (JCS) clinical practice guideline. METHODS AND RESULTS:We drafted 4 and 7 clinical questions for TAK and GCA, respectively. A systematic review (SR) of RCTs was conducted using PubMed, CENTRAL, EMBASE, and the Japan Medical Abstracts Society through March 2024. Assessed with the GRADE approach, the certainty of evidence was very low for the most critical outcomes, low for some outcomes, and moderate for only 1 outcome. Evidence for TAK was limited. Tocilizumab (TCZ) resulted in a numerically lower relapse rate vs. placebo (risk ratio (RR) 0.73, 95% confidence interval (CI) 0.39-1.37) and was similar to adalimumab. No clear difference between mycophenolate mofetil (MMF) and methotrexate (MTX), or between abatacept (ABA) and placebo was observed. In GCA, TCZ reduced relapse (RR 0.29, 95% CI 0.09-0.98) and increased remission (RR 3.56, 95% CI 2.29-5.54) over placebo at 52 weeks. Tumor necrosis factor inhibitor, ABA, and MTX showed no benefit in cranial GCA. Serious adverse events were comparable between treatment groups. Geographic variation and differences in entry criteria were noted. CONCLUSIONS:This SR was comprehensive synthesis of evidence from RCTs for LVV therapies to support the 2026 JCS guideline.

Background:Whether upstroke time (UT) predicts outcomes after percutaneous coronary intervention (PCI) in patients with normal ankle-brachial index (ABI) remains unclear. Methods and Results:In a registry, 713 patients with ABI 0.90-1.40 and no atrial fibrillation or known severe aortic stenosis were analyzed. UT was measured from brachial-ankle pulse volume recordings and classified as <180 or ≥180 ms. Prolonged UT was associated with high risk of unplanned revascularization for de novo lesions (adjusted hazard ratio 1.84, P=0.015). Conclusions:UT prolongation indicates risk of de novo revascularization after PCI despite normal ABI.

BACKGROUND:Mean pulmonary arterial pressure (mPAP) after balloon pulmonary angioplasty (BPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) consistently has been lower in Japan compared with non-Japanese cohorts. RESEARCH QUESTION:What factors are associated with higher mPAP after BPA? STUDY DESIGN AND METHODS:This retrospective multicenter study screened 200 consecutive patients with CTEPH who underwent BPA at 2 centers in The Netherlands and 2 centers in Japan. After excluding patients with prior pulmonary endarterectomy or lacking follow-up hemodynamic data, 138 patients were analyzed. Comorbidities, lesion distribution and morphologic features, PH medication, procedural details, and hemodynamic parameters were compared between the 2 countries' cohorts. Multivariable regression analysis was performed to identify factors associated with high mPAP at follow-up. RESULTS:The proportion of male patients (55% vs 24%), BMI (mean [SD], 26 [4] kg/m2 vs 23 [5] kg/m2), the prevalence of atrial fibrillation (9% vs 0%), and smoking rate (43% vs 23%) were higher and diffusing capacity of the lung for carbon monoxide (Dlco) was lower (mean [SD], 69% [15%] vs 79% [19%]) in the cohort from The Netherlands. Proximal lesion distribution and severity on CT imaging were comparable between the 2 countries. The Japanese cohort demonstrated a greater mPAP reduction (-16 mm Hg [40%] vs -10 mm Hg [28%]; P < .01 ), with a lower mPAP at follow-up (mean [SE], 22 [1] mm Hg vs 30 [1] mm Hg; P < .01). Independent factors associated with higher follow-up mPAP included lower Dlco, higher right atrial pressure, higher pulmonary arterial wedge pressure at follow-up, a greater number of BPA sessions, and less frequent use of intermediate-sized balloons. INTERPRETATION:Our results show that higher mPAP after BPA was associated with impaired lung and left-sided heart function and procedural parameters, rather than nationality or lesion distribution. These findings suggest that occult cardiopulmonary comorbidities are important contributors to international differences in BPA outcomes and that differences in procedural practice may play a role.

