記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Long QT syndrome (LQTS) refractory to standard treatments, including β-blockers and implantable cardioverter-defibrillators (ICDs), has been indicated for left cardiac sympathetic denervation (LCSD) in Europe and the United States. However, the clinical implementation of LCSD remains rarely performed in Japan as it is not covered by national health insurance. CASE PRESENTATION: A 49-year-old woman with LQTS experienced frequent ICD activations, and β-blocker up-titration was limited due to severe heart failure. As a stellate ganglion block transiently shortened QT interval, LCSD was considered to prevent life-threatening arrhythmic events. Total intravenous anesthesia combined with a left thoracic paravertebral block was used to attenuate sympathetic nervous activation. Thoracoscopic LCSD was performed without arrhythmia or hemodynamic instability. The patient remained stable postoperatively, with no further ICD activations. CONCLUSION: This case demonstrates the safe perioperative management of LCSD for drug-refractory LQTS by incorporating strategies to minimize QT prolongation and suppress malignant arrhythmias.

DOI： 10.1186/s40981-025-00815-7

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

UNLABELLED: The efficacy of left bundle branch pacing (LBBP) as cardiac resynchronization therapy (CRT) has been reported, but LBBP may not always improve conduction disturbance in the left ventricle (LV). To evaluate LV electrical conduction delay during LBBP, we measured conduction time from the pacing at left bundle branch to LV lead sensing (LBBP-LV) in two patients in whom left bundle branch-optimized cardiac resynchronization therapy (LOT-CRT) was attempted. Case 1 was a 77-year-old female with dilated cardiomyopathy (DCM) and left bundle branch block. The QRS duration during LBBP was 160 ms and the interval between the stimulus artifact and peak of the R wave in lead V6 (Stim-V6RWPT) was 74 ms. LBBP-LV at the LV mid-lateral wall and LV mid-posterolateral wall were 112 ms and 102 ms, respectively. Case 2 was a 75-year-old female with DCM and nonspecific intraventricular conduction delay. The QRS duration during LBBP was 156 ms and Stim-V6RWPT was 66 ms. LBBP-LV at the LV mid-anterolateral wall, LV mid-lateral wall, and LV mid-posterolateral wall were 96 ms, 107 ms, and 121 ms, respectively. In conclusion, LBBP-LV at the LV mid-lateral area was relatively long. If LBBP does not improve LV conduction disturbances, LOT-CRT may be more effective. LEARNING OBJECTIVE: The efficacy of left bundle branch pacing (LBBP) for cardiac resynchronization therapy (CRT) has been reported. However, if the conduction time from pacing to the left ventricular (LV) lateral area during LBBP is long, LBBP may not improve LV conduction disturbance, resulting in ineffective CRT. In such cases, more effective CRT would be expected with LV lead implantation at the area of the LV conduction delay during LBBP.

DOI： 10.1016/j.jccase.2024.04.002

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Perforation by pacemaker leads, although rare, is a complication reported since the introduction of pacemaker therapy. Although historically reported frequencies were as high as 5%, recent reports have cited frequencies ranging from 1 to 2%. We report a case where a screw-type atrial lead slightly penetrated the right atrial wall, causing chronic abrasion of the ascending aorta, resulting in shock. CASE PRESENTATION: A 54-year-old male presented with dilated cardiomyopathy diagnosed at 40 years of age when he developed decompensated heart failure. Despite ongoing treatment, his heart failure worsened, leading to hospitalization at the age of 54. During his hospital stay, he experienced cardiac arrest that required cardiopulmonary resuscitation, followed by a return of spontaneous circulation. He was subsequently transferred to our institution after initiation of venoarterial extracorporeal membrane oxygenation (VA-ECMO) and an intra-aortic balloon pump (IABP). Echocardiography revealed an ejection fraction of 25%, left ventricular end-diastolic diameter of 60 mm, and severe mitral regurgitation (MR). Transcatheter mitral valve repair was performed to treat severe MR, followed by implantation of a cardiac resynchronization therapy defibrillator (CRT-D). Three months later, the patient was brought to our emergency department by ambulance because of hypotension. Contrast-enhanced computed tomography revealed pericardial effusion causing cardiac tamponade, necessitating emergency pericardial decompression via left fourth intercostal mini-thoracotomy and drain placement. Upon transfer to the intensive care unit, 1200 mL of blood was drained from the chest tube, prompting a return to the operating room for a median sternotomy. It was discovered that the pacemaker lead on the left side of the right atrium had slowly eroded into the aorta, leading to perforation. The ascending aorta was repaired and hemostasis was achieved; the patient recovered uneventfully and was discharged on postoperative day 18. CONCLUSIONS: The pacemaker lead perforated the right atrium; chronic abrasion of the lead against the ascending aorta resulted in bleeding from the ascending aorta 3 months later.

