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Background: Systemic sclerosis-associated pulmonary hypertension (SSc-PH) is widely recognized as the most severe subtype of connective tissue disease-associated pulmonary hypertension (CTD-PH), particularly in patients with complicating factors such as interstitial lung disease (ILD) and biventricular failure. This condition is associated with the poorest clinical outcomes among PH subtypes, presenting significant challenges in both management and prognosis. Despite the use of conventional therapies, including intravenous administration of epoprostenol, a promising prostacyclin analogue, treatment outcomes for SSc-PH remain suboptimal. While epoprostenol has demonstrated efficacy in reducing pulmonary arterial pressures, its clinical application is often constrained by the risk of ventilation–perfusion (V-Q) mismatch, particularly at higher doses. Case presentation: We report the case of a 73-year-old woman with SSc-PH complicated by ILD, who experienced progressive hemodynamic deterioration despite receiving optimized therapy with intravenous epoprostenol. Efforts to escalate the dose of epoprostenol were limited by the development of severe V-Q mismatch, precluding further dose increases. In light of these challenges, inhaled treprostinil was introduced as an adjunctive therapy. There were significant improvements in her pulmonary hypertension and hemodynamic parameters, ultimately allowing the discontinuation of intravenous dobutamine and stabilization of her hemodynamics, as well as her respiratory function, exercise capacity, and quality of life. Conclusions: This case highlights the potential clinical utility of combining inhaled treprostinil with intravenous epoprostenol for the treatment of SSc-PH in patients with concurrent ILD. By addressing the limitations associated with high-dose intravenous prostacyclin therapy, this combination approach may represent a promising therapeutic strategy for improving outcomes in this difficult-to-treat patient population. Further investigation is warranted to establish the efficacy and feasibility of this combination therapy in larger cohorts of patients with SSc-PH and associated ILD.

Impella (Abiomed, Danvers, MA, USA) is a percutaneous trans-catheter left ventricular assist device. Anticoagulant therapy targeting whole activated clotting time (ACT) between 160 and 180 s is recommended to prevent pump thrombosis during support. However, we sometimes experience fatal bleeding despite achieving the target ACT range. Consecutive patients who received Impella support in our institute between March 2018 and October 2020 were included in this retrospective study. The association between the averaged ACT levels during the Impella support and 30-day mortality was investigated. A total of 36 patients (71 years old, 61% males) were included. Most of the patients were managed within the recommended therapeutic range of ACT, and the average ACT level was 162 s. The higher ACT group (> 168 s) had older age, smaller body mass index, and higher serum creatinine compared with the lower ACT group (p < 0.05 for all). A higher ACT level was an independent risk factor of 30-day mortality with an adjusted hazard ratio of 1.085 (95% confidence interval 1.037–1.154) with a cut-off level of 168 s. There were only two thromboembolic events. Patients managed with higher ACT levels had a higher risk of 30-day mortality during Impella support. A low-dose heparin purge solution might be recommended in patients with high-risk for bleeding events.

: Advances in diagnostic criteria for transthyretin cardiac amyloidosis (ATTR-CM) and expanded insurance coverage for bone scintigraphy have facilitated earlier detection of ATTR-CM. However, whether these changes have translated into improved clinical outcomes among patients receiving disease-modifying therapy remains uncertain, especially in non-high-volume centers. : Consecutive patients with ATTR-CM who started disease-modifying therapy at our institute between May 2019 and March 2025 were retrospectively analyzed. Baseline characteristics and clinical outcomes were compared between the early period (2019-2021) and the late period (2021-2025). : A total of 31 patients (median age 77 years, 77% male) were included. Duration of heart failure was significantly shorter and the dose of loop diuretics at baseline was significantly lower in the late period ( < 0.05 for both). The prevalence of National Amyloid Center (NAC) stage I at baseline tended to be higher in the late period (75.0% versus 53.5%, = 0.273). The cumulative incidence of worsening heart failure hospitalization and all-cause death was significantly lower in the late period (6.3% versus 44.2%, = 0.024) during a median follow-up of 5 years. NAC stage I at baseline was independently associated with the lower primary outcome with an adjusted hazard ratio of 0.10 (95% confidence interval 0.01-0.90, = 0.040). : Patients with ATTR-CM in the late group experienced more favorable clinical outcomes after disease-modifying therapy, probably due to earlier diagnosis and therapeutic intervention, although further studies are warranted to verify the hypothesis.