BACKGROUNDS:We aimed to simultaneously investigate the relationship between left ventricular (LV) mechanical synchrony and intra-LV blood flow energy in patients with ischemic heart disease (IHD) using hybrid PET/MR. METHODS:Sixty-six patients (mean age 67 years, male 54) who underwent rest-pharmacological stress 13N ammonia PET and 4D flow MR were included. Left ventricular ejection fraction (LVEF), phase analysis (PA) parameters including histogram bandwidth (BW), phase standard deviation (PSD), and entropy were obtained from gated PET. Perfusion defect was assessed as summed defect score from static PET images. Intra-LV 4D flow MR acquisition was simultaneously performed for rest and stress. Intra-LV blood flow kinetic energy (KE mJ) was obtained from dynamic flow curve, and indexed with end-diastolic volume (KEi mJ/mL). RESULTS:A significant positive correlation was observed between LVEF and KEi at rest and stress (r = 0.30, p = 0.014; r = 0.42, p < 0.0001 for rest and stress, respectively). In contrast, summed defect score did not show a significant correlation to KEi. All PA parameters demonstrated a weak inverse correlation with KEi at rest (BW: r = -0.26, p = 0.019; PSD r = -0.27, p = 0.026; entropy r = -0.27, p = 0.031) and stress (BW: r = -0.29, p = 0.016; PSD r = -0.37, p = 0.0021; entropy r = -0.33, p = 0.0062). CONCLUSION:PET derived PA was associated with intra-LV blood flow energy, suggesting impaired LV mechanical synchrony is linked to intra-LV blood flow insufficiency. These findings highlight the novel assessment of PA in clinical practice for IHD.

Objectives:This study aimed to simultaneously evaluate 4D cardiac magnetic resonance (MR)-derived kinetic energy (KE) of intra-left ventricular (LV) blood flow, wall kinetics, and myocardial perfusion under pharmacological stress in patients with coronary artery disease (CAD) using a hybrid PET/MR system.Methods:Sixty-five patients (mean 68 +/- 12 y; male, 53) with CAD who underwent rest-stress 13N-ammonia PET/MR were included. MR acquisition was performed simultaneously during the PET scan to obtain rest-stress 4D flow, and followed by cine MR to measure LV ejection fraction (LVEF) and myocardial strain, including global longitudinal strain (GLS). The maximum KE during the cardiac cycle was calculated and indexed to the end-diastolic LV volume (maxKEi, mu J/mL) at rest and during stress. Perfusion defect, myocardial flow (MBF), and flow reserve (MFR) were assessed through rest-stress PET.Results:MaxKEi showed a significant correlation with LVEF and GLS for both rest and stress (r=0.3, P=0.01, r=-0.4, P=0.04 for rest LVEF and GLS; r=0.4, P=0.0009, r=-0.4, P=0.003, for stress LVEF and GLS, respectively). Stress maxKEi showed a significant correlation with stress MBF and MFR (r=0.3, P=0.006, and r=0.3, P=0.03, for stress MBF and MFR, respectively).Conclusion:Noninvasive assessment of 4D flow MR-derived intra-LV KE demonstrated a significant association with wall kinetics and endothelial function under pharmacological stress.

Objective The pathophysiology of right ventricular failure in patients with pulmonary hypertension has not been completely examined and monitoring strategies are lacking. The 14-3-3 protein family plays a regulatory role in cardiac fibroblasts. This study investigated whether 14-3-3 epsilon, a member of the 14-3-3 protein family, was associated with right ventricular failure. Methods A monocrotaline-induced pulmonary hypertension rat model was used to investigate the role of 14-3-3 epsilon in right ventricular failure. Plasma samples from patients with pulmonary hypertension were analyzed using an enzyme-linked immunosorbent assay for 14-3-3 epsilon. Patients Right ventricular samples from a monocrotaline-induced pulmonary hypertension rat model and plasma samples (n=83) from patients with pulmonary hypertension were used. Results The right ventricular 14-3-3 epsilon expression was higher in the monocrotaline group than in the control group. In patients with pulmonary hypertension, the median plasma 14-3-3 epsilon level was 95 ng/mL. Plasma 14-3-3 epsilon levels were significantly associated with tricuspid lateral annular peak systolic velocity and right ventricular fractional area changes in patients with pulmonary hypertension. Conclusion This is the first study to demonstrate an association between 14-3-3 epsilon and right ventricular failure. Plasma 14-3-3 epsilon level may be a useful circulating marker for evaluating right ventricular dysfunction in patients with pulmonary hypertension.

