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BACKGROUND: Successful bipolar radiofrequency catheter ablation (RFCA) of refractory ventricular arrhythmias (VAs) has been reported. However, the efficacy, safety, and long-term outcomes of bipolar RFCA of VAs are not fully determined. OBJECTIVE: The purpose of this study was to evaluate the effectiveness and safety of bipolar RFCA in treating refractory VAs during long-term follow-up. METHODS: Eighteen patients who underwent bipolar RFCA for ventricular tachycardia (VT) at 7 institutions were retrospectively investigated. Underlying heart diseases included remote myocardial infarction (n = 3 [17%]) and nonischemic cardiomyopathy (n = 15 [83%]). Although unipolar RFCA was performed in all patients, either it failed to suppress VT or VT recurred. The interventricular septum, left ventricular free wall, and left ventricular summit were targeted for bipolar RFCA. RESULTS: Acute success (VT termination and/or noninducibility) was achieved with bipolar RFCA in 16 patients (89%). Complications during the procedure included complete atrioventricular block (n = 2) and coronary artery stenosis (n = 1). One patient underwent chemical ablation after bipolar RFCA failure. At 12-month follow-up, VT reoccurred in 8 patients (44%). However, in patients with recurrence, VT burden had decreased: only 4 patients underwent re-RFCA, and only 1 of the 4 required chemical ablation. In the remaining 4 patients, re-RFCA was not required, as VT was controlled by medication or an implantable cardioverter-defibrillator. CONCLUSION: Bipolar RFCA is useful for acute suppression of refractory VT. Although VT recurrence rates during long-term follow-up were relatively high, we observed a significant reduction in VT burden.

DOI： 10.1016/j.hrthm.2020.04.028

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A 69-year-old Japanese woman was admitted to our hospital with progressive muscle weakness and dysphagia. She was taking pitavastatin for dyslipidemia. Her serum creatine kinase was 6,300 U/L. Pitavastatin was stopped, but her symptoms deteriorated, and cardiac congestion appeared. A muscle biopsy showed necrotizing myopathy (NM), and anti-signal recognition particle (SRP) antibody was positive. (18)F-fluorodeoxyglucose-positron emission tomography showed an abnormal uptake, and magnetic resonance imaging showed abnormal gadolinium enhancement in the left ventricular wall. An endomyocardial biopsy revealed inflammatory cardiomyopathy. Steroid, tacrolimus, and intravenous immunoglobulins were effective against the symptoms. This is the first case of biopsy-proven secondary cardiomyopathy due to anti-SRP-positive NM.

DOI： 10.2169/internalmedicine.2564-18

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DOI： 10.1161/CIRCIMAGING.119.009691

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Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by abnormalities in the alpha-galactosidase (Gal) A gene (GLA; MIM:300644). The reduced activity of the lysosomal enzyme, alpha-galactosidase A (alpha-Gal A) leads to classic early manifestations and vascular disease of the heart, kidneys, and brain. As a high-risk screening for symptomatic AFD using an enzymatic assay on dried blood spot samples, we enrolled 2325 individuals (803 females and 1522 males; median age: 66 years) with cardiac, renal, or neurological manifestations that met at least one of the following criteria: (a) family history of early-onset cardiovascular diseases; (b) typical classic manifestations, such as acroparesthesias, clustered angiokeratoma, cornea verticillata, and hypo-anhidrosis; (c) proteinuria; (d) receiving dialysis; (e) left ventricular hypertrophy on electrocardiography or echocardiography; or (f) history of stroke. Ninety-two patients displayed low alpha-Gal A activity. Four males and two females had different pathogenic GLA mutations (0.26%) including a novel mutation c.908-928del21. Four males (0.17%) harbored the GLA c.196G>C (p.E66Q) variant. This simple screening protocol using dried blood spot samples is useful for early diagnosis of AFD in high-risk and underdiagnosed patients suffering from various cardiac, renal, or neurological manifestations.

DOI： 10.1038/s10038-019-0633-1,

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DOI： 10.1161/CIRCIMAGING.119.008913

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BACKGROUND: Although we and others have reported cases of patients with Anderson-Fabry disease (AFD) complicated by coronary spastic angina (CSA), the prevalence of CSA in these patients remains unknown. Methods and Results: We performed the acetylcholine-induced provocation test, according to the Japanese guidelines for the diagnosis and treatment of patients with CSA, in 9 consecutive patients having 5 independent AFD pedigrees. Coronary spasms were provoked in conjunction with symptoms and ECG ischemic changes in 8 of 9 (89%) patients with AFD. CONCLUSIONS: We found an unexpectedly high prevalence of CSA in patients with AFD.