Remote dielectric sensing (ReDS) system non-invasively quantifies pulmonary congestion. Re-admission following trans-catheter aortic valve replacement (TAVR) remains an unsolved matter. Residual pulmonary congestion is a strong risk factor of worse clinical outcomes in patients with heart failure. ReDS system may have a prognostic impact in patients undergoing TAVR. Patients who received TAVR and ReDS measurements during index hospitalization between 2021 and 2022 were included. The prognostic impact of ReDS value on the composite endpoint of death or re-admission following index discharge was investigated. Totally, 42 patients (median 84 years, 14 men) were included. Median ReDS value at index discharge was 27% (24%, 30%) and 10 patients had ReDS values > 30%. During a median of 316 (282, 354) days following index discharge, a higher ReDS value at baseline was independently associated with the incidence of composite endpoint with an adjusted hazard ratio of 1.32 (95% confidence interval between 1.10 and 1.58) with a calculated cutoff of 30%, which significantly stratified the cumulative incidence of the composite endpoint (78% in the high ReDS group [ N  = 10] and 36% in the normal ReDS group [ N  = 32], p  = 0.002). ReDS technology may be a promising tool to predict future clinical outcomes following TAVR by quantifying residual pulmonary congestion. The clinical implication of ReDS-guided aggressive intervention following TAVR remains the next concern.

Patients with atrial fibrillation (AF) often present with concomitant significant mitral regurgitation (MR). Percutaneous left atrial appendage closure (LAAC) is indicated for patients with AF to prevent thromboembolism and reduce the need for long-term anticoagulation. Transcatheter edge-to-edge repair (TEER) is recommended for patients with significant MR. However, the feasibility and efficacy of combining these therapeutic interventions remain uncertain. This study included consecutive patients who underwent LAAC. Feasibility was assessed by comparing outcomes between those undergoing combined LAAC + TEER and those receiving LAAC alone. Among 192 patients, 11 underwent the combined LAAC + TEER procedure, while 181 underwent LAAC alone. Procedural success was high in both groups (100% vs. 99%). At the 1-year follow-up, the incidence of significant device leak and device-related thrombus did not differ significantly between the groups (0% vs. 1.1% and 0% vs. 6.7%, respectively; p  > 0.05 for both). Additionally, the cumulative incidence of thromboembolic and bleeding events was comparable between the two groups (thromboembolic p  = 0.57 and bleeding p  = 0.42). The combination of LAAC + TEER may be a feasible and effective therapeutic strategy when performed in carefully selected patients at high-volume, experienced centers. Graphical abstract

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a phenotype of pulmonary hypertension due to chronic and multiple organized thrombus. The therapeutic strategy for patients with CTEPH and comorbid protein S deficiency remains unknown due to its rarity. Case: We encountered a 49-year-old male patient with CTEPH and concomitant mild protein S deficiency (type III). We could successfully perform balloon pulmonary angioplasty without any major complications, including thromboembolism and bleeding, followed by standard-dose oral anticoagulation therapy instead of warfarin. Conclusion: A currently established standard therapeutic strategy for CTEPH, including pulmonary angioplasty, may be safe and effective even in patients with concomitant inherent coagulation abnormalities.

Background: Sodium–glucose cotransporter 2 inhibitors (SGLT2i) reduce mortality and morbidity in patients with heart failure (HF), but are discontinued in some patients. Such patients may not enjoy favorable benefits of SGLT2i therapy. We evaluated the risk factors for SGLT2i discontinuation in a real-world population with HF. Methods: We retrospectively included consecutive patients who were hospitalized for HF and administered SGLT2i during the index hospitalization between February 2016 and September 2021. We assessed the baseline clinical factors associated with post-discharge discontinuation of SGLT2i. Results: This study included a total of 159 patients (median age = 73 years, 57 women). Among baseline characteristics, a lower serum albumin level (odds ratio = 0.23, 95% confidence interval = 0.07–0.76, p = 0.016) and a higher dose of furosemide (odds ratio = 1.02, 95% confidence interval = 1.00–1.05, p = 0.046) were independently associated with the future discontinuation of SGLT2i following index discharge. Patients who terminated SGLT2i (n = 19) had a higher incidence of HF recurrence or cardiovascular death during the 1-year therapeutic period (32% versus 11%, p = 0.020). Conclusions: Among patients who initiated SGLT2i during hospitalization for HF, lower serum albumin levels and higher doses of loop diuretic at index discharge were associated with the discontinuation of SGLT2i following index discharge. We should pay special attention to patients with such characteristics during the initiation of SGLT2i and during SGLT2i therapy.