Positron emission tomography (PET) provides robust assessment of cardiac function, whereas evaluation of the right ventricle (RV) remains challenging. Myocardial strain analysis has emerged as a sensitive marker for subtle wall dysfunction. We aimed to evaluate the feasibility of RV strain quantification using a newly developed PET feature tracking technique, with magnetic resonance (MR) strain as the reference standard. Methods Consecutive 123 patients (mean 68 ys, male 101) who underwent rest 13N ammonia PETMR were retrospectively enrolled. Semi-automatic PET feature tracking was performed using previously validated in-house MATLAB-based software. RV global longitudinal (GLS %) and circumferential strain (GCS %) were derived from 4-chamber and short-axis gated PET cine images, respectively. For comparison, reference values were quantified from simultaneously acquired breath-hold cine MR using commercially available software. Results Mean GLS and GCS were -24.1±6.9% vs. -23.4±5.6% and -16.2±5.4% vs. -16.6±4.2% for PET and CMR, respectively. Both parameters showed strong correlations between the two modalities (r=0.77 and 0.74 for GLS and GCS, respectively; p<0.0001). Bland-Altman analysis demonstrated acceptable agreement, despite the presence of significant proportional bias (GLS: bias 0.1±4.4% [limits of agreement (LOA) -8.6 to 8.8], r=0.2, and p=0.01; GCS: bias -0.7±3.6% [LOA -7.8 to 6.4], r=-0.4, and p<0.0001, respectively). Conclusion PET derived RV wall strain analysis demonstrated a significant correlation with MR strain. PET feature tracking has the potential as a useful tool to detect RV wall dysfunction in routine clinical practice.

Pulmonary arterial hypertension (PAH) harboring the heterozygous ring finger protein 213 (RNF213) p.Arg4810Lys variant responds poorly to PAH treatment and has a poor prognosis. Here, we present a case of severe PAH and moyamoya disease (MMD) harboring a heterozygous RNF213 p.Arg4810Lys variant that responded well to PAH monotherapy. A 23-year-old woman with cerebral infarction due to MMD was referred to our center for MMD bypass surgery and treatment of severe PAH. Genetic analysis revealed a heterozygous RNF213 p.Arg4810Lys variant. We initiated PAH-targeted therapy before MMD bypass surgery, as bypass surgery was considered high-risk in the presence of severe PAH. Despite a positive response to acute vasoreactivity testing with nitric oxide (NO) inhalation, calcium channel blockers were avoided due to the risk of hypotension-induced cerebral ischemia. We initiated endothelin receptor antagonist (macitentan, 10 mg/day) monotherapy because upfront combination therapy may also cause hypotension. Endothelin receptor antagonist monotherapy improved symptoms and pulmonary hemodynamics, reducing vascular resistance from 11.4 to 7.1 Wood Units after 1 month. Bilateral cerebral bypass surgery was performed after hemodynamic improvement. The clinical spectrum of RNF213-associated PAH may be broader than previously reported. This case highlights the importance of personalized medicine that considers patient conditions and comorbidities when selecting treatment options for patients with PAH harboring the RNF213 p.Arg4810Lys variant.