DOI： 10.1253/circj.CJ-18-0734

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Background: Cardiac sarcoidosis (CS) is a noncaseating granulomatous disease of unknown etiology. Lifelong immunosuppressive therapy, most frequently using corticosteroids, is a standard therapy to control hypersensitivity of immune reactions and prevent inflammation. However, it sometimes causes various systemic adverse effects and requires dose escalation. Thus, additional therapy may be required for the treatment of this disease. Recently, Propionibacterium acnes (P. acnes) was reported as one of the etiologic agents of CS, indicating that antibacterial drugs (ABD) may be effective for the treatment of CS. The objective of this study was to investigate the effect of ABD treatment, in addition to standard corticosteroid therapy, in patients with CS. Methods: The Japanese Antibacterial Drug Management for Cardiac Sarcoidosis (J-ACNES) trial was designed as a prospective, multicenter, randomized, open-label, controlled clinical trial. The patients will be randomized to receive either standard corticosteroid therapy plus ABD therapy (ABD group) or standard corticosteroid therapy (standard group). The primary endpoint is change in the total standardized uptake value at 6 months vs baseline using fluorine-18 fluorodeoxyglucose positron emission tomography and computed tomography. Secondary endpoints include efficacy, prognosis, and safety. Results: The results of this study are currently under investigation. Conclusion: The J-ACNES trial will be the first prospective study assessing the clinical benefit and safety of ABD therapy, in addition to corticosteroid treatment, in patients with CS. Our findings may improve treatment of patients with CS, as additional ABD therapy reduces recurrence of inflammation and elucidates the mechanism of sarcoidosis.

DOI： 10.1002/joa3.12084,

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The pro-arrhythmic triggers in Brugada and early repolarization syndromes (BrS, ERS) have not been analyzed systematically except for case reports. We clinically investigated the circumstances which precede/predispose to arrhythmic events in these syndromes during long-term follow-up. A detailed history from the patients/witnesses was taken to investigate the antecedent events in the last few hours that preceded syncope/ventricular fibrillation (VF); medical records, ECG and blood test from the emergency room (ER) were reviewed. 19 patients that fulfilled the investigation criteria were followed up for 71 +/- 49 months (34-190 months). Prior to the event (syncope/VF), the patients were partaking different activities in the following decreasing order; drinking alcoholic beverage, having meal, and getting up from sleep, exercise. 3 patients reported mental/physical stress prior to the event and 2 patients developed VF several days after starting oral steroid for treatment of bronchial asthma. In the ER, elevated J-wave amplitude (0.27 +/- 0.15 mV) was found with 58 % of the patients having hypokalemia. After electrolyte correction and cessation of steroids, the following day plasma K+ (4.2 +/- 0.3 mEq/L, P < 0.001) was significantly increased and J-wave amplitude (0.13 +/- 0.1 mV, P < 0.001) was remarkably reduced. Three patients were kept on oral spironolactone/potassium supplements. During follow-up for 71 +/- 49 (34-190) months, among 4 patients with VF recurrence, one patient developed VF after taking oral steroid. In ERS and BrS, hypokalemia and corticosteroid therapy add substantial pro-arrhythmic effects, but potentially treatable. Stopping steroid therapy and avoiding hypokalemia had excellent long-term outcome.

DOI： 10.1007/s00380-016-0828-8

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BACKGROUND: The distribution of late gadolinium enhancement (LGE) on the cardiac MRI (CMR) indicates myocardial fibrosis and provides information of possible reentry substrates. QT dynamicity reflecting repolarization abnormalities has gained attention as a potential prognostic predictive factor. OBJECTIVE: To clarify the correlation between the LGE distribution on CMR and QT dynamicity represented by the QT/RR relationship. METHODS: CMR and QT/RR analyses using Holter monitoring were performed in 34 patients (24 males, 60 +/- 11 years) with ventricular tachycardia (VT) and/or ventricular fibrillation (VF). The LGE on CMR was scored using a 4-point score in 17 left ventricular segments. The sum of the LGE scores was calculated for each patient. The QT/RR slope and daytime/nighttime QT/RR ratio (day/night ratio) were calculated. The correlation between the slope or the day/night QT/RR ratio and late enhancement findings was analyzed. RESULTS: All patients were divided into 23 LGE positive (LGE(+)) and 11 LGE negative (LGE(-)) patients. The slopes of the QTe/RR and QTa /RR were significantly steeper in the LGE(+) than in LGE(-) patients (0.21 +/- 0.03 vs 0.13 +/- 0.02; P < 0.001, 0.19 +/- 0.03 vs 0.13 +/- 0.02; P < 0.001, respectively), and both slopes were significantly correlated with the total LGE scores (r = 0.83, P < 0.001; r = 0.71, P < 0.001, respectively). In the LGE(+) patients, the QTe day/night (1.37 +/- 0.38 vs 0.91 +/- 0.33; P = 0.002) and QTa day/night ratios (1.33 +/- 0.26 vs 1.06 +/- 0.30; P = 0.011) were significantly greater than those in the LGE(-) patients. CONCLUSION: The LGE distribution was closely related to the QT dynamicity, suggesting that a combination of these markers can be a powerful tool for understanding the background pathophysiology.

