Shiro Adachi

We aimed to evaluate the role of medical therapy before and after balloon pulmonary angioplasty (BPA) for non‐operable chronic thromboembolic pulmonary hypertension (CTEPH) in the modern management era. This Japanese nationwide, multicenter, prospective observational registry, including 37 centers, analyzed data newly registered from August 2018–December 2023. Pretreatment before BPA was analyzed in patients who were treatment naïve or received medical therapy only at registration ( n = 557). De‐escalation or escalation of medical therapy after BPA was analyzed in patients who underwent BPA ( n = 966). The BPA pretreatment analysis in 557 patients showed that approximately 70% of the patients received medical therapy, of which 18% received combination therapy. Patients who received monotherapy or combination therapy had more severe baseline hemodynamics than those who did not. The 3‐year survival did not significantly differ between the no, mono, and combination pretreatment groups (94%, 98%, and 97%, respectively; p = 0.852). The BPA post‐treatment analysis in 966 patients showed that 20% of patients underwent de‐escalation of medical therapy. Patients with de‐escalation of medical therapy had lower mean pulmonary artery pressure (PAP) (20.9 ± 5.8 vs. 24.1 ± 7.3 mmHg, p < 0.01); however, they had a lower cardiac index at last follow‐up compared to patients without de‐escalation (2.66 ± 0.61 vs. 2.84 ± 0.70 L/min/m ² , p = 0.01). Patients with severe disease tended to undergo pre‐treatment before BPA; however, patient survival did not differ across pretreatment types. De‐escalation of medical therapy after BPA can affect cardiac index. Therefore, de‐escalation or continuation of medical therapy should be carefully considered even after mean PAP normalizes after BPA.

Abnormal pulmonary hemodynamics during exercise may persist even after balloon pulmonary angioplasty (BPA) for inoperable chronic thromboembolic pulmonary hypertension (CTEPH). We report two female patients who underwent long‐term supervised outpatient cardiac rehabilitation (CR) after BPA with continuous pulmonary vasodilators. In Case 1, training was performed at an intensity close to the anaerobic threshold. In Case 2, high‐intensity interval training (HIIT) was gradually introduced. Exercise right heart catheterization was used for serial assessment of the mean pulmonary arterial pressure/cardiac output slope (mPAP/CO slope). Functional outcomes were also evaluated by cardiopulmonary exercise testing. No adverse events occurred during follow‐up. The mPAP/CO slope improved from 6.28 to 4.06 mmHg/L/min at 12 months in Case 1 and from 8.40 to 6.71 mmHg/L/min at 18 months in Case 2. Peak VO ₂ also increased from 16.2 to 20.2 mL/kg/min in Case 1 and from 18.3 to 24.9 mL/kg/min in Case 2. Long‐term supervised outpatient CR after BPA appears to be feasible and may improve exercise pulmonary hemodynamics and exercise capacity.

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Dynamic chest radiography (DCR) can estimate haemodynamic parameters in patients with heart failure (HF). Atrial fibrillation (AF) often coexists with HF; however, owing to its sometimes paroxysmal nature and minimal or absent symptoms, many patients with AF remain undiagnosed. Additional tools for AF diagnosis may be beneficial; therefore, we evaluated the ability of DCR to distinguish patients with HF in sinus rhythm (SR) from those with AF. In this small-sample pilot study, 20 patients with HF (median age, 67 years; males, 85%) underwent 12-lead electrocardiography and DCR on the same day. Aortic arch (Ao), right atrial (RA), right and left pulmonary artery (PA), and left ventricular (LV) apex pixel values (PVs) were measured. Seventeen patients were in SR and three demonstrated AF on 12-lead electrocardiography before DCR. The PV and PV change rate waveforms of the Ao, RA, PAs, and LV apex were regular in SR and irregular with AF. The difference between patients in SR and those with AF was particularly clear in the LV apex PV change rate waveforms. In addition, the heart rates (HRs) of patients in SR and with AF could be calculated from the PV change rate waveforms and were similar to those calculated by 12-lead electrocardiography. DCR can detect AF in patients with HF and may be able to infer HR.