Takayasu arteritis (TAK) is a chronic, refractory large-vessel vasculitis designated as a Designated Intractable Disease in Japan. The Japanese diagnostic criteria for TAK, last revised in 2017, have been updated to the 2026 version as part of the Japanese Circulation Society 2026 guidelines on the management of large vessel vasculitis, and are presented here in English for the first time. The revision was conducted through a 3-round modified Delphi process involving a panel of 21 experts, supplemented by email discussion and an in-person meeting. The updated criteria incorporate 3 major changes: new symptoms were added and a qualifying statement introduced in Section A; pulmonary artery involvement was elevated to a main criterion in Section B; and IgG4-related periaortitis, Cogan syndrome, and drug-induced aortitis were added in Section C. These updates reflect advances in imaging technology and international classification frameworks, with an emphasis on improving diagnostic sensitivity to reduce missed diagnoses.

BACKGROUND:Positron emission tomography (PET) provides robust assessment of cardiac function, whereas evaluation of the right ventricle (RV) remains challenging. Myocardial strain analysis has emerged as a sensitive marker for subtle wall dysfunction. We aimed to evaluate the feasibility of RV strain quantification using a newly developed PET feature tracking technique, with magnetic resonance (MR) strain as the reference standard. METHODS:Consecutive 123 patients (mean age: 68 years; males: 101) who underwent rest 13N ammonia PET/MR were retrospectively enrolled. Semiautomatic PET feature tracking was performed using previously validated in-house MATLAB-based software. RV global longitudinal strain (GLS %) and circumferential strain (GCS %) were derived from 4-chamber and short-axis gated PET cine images, respectively. For comparison, reference values were quantified from simultaneously acquired breath-hold cine MR using commercially available software. RESULTS:Mean GLS and GCS were -24.1 ± 6.9% vs -23.4 ± 5.6% and -16.2 ± 5.4% vs -16.6 ± 4.2% for PET and cardiovascular magnetic resonance imaging, respectively. Both parameters showed strong correlations between the two modalities (r = 0.77 and 0.74 for GLS and GCS, respectively; P < 0.0001). Bland-Altman analysis demonstrated acceptable agreement, despite the presence of significant proportional bias (GLS: bias 0.1 ± 4.4% [limits of agreement: -8.6 to 8.8], r = 0.2, and P = 0.01; GCS: bias of -0.7 ± 3.6% [limits of agreement: -7.8 to 6.4], r = -0.4, and P < 0.0001, respectively). CONCLUSIONS:PET-derived RV wall strain analysis demonstrated a significant correlation with MR strain. PET feature tracking has the potential as a useful tool to detect RV wall dysfunction in routine clinical practice.

Purpose: The aim of this study was to validate positron emission tomography feature tracking (PETFT) for assessing endocardial wall strain by comparing it with conventional tagging-cine magnetic resonance (MR) derived strain analysis (TAG). Methods: Consecutive 62 patients who underwent 13N-ammonia PETMR (52 males, mean age 66 years) were enrolled. PETFT and TAG were obtained through simultaneous acquisition with electrocardiography-gated PET and cine-MR for rest scan. Global longitudinal and circumferential strain (GLS and GCS) were calculated. Correlations and Bland-Altman plots were employed to evaluate associations, bias, and 95% limit of agreement (LOA) between PETFT and TAG. Results: PETFT and TAG showed significant correlations (r=0.69 [95% CI: 0.54 to 0.80], p<0.0001; r=0.55 [95% CI: 0.33-0.80], p < 0.0001 for GCS and GLS, respectively). Bland-Altman plot showed acceptable agreements (Bias 0.7 ± 6.7, LOA -12.5 to 13.9; Bias 1.3 ± 5.5, LOA -9.5 to 12.0 for GCS and GLS, respectively). In patients with abnormal perfusion, the correlations were still significant (r=0.76 [95% CI: 0.62 to 0.93], p<0.0001; r=0.59 [95% CI: 0.18 to 0.82], p=0.007 for GCS and GLS, respectively). Conclusion: PETFT has been identified as a feasible technique compared to TAG, demonstrating its potential as a novel tool for assessing wall strain in routine clinical practice. However, discrepancies in strain values may arise due to differences in algorithms and the presence of perfusion defects.

