Nakamura Memorial Hospital

Loeys-Dietz syndrome (LDS) is a rare autosomal dominant disorder whose clinical phenotype overlaps with that of Marfan syndrome (MFS) and vascular Ehlers-Danlos syndrome (vEDS), including aortic and/or arterial aneurysms, skeletal abnormalities, and spontaneous pneumothorax. Although the pathological features of MFS- and vEDS-associated pulmonary lesions have been described, the pulmonary pathology of LDS remains virtually unknown. Herein, we report the detailed histopathological features of LDS-associated pulmonary vascular abnormalities and pneumothorax. A 32-year-old Japanese woman underwent pulmonary surgery for spontaneous pneumothorax. She had undergone aortic valve surgery 7 years earlier, and genetic testing identified a heterozygous germline missense variant in TGFBR2 (c.1150 A > C, p.Asn384His). Histologically, the resected lung showed a ruptured bulla/bleb. Localized distal acinar emphysema was present in continuity with the bullous lesion. In the remaining parenchyma, the alveolar septa exhibited a distinctive proliferation of irregularly dilated and tortuous capillaries, reminiscent of pulmonary capillary hemangiomatosis. Hemosiderin-laden macrophages were conspicuous in some air spaces. In addition, several pulmonary muscular arteries and veins showed irregular dilatation and tortuosity, with elastic fibers that were fragmented and disorganized. Aberrant TGF-β signaling, together with deranged matrix formation, may underlie both the dilated, tortuous vasculature and the emphysematous/bullous changes leading to pneumothorax in the LDS lung.

PurposeWe report the treatment results of AVAgamma therapy combining gamma knife (GK) and bevacizumab (Bev) for recurrent glioblastoma.SubjectsFrom August 2013 to Jun 2025, 76 patients with recurrent glioblastoma treated with AVAgamma therapy as salvage therapy at the time of relapse after initial treatment. When the GK irradiated volume was 15 ml or less, a borderline dose of 20∼26 Gy was administered in a single fraction, and when it was greater than that, a borderline dose of 24∼30 Gy was administered in 2∼5 fractions. The mean therapeutic marginal dose was 24. 1 Gy. Bev was administered 10 mg/kg or 15 mg/kg 1 to 10 times after GK.MethodsMedian progression-free survival (mPFS) and median survival (mOS) from AVAgamma treatment, and mOS and 5-year survival rate from initial treatment were compared with historical controls (GK group: 30 patients) who received only GK at the time of recurrence.ResultsThe mPFS from AVAgamma therapy was 6 months, PFS-6month was 38%, OS-6month was 78%, and mOS was 11 months. The mOS from initial treatment was 25 months, which was prolonged compared to 21 months in the GK group (p = 0.08). The 5-year survival rate for recurrent glioblastoma was 5% in the GK group and 19% in the AVAgamma group (p = 0.08). The mOS of patients with recurrent glioblastoma with a KPS of 70% or more was 29 months, and the 5-year survival rate was 25%.DiscussionCombined use of Bev with GK was considered to provide local control of recurrent lesions and prolong life prognosis in patients with good KPS at the time of recurrence.ConclusionAVAgamma therapy is thought to prolong the survival of recurrent glioblastoma and play an important role as salvage treatment.

The relationship of body mass index (BMI) with clinical outcomes in patients with acute myocardial infarction (AMI) remains incompletely understood. In this study, 1113 patients with AMI who underwent percutaneous coronary intervention (PCI) were identified in the Rural AMI registry. Patients were divided into three groups based on BMI: underweight < 18.5 kg/ m², normoweight ≥ 18.5 to < 25.0 kg/ m², and overweight ≥ 25.0 kg/ m². The following data are given in this order. The endpoint of the study was in-hospital mortality. Underweight patients were older (77 ± 10 versus 69 ± 12 versus 63 ± 13 years; P < 0.001) and more likely to be female (38.5 versus 22.9 versus 20.2%; P = 0.009), and had a higher incidence of Killip class ≥ III (24.6 versus 12.7 versus 11.4%; P = 0.03). The median B-type natriuretic peptide levels on admission were 165 (interquartile range [IQR]: 73 - 565), 93 (IQR: 30 - 281), and 63 (IQR: 24 - 189) pg/mL (P < 0.001). The door-to-balloon time and postprocedural Thrombosis In Myocardial Infarction flow grades were similar among the BMI groups. During hospitalization, there were 65 all-cause deaths, with in-hospital mortality of 18.5%, 5.2%, and 4.7% in underweight, normoweight, and overweight patients, respectively (P = 0.001). In logistic regression analysis, the underweight group was significantly associated with in-hospital mortality using inverse probability of treatment weighting (odds ratio, 2.61; 95% confidence interval: 1.18 - 5.79, P = 0.02). These results suggest that assessment of BMI provides useful information for predicting in-hospital mortality in patients with AMI undergoing PCI.