Clinical utility of dynamic chest radiography in the oblique view to evaluate cardiac contraction: a case description

Dynamic chest radiography (DCR) can estimate haemodynamic parameters in heart failure (HF). However, no studies have evaluated its ability to determine cardiac systolic function in HF. This experimental study investigates the correlation between left ventricular (LV) ejection fraction (LVEF) and DCR image parameters in HF. Ninety-one patients with acute HF (median age, 58 years; males, 75%) (cardiologist diagnosis using the Framingham criteria) underwent DCR and transthoracic echocardiography after treatment for the uncompensated phase of HF. The LV apex pixel value (PV) change was measured by DCR. Correlations between the PV change and LVEF, as well as sensitivity, specificity, and area under the receiver operating characteristic curve (AUC) of DCR, were evaluated. LVEF and LV apex PV change were correlated in all patients (R = 0.428, P < 0.001) and in patients with LVEF < 50% (n = 38; R = 0.355, P = 0.029), < 40% (n = 31; R = 0.343, P = 0.059), and < 30% (n = 23; R = 0.321, P = 0.135). There was no significant correlation for patients with LVEF ≥ 50% (n = 53; R = − 0.004, P = 0.980). The LV apex PV change rate cutoff values for identifying LVEF < 50%, < 40%, and < 30% were 9.3% (AUC: 0.761, sensitivity: 0.698, specificity: 0.789, P < 0.001), 5.5% (AUC: 0.765, sensitivity: 0.883, specificity: 0.645, P < 0.001), and 5.5% (AUC: 0.767, sensitivity: 0.838, specificity: 0.696, P < 0.001), respectively. DCR may be useful to identify LV systolic dysfunction based on LVEF in acute HF. Graphical abstract

Direct oral anticoagulants (DOACs) have been used clinically in patients with chronic thromboembolic pulmonary hypertension (CTEPH) for secondary prevention after acute venous thromboembolism, although the data are limited. We evaluated the effects of DOACs—especially factor Xa (FXa) inhibitors—by measuring anti-factor Xa activity (AXA). Fifty consecutive CTEPH patients treated with rivaroxaban, apixaban, or edoxaban were enrolled. Heparin-calibrated AXA was measured at peak and trough. The median peak heparin-calibrated AXA across all 50 patients was 1.90 IU/mL and was comparable among the three FXa inhibitors. At trough, heparin-calibrated AXA was significantly higher in apixaban-treated patients (median 0.70 IU/mL) than in those given rivaroxaban (median 0.11 IU/mL) or edoxaban (median 0.11 IU/mL, p < 0.001). Peak heparin-calibrated AXA was significantly lower with reduced-dosage FXa inhibitor (edoxaban 30 mg/day) than with the reference dosage (edoxaban 60 mg/day, apixaban 10 mg/day, or rivaroxaban 15 mg/day, p = 0.01). The heparin-calibrated AXA of both rivaroxaban and apixaban was strongly significantly correlated with the plasma concentration of each drug. The cumulative rate of major and clinically relevant non-major bleeding was significantly higher in patients with peak heparin-calibrated AXA ≥ 2.09 IU/mL. Heparin-calibrated AXA could provide useful information for treating CTEPH patients with FXa inhibitors.

Background: The prognosis for patients with chronic thromboembolic pulmonary hypertension (CTEPH) using their nutritional status has not been established. We investigated the relationship between the prognosis of patients with CTEPH and the Controlling Nutritional Status (CONUT) score, which is a nutritional assessment tool.Methods and Results: A total of 157 patients with CTEPH was enrolled in the study. The primary outcome was defined as the composite outcome of all-cause mortality and non-elective hospitalization due to heart failure. Receiver operating characteristic (ROC) curve analysis was used to determine the cutoff CONUT score for predicting the 1-year rate of the primary outcome. Patients were divided into 2 groups according to the significant cutoff value and compared. Undernutrition was observed in 51.6% of patients. ROC analysis revealed a significant cutoff CONUT score of 3.5 (area under the curve=0.789). The incidence rate of the primary composite outcome was higher in the high CONUT group (score ≥4) than in the low CONUT group (score ≤3; 20% vs. 2.2%; P<0.001). Cox analysis revealed the CONUT score per point increase was an independent risk factor for the primary composite outcomes (hazard ratio 2.301; 95% confidence interval 1.081–4.895; P=0.031).Conclusions: The CONUT score can predict the 1-year rate of all-cause death and non-elective hospitalization in patients with CTEPH.

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.