Background: The assessment of left ventricular (LV) mechanical synchrony using phase analysis (PA) provides incremental diagnostic value over conventional indicators and is widely used in clinical practice. Four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) has emerged as an imaging technique to visualize blood flow dynamics. Intra-LV kinetic energy (KE) obtained from 4D-CMR is a novel parameter refers to the quantification of the energy associated with the motion of blood within LV. This study aimed to simultaneously investigate the relationship between LV mechanical synchrony and intra-LV blood flow energy in patients with ischemic heart disease (IHD), using hybrid PET/MR system. Methods: Sixty-six patients (mean age 67 years, male 54) who underwent rest-pharmacological stress 13 N ammonia PET and 4D flow CMR at our hospital between January 2022 and February 2024 were included. Left ventricular ejection fraction (LVEF), PA parameters including histogram bandwidth (BW), phase standard deviation (PSD), and entropy were obtained from rest and stress gated PET. Perfusion defect was assessed as summed defect score from rest and stress static PET images. Intra-LV 4D flow MR acquisition was simultaneously performed for rest and stress. Intra-LV blood flow KE indexed with end-diastolic volume (KEi mJ/mL) was obtained from dynamic 4D flow curve. Results: BW[KF1] was 24.6 ± 17.9 and 24.4 ± 15.4%, PSD was 6.2 ± 3.7 and 6.2 ± 3.5, entropy was 61.6 ± 7.9 and 60.3 ± 9.3 for rest and stress, respectively. Summed defect score was 6.1 ± 7.1 and 11.2 ± 9.4 for rest and stress. KEi[KF2] was 0.018 ± 0.01, and 0.019 ± 0.01 (mJ/mL) for rest and stress. A significant positive correlation was observed between LVEF and KEi at both rest (R=0.30, P=0.014) and stress (R=0.42, P<0.001). Summed defect score was not significantly correlated with KEi at either rest or stress. All PA parameters demonstrated a weak inverse correlation with KEi at rest (BW: R= –0.29, P=0.019; PSD R = –0.27, P=0.026; entropy R = –0.27, P=0.031) and stress (BW: R= –0.28, P=0.023; PSD R = –0.36, P=0.003; entropy R = –0.31, P=0.010). Conclusions: PET-derived PA parameters were significantly associated with intra-LV blood flow KE, suggesting impaired LV mechanical synchrony is linked to intra-LV blood flow energy insufficiency.

This study aimed to investigate the correlation and assess the interchangeability between myocardial flow reserve (MFR) quantified by positron emission tomography (PET) and coronary sinus flow (CSF) reserve measured by cardiac magnetic resonance (CMR) in patients with ischemic heart disease, utilizing a hybrid PET/MR system. Consecutive 119 patients (mean 68.5 ± 13.1ys, male 95) who underwent rest-pharmacological stress 13N ammonia PETMR were enrolled. Myocardial blood flow (MBF mL/g/min) at rest and stress, along with MFR, were quantified from PET data. Myocardial perfusion defect was assessed using the summed stress score (SSS). CSF (mL/min/g) was obtained by phase-contrast cine CMR for rest and stress, and CSF reserve was calculated as the stress-to-rest ratio. Rest and stress CSF showed a significant but weak correlation to MBF (r = 0.40, p < 0.0001; r = 0.44, p < 0.0001 for rest and stress, respectively). CSF reserve showed a significant but moderate correlation with MFR (r = 0.43, p < 0.0001) and a weak inverse correlation with SSS (r = − 0.27, p = 0.003). Brand-Altman plots revealed a poor agreement with a proportional error (bias − 0.60 ± 1.34, 95