Abstract Background: The ischiofemoral ligament (ISFL) contributes to posterior hip stability, but the extent to which the ISFL prevents dislocation after total hip arthroplasty (THA) remains unclear. This study aimed to investigate whether the ISFL independently contributes to dislocation resistance during hip flexion and internal rotation. Methods: A total of nine fresh-frozen cadaveric hips were tested using a custom jig under three conditions: (1) ISFL preserved, posterior capsule incised; (2) ISFL preserved, capsule repaired; and (3) both ISFL and posterior capsule disrupted. Dislocation occurrence and internal rotation angle under 2 N·m torque were measured at flexion angles from 0 to 105°, in 15° increments. Results: In condition (1), dislocation occurred in four hips at 60°, eight at 75°, and all nine by 90°. In condition (2), no dislocations occurred at any flexion angle. In condition (3), dislocation began at 30° and was universal by 60°. Following capsular repair in condition (2), the internal rotation angle increased by 10.8% overall compared to the angle before capsulotomy. At flexion angles ≥60°, the increase was 15.2%, which was statistically significant. Conclusion: The ISFL contributes to posterior hip stability in shallow flexion but is insufficient to prevent dislocation in mid-to-deep flexion. Posterior capsular reconstruction, particularly of the inferior portion, markedly enhances dislocation resistance, underscoring its importance for achieving stable THA.

Evaluation of left ventricular (LV) myocardial contractility to predict outcome in aortic stenosis (AS) is challenging because global longitudinal strain (GLS) does not take afterload into account. We thus tested prognostic value of an echocardiographic index of LV external work, stroke work index (SWI) in AS patients undergoing transcatheter aortic valve implantation (TAVI). We evaluated 1583 patients who underwent TAVI from database of a multicenter retrospective study. As an index of LV external work, echocardiographic LV SWI was calculated as 0.0136 × [stroke volume index × (mean aortic pressure + mean transvalvular pressure gradient-LV end-diastolic pressure)] [g × min/m2], where LV end-diastolic pressure was estimated as 4.9 + (0.62 × E/e') [mmHg]. GLS data was available in 902 patients. Primary endpoint was defined as cardiac death or worsening heart failure. During a median follow-up period of 725 days, 262 patients experienced primary endpoint. Kaplan-Meyer analysis showed that patients having lower LV SWI had lower event-free survival. Furthermore, Cox regression analysis showed that LV SWI was associated with primary endpoint independently of other clinically relevant factors. When influence of LV SWI on outcome was tested in preserved and reduced GLS groups respectively, LV SWI discriminated event-free survival in patients showing reduced GLS. Reduced LV SWI before TAVI was associated with poorer outcomes in AS patients. Furthermore, combined assessment of LV SWI and GLS is expected to improve prognostic risk stratification in AS patients undergoing TAVI.

Battery longevity in high-voltage devices (HVDs), specifically implantable cardioverter-defibrillators (ICDs) and cardiac resynchronization therapy-defibrillators (CRT-Ds), is critical for reducing the frequency of generator replacements, minimizing procedural risks, and enhancing patient outcomes. Despite technological advancements, significant variability in battery performance remains among the major manufacturers. This study aimed to evaluate the battery longevity among ICDs and CRT-Ds from the major manufacturers implanted at a single institution and identify the factors influencing battery depletion. We conducted a retrospective analysis of 353 patients implanted with HVDs (63 Abbott, 150 Boston Scientific, 140 Medtronic) at Hokkaido University Hospital between 2012 and 2021. Kaplan-Meier curves and Cox proportional hazards models were used to analyze the device longevity, with a primary endpoint of the time to battery depletion, defined by the elective replacement indicator. A multivariate analysis adjusted for the potential confounders. Boston Scientific devices exhibited a significantly longer battery life than Abbott and Medtronic devices (p < 0.001), with a 6-year replacement-free survival of 99% for ICDs and 93% for CRT-Ds. A multivariate analysis identified the device manufacturer, device type (ICD vs. CRT-D), and ventricular pacing rate as independent predictors of battery depletion (p < 0.001). Battery longevity differed significantly by the manufacturer, which may influence device selection. Devices with a longer battery life may help reduce the replacement frequency and could potentially contribute to improved patient outcomes and cost-effectiveness.

Anatomical variations in the anterior cerebral artery and/or anterior communicating artery complex are common. However, partial duplication is a rare variant of the anterior communicating artery. To the best of our knowledge, there are only a few reports describing a partially duplicated anterior communicating artery. We report a case of a partially duplicated anterior communicating artery associated with an intracranial aneurysm, which was diagnosed by 3-Tesla magnetic resonance angiography and digital subtraction angiography. A 43-year-old woman was admitted to our hospital for postoperative examinations following coil embolization of an unruptured right internal carotid artery aneurysm performed 1 year earlier. Magnetic resonance angiography and digital subtraction angiography revealed no recurrence of the treated aneurysm, and a partially duplicated anterior communicating artery—there were 2 separate anterior communicating arteries on the right side and one on the left side. Digital subtraction angiography under contralateral carotid artery compression showed no aneurysm in the anterior communicating artery complex. This report provides additional evidence of partially duplicated anterior communicating arteries associated with an intracranial aneurysm. Careful imaging assessment is important to identify this rare anatomical variant, as well as other concomitant variations and associated aneurysms.