Background Endovascular therapy (EVT) is a well-established revascularization strategy for patients with peripheral artery disease. However, achieving optimal wire crossing in complex chronic total occlusion lesions remains technically challenging. Intravascular ultrasound (IVUS)-guided wiring facilitates safer and more effective procedures. However, aligning IVUS findings with fluoroscopic imaging is challenging due to catheter rotation. We report a novel technique—termed the IDEAL technique—that leverages the deep vein as an anatomical landmark to correct IVUS rotational orientation. Case presentation. A 76-year-old female presented with chronic limb-threatening ischemia in the right toe. Contrast-enhanced computed tomography revealed a chronic total occlusion in the right superficial femoral artery. EVT was performed via the right common femoral artery. IVUS revealed that the first guidewire had entered the subintimal space partway through its course. Using preprocedural computed tomography, the deep vein was identified and its clock-face position relative to the artery was determined. This anatomical landmark enabled correction of IVUS image rotation, allowing accurate re-direction of a second guidewire under fluoroscopic guidance, thereby successfully crossing into the intraplaque lumen. Conclusions The IDEAL technique quickly provides anatomical orientation by utilizing the deep vein as a landmark during IVUS-guided wiring—making it, quite literally, ideal.

Background Sodium-glucose cotransporter 2 inhibitor (SGLT2i) reduces the risk of the composite renal endpoint and weakens the progressive decline in renal function in patients with chronic heart failure (HF). However, a detailed mechanism of SGLT2i on renal function and outcome remains uninvestigated. Methods We prospectively included 40 type 2 diabetic mellitus (T2DM) patients (median 68 years old, 29 male) who were hospitalized for decompensated HF and received SGLT2i during the index hospitalization. Of them, 24 patients had increases in estimated glomerular filtration rate (eGFR) at 12-month follow-up and 16 had decreases in eGFR. We investigated the baseline factors associating with the improvement in renal function. Results Lower plasma B-type natriuretic peptide (BNP) level and the use of renin-angiotensin system inhibitor (RASI) were independently associated with increases in eGFR during the follow-up period ( p  < 0.05 for both). Patients with both low plasma BNP levels and uses of RASI achieved significant increases in eGFR irrespective of the baseline HbA1c levels. Conclusions Lower plasma BNP level and the use of RASI at baseline were the key factors contributing to the renoprotective effects of SGLT2i among patients with decompensated HF and T2DM.

Background: The six-minute walk test is a practical tool for assessing functional capacity in patients with a variety of etiologies including pulmonary disease and heart failure. Six-minute walk distance (6MWD) is associated with mortality and morbidity in patients with a variety of comorbidities. We aimed to assess the prognostic impact of baseline 6MWD in patients with severe aortic stenosis undergoing trans-catheter aortic valve replacement (TAVR). Methods: Patients with severe aortic stenosis who underwent a six-minute walk test after index admission and underwent TAVR between 2015 and 2022 were included in this retrospective study. Patients were followed up for two years or until November 2022 following TAVR. The impact of baseline 6MWD on the primary composite outcome, defined as all-cause death and all-cause readmission during the 2-year observation period following index discharge, was assessed. Results: A total of 299 patients (median age 86 years old, 85 men) were included. They received a 6-min walk test prior to TAVR, underwent successful TAVR procedures, and were discharged alive. The median baseline 6MWD was 204 (143, 282) meters. Shorter baseline 6MWD was associated with higher cumulative incidence of the primary outcome with an adjusted hazard ratio of 0.76 (95% confidence interval 0.58–1.01, p = 0.055) with a cutoff 6MWD of 178 m during the 2-year observation period after index discharge. Conclusions: Overall, functional capacity was impaired in the elderly patients with severe aortic stenosis prior to TAVR. Patients with severe aortic stenosis having shorter baseline 6MWD tended to have higher rates of morbidity and mortality after successful TAVR. The clinical implication of aggressive cardiac rehabilitation to improve patients’ functional capacity and 6MWD-guided optimal patient selection remain the future concerns.