Introduction: Prior studies showed notable hemodynamic differences in the response to balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension (CTEPH) between Japan and European countries. These findings may be explained by differences in patient backgrounds. Research Question: This study aims to investigate international differences in hemodynamic outcomes of BPA and to explore the associations between these outcomes and patient backgrounds. Methods: Retrospective data was collected from two centers in the Netherlands and two centers in Japan; 50 consecutive patients diagnosed with CTEPH in 2018 were included from each center (Figure 1). Patients scheduled for pulmonary endarterectomy or without follow-up right heart catheterization were excluded. Statistical analyses included chi-square test, independent t-test, and repeated-measures ANOVAs with Bonferroni-corrected post-hoc comparisons. Results: A total of 67 patients from the Netherlands and 71 from Japan were included in the analysis (Figure 1). The proportion of male patients, smoking rate, body mass index, and prevalence of atrial fibrillation were significantly higher in the Netherlands (Table 1). At the time of diagnosis, Dutch patients exhibited significantly higher right atrial pressure, mean pulmonary arterial pressure (mPAP), pulmonary arterial wedge pressure (PAWP), cardiac index (CI), and significantly lower pulmonary vascular resistance (PVR) than Japanese patients (Table 1). PH medications were more frequently prescribed in the Netherlands. The median number of BPA sessions was 5 in the Netherlands and 6 in Japan (p = 0.161). Both cohorts demonstrated significant reductions in mPAP and PVR (mPAP: 42 to 30 mmHg in the Netherlands vs. 38 to 22 mmHg in Japan, p < 0.001, PVR: 6.4 to 3.2 WU in the Netherlands vs. 9.2 to 3.5 WU in Japan, p<0.001), and significant increases in PAWP and CI. However, the magnitude of reduction in mPAP and PVR was significantly greater in the Japanese group (p = 0.03 for mPAP and p<0.001 for PVR) (Figure 2). Conclusion: Although the number of BPA sessions were similar between the Netherlands and Japan, the effect of BPA on PVR and mPAP was much larger in Japan. Dutch patients had higher CI at baseline and at follow-up, possibly related to a higher use of PAH specific medication. Future multivariate regression analyses will determine which factors contribute to the distinct response to BPA of the 2 cohorts.

Background: Acute right heart failure (RHF) is a syndrome characterized by sudden right ventricular dysfunction leading to systemic hypoperfusion, which carries a poor prognosis, particularly in patients with pulmonary hypertension (PH). Early reduction of pulmonary vascular resistance (PVR) is crucial for improving RHF and reducing acute mortality. Compared with pulmonary vasodilators approved for pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH), inhaled nitric oxide (iNO) therapy has the advantages of being fast acting, an excellent selective pulmonary vasodilation, and has less effect on systemic blood pressure. Methods and Results: We describe a phase II, investigator-initiated, randomized, open-label trial (Japan Registry of Clinical Trials ]RCT2051220042) to evaluate the efficacy and safety of iNO therapy (INOflo (R) for inhalation 800 ppm), as an acute-phase treatment for severe RHF associated with PAH or CTEPH over a 1-week course. Thirty patients will be enrolled and randomized to receive the study drug, or not, in addition to conventional therapy. The primary endpoint is the change in PVR from baseline to 30 min after the start of inhalation, measured using right heart catheterization. Secondary endpoints include changes in hemodynamic parameters, arterial blood tests, and echocardiography findings, and the safety of iNO therapy, assessed through blood methemoglobin concentration, blood pressure, and adverse events. Conclusions: iNO therapy is expected to play a significant role in rapidly improving acute severe RHF associated with PH.

Manual aspiration thrombectomy (MAT) is a catheter intervention for cases with a high risk of bleeding. It is a rapid, simple, inexpensive, and single-session intervention for patients with proximal deep vein thrombosis (DVT). We evaluated the long-term outcome of MAT for patients with proximal DVT. All consecutive patients with acute proximal DVT treated at our center from April 2012 to December 2022 were retrospectively screened. Twenty-one patients who underwent MAT were enrolled. We evaluated the incidence and clinical characteristics of post-thrombotic syndrome (PTS) post-MAT in the chronic phase. The venous clot burden extent was assessed using the venographic segment (VS) score during catheter intervention. We evaluated the pre- and post-treatment total VS score. PTS was evaluated by PTS onset at any time between 6 and 24 months. We compared the clinical characteristics of patients with and without PTS (PTS and non-PTS groups, respectively). PTS onset at any time between 6 and 24 months post-MAT was 33