DOI： 10.1186/s43044-024-00494-2

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Earlier studies have shown male dominance of an early repolarization (ER) pattern and frequent coexistence with high Sokolow-Lyon voltage. Although possible involvement of androgen is speculated, the underlying mechanism has not been clarified yet. Previous studies were conducted in adult populations or only in children, and there has been no study in which the ER pattern was investigated in a series of individuals ranging from children before puberty to adults. METHODS: We included 600 individuals comprising six groups according to age: 10-14 years old, 15-19 years old, twenties, thirties, forties, and fifties. Each group had 50 males and 50 females. The distribution of an ER pattern and related ECG parameters were assessed by age and gender. RESULTS: In early teenagers, there was no significant gender difference in the prevalence of an ER pattern (24% in men vs. 28% in women, p = .82). The prevalence of an ER pattern increased after puberty and reached a peak in men in their twenties (42%). With further advance of age, the prevalence of an ER pattern decreased. On the other hand, the prevalence of an ER pattern in women peaked at 28% in teenagers, and it decreased through twenties (20%) to thirties (10%). Similar male dominance after puberty was observed in Sokolow-Lyon voltage and J-point elevation but not in P-wave amplitude. CONCLUSION: The prevalence of an ER pattern, Sokolow-Lyon voltage, and J-point elevation are all augmented after puberty and decrease with aging, leading to frequent coexistence of these ECG findings in young men.

DOI： 10.1002/joa3.12813

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

AIMS: The role of necroptosis in dilated cardiomyopathy (DCM) remains unclear. Here, we examined whether phosphorylation of mixed lineage kinase domain-like protein (MLKL), an indispensable event for execution of necroptosis, is associated with the progression of DCM. METHODS AND RESULTS: Patients with DCM (n = 56, 56 ± 15 years of age; 68% male) were enrolled for immunohistochemical analyses of biopsies. Adverse events were defined as a composite of death or admission for heart failure or ventricular arrhythmia. Compared with the normal myocardium, increased signals of MLKL phosphorylation were detected in the nuclei, cytoplasm, and intercalated discs of cardiomyocytes in biopsy samples from DCM patients. The phosphorylated MLKL (p-MLKL) signal was increased in enlarged nuclei or nuclei with bizarre shapes in hypertrophied cardiomyocytes. Nuclear p-MLKL level was correlated negatively with septal peak myocardial velocity during early diastole (r = -0.327, P = 0.019) and was correlated positively with tricuspid regurgitation pressure gradient (r = 0.339, P = 0.023), while p-MLKL level in intercalated discs was negatively correlated with mean left ventricular wall thickness (r = -0.360, P = 0.014). During a median follow-up period of 3.5 years, 10 patients (18%) had adverse events. To examine the difference in event rates according to p-MLKL expression levels, patients were divided into two groups by using the median value of nuclear p-MLKL or intercalated disc p-MLKL. A group with high nuclear p-MLKL level (H-nucMLKL group) had a higher adverse event rate than did a group with low nuclear p-MLKL level (L-nucMLKL group) (32% vs. 4%, P = 0.012), and Kaplan-Meier survival curves showed that the adverse event-free survival rate was lower in the H-nucMLKL group than in the L-nucMLKL group (P = 0.019 by the log-rank test). Such differences were not detected between groups divided by a median value of intercalated disc p-MLKL. In δ-sarcoglycan-deficient (Sgcd-/- ) mice, a model of DCM, total p-MLKL and nuclear p-MLKL levels were higher than in wild-type mice. CONCLUSION: The results suggest that increased localization of nuclear p-MLKL in cardiomyocytes is associated with left ventricular diastolic dysfunction and future adverse events in DCM.

DOI： 10.1002/ehf2.14059

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