BACKGROUND:Balloon pulmonary angioplasty (BPA) improves haemodynamics in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Previous studies on BPA have set the treatment objective to achieve a mean pulmonary arterial pressure (mPAP) of <30 mm Hg. However, the clinical impact of mPAP after BPA remains unclear. This study aimed to stratify patients according to their mPAP after BPA and evaluate its association with clinical status and long-term outcomes. METHODS:We retrospectively reviewed 304 patients with inoperable CTEPH (median age, 72 (61 to 79) years) who underwent BPA and follow-up right heart catheterisation. Patients were categorised by mPAP after BPA: ≤20, >20-<30 and ≥30 mm Hg groups. RESULTS:The WHO functional classification, 6 min walk distance and right ventricular ejection fraction were significantly better in the group with a lower mPAP (trend test p=0.007, p<0.001 and p=0.002, respectively). Additionally, the proportions of patients who required pulmonary vasodilators or oxygen therapy were significantly lower in the group with a lower mPAP (trend test p<0.001 and p<0.001, respectively). Across all multivariable models, the mPAP ≥30 mm Hg group had a significantly poorer prognosis compared with the mPAP ≤20 mm Hg group. In contrast, no significant difference was observed between the mPAP ≤20 mm Hg and >20-<30 mm Hg groups. CONCLUSIONS:In patients with CTEPH after BPA, an mPAP of <30 mm Hg was associated with a favourable prognosis, and patients with an mPAP of ≤20 mm Hg presented with better symptoms, exercise capacity, right ventricular function and more withdrawal from oxygen therapy and pulmonary vasodilators. The BPA treatment goal may require individual adaptation.

This study aimed to simultaneously evaluate the association between diastolic left ventricular (LV) inflow and myocardial flow reserve (MFR) using a hybrid PET/MR system in patients with coronary artery disease (CAD). Sixty-seven patients (mean 66 ± 15 years, male 55) with CAD who underwent rest-pharmacological stress 13N-ammonia PET/MR were included. MFR, perfusion defect, and peak filling rate (PFR) were obtained through rest-stress PET. MR acquisition was performed simultaneously during the PET scan to obtain rest-stress 4D flow. Diastolic LV inflow volume (LVinf)(mL/s), peak velocity (peakV)(cm/s), and the change from the rest scan (Δvalue) were computed. Diastolic LV inflow parameters were compared based on the presence or absence of preserved MFR and perfusion defects. In all patients, diastolic LV inflow parameters significantly increased in the stress scan compared to the rest (53.7 ± 23.1 vs. 64.1 ± 32.9 ml/s, p = 0.0002; 68.1 ± 15.1 vs. 76.4 ± 20.6 cm/s, p = 0.0001 for LVinf and peakV, respectively). PeakV and PFR showed a significant correlation for rest and stress (r = 0.4, p = 0.01; r = 0.3, p = 0.03 for rest and stress, respectively).ΔpeakV significantly correlated to stress MBF, and MFR (r = 0.3, p = 0.007; r = 0.3, p = 0.04 for stress MBF, and MFR, respectively). Among patients with preserved LVEF (n = 47), when were divided into 4 subgroups based on the median myocardial flow reserve (MFR, 1.86) and the presence of perfusion defect, ΔpeakV was significantly higher in those with MFR above median and without abnormal perfusion compared to the other groups (21.8 ± 13.6 vs. 13.5 ± 17.0, 13.0 ± 13.5, and 4.6 ± 19.1; p = 0.04, 0.04, and 0.04 for MFR ≥ 1.86 or < 1.86 with or without abnormal perfusion, respectively). Non-invasive assessment of diastolic intra-LV hemodynamics derived from 4D flow MR demonstrated a significant association with coronary vasodilation.