DOI： 10.1111/anec.12280

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BACKGROUND: The presence of myocardial scar detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) has been described as a good independent predictor of mortality in patients with hypertrophic cardiomyopathy (HCM). Time-domain T-wave alternans (TWA) is also a potential predictor of cardiac mortality in patients with LV dysfunction. OBJECTIVE: To elucidate the relationship between the LGE distribution and TWA in patients with HCM. METHODS: CMR and TWA analyses using Holter monitoring were performed in 42 patients with HCM. The average transmural extent of the LGE was scored as 1 to 4 in each segment and the sum of the LGE scores (Total LGE score) was calculated in each patient. The correlation between the maximal time-domain TWA voltage and LGE findings was analyzed and the differences in the time-domain TWA, total LGE, and cardiac function assessed by CMR in the presence or absence of VT were also compared. RESULTS: The total LGE score was significantly and positively correlated with the maximal time-domain TWA voltage (r=0.59, P<0.001). Furthermore, the total LGE and maximal time-domain TWA voltage were significantly greater in the patients who had episodes of ventricular tachycardia (VT) (n=21) than in those without [23+/-7 vs. 10+/-8 (P<0.001); 87+/-26 vs. 62+/-12 muV (P<0.001), respectively]. However, the left ventricular ejection fraction did not statistically differ between the patients with VT and those without (56+/-14 vs. 61+/-7%, P=0.102). CONCLUSION: The magnitude of the localized LGE was significantly correlated with abnormalities in ventricular repolarization as assessed by TWA and QTd.

DOI： 10.1016/j.hrthm.2015.04.028

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BACKGROUND: The presence of a myocardial scar detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) has been described as a predictor of all-cause mortality in hypertrophic cardiomyopathy (HCM). However, the detailed spatial relationship between LGE site and electrical abnormality is unclear in high-risk HCM with malignant arrhythmia. OBJECTIVE: The purpose of this study was to elucidate the detailed relationship between the site on CMR imaging and the electrically damaged site, a potential origin of ventricular arrhythmias in patients with HCM. METHODS: Fifty consecutive HCM patients underwent contrast-enhanced CMR. Of those patients, 18 patients with ventricular tachycardia underwent electrophysiology study including endocardial mapping of the left ventricle (LV). The LGE area was calculated at 12 different LV sites: anterior, lateral, posterior, and septal segments of the basal, middle, and apical portions. At each LV site, the bipolar electrogram, effective refractory period (ERP), and monophasic action potential were recorded. RESULTS: LGE-positive segments demonstrated a significantly lower amplitude (4.0 +/- 2.8 mV vs 7.3 +/- 3.6 mV; P < .001), longer duration (54.7 +/- 17.8 vs 40.6 +/- 7.8 ms; P < .001), longer ERP (320 +/- 42 ms vs 284 +/- 37 ms; P = .001), and longer monophasic action potential duration measured at 90% repolarization (321 +/- 19 ms vs 283 +/- 25 ms; P < .001) than did LGE-negative segments. The LGE area negatively correlated with the amplitude (r = -0.59; P < .001) and positively correlated with the duration (r = 0.64; P < .001), ERP (r = 0.44; P < .001), and action potential duration measured at 90% repolarization (r = 0.63; P < .001). All the observed VTs originated from LGE-positive segments. CONCLUSION: The spatial distribution of LGE significantly correlates with depolarizing and repolarizing electrical damage in high-risk HCM with malignant ventricular arrhythmia.

DOI： 10.1016/j.hrthm.2015.02.004

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BACKGROUND: Intravenous administration of magnesium (Mg(2+)) is effective for polymorphic ventricular tachycardia via homogenization of transmural ventricular repolarization. Mg(2+) likely plays some role in the heterogeneity of repolarization in J wave syndromes. OBJECTIVE: To investigate the relationship between the repolarization parameters and serum Mg(2+), potassium (K(+)), and calcium (Ca(2+)) levels in J wave syndromes. METHODS: Thirteen J-wave syndrome patients (Brugada and early repolarization [ER] syndromes), with documented episodes of ventricular fibrillation (VF), and 13 ER pattern (ERP) or Brugada type ECG patients were enrolled (25 males, mean age 48 +/- 15 years). The 12-lead ECG-derived parameters including the QT, QT dispersion (QTd), Tpeak-Tend (Tp-e) interval, Tp-e dispersion (Tp-ed), Tp-e/QT ratio, and activation recovery interval (ARI) dispersion were calculated; the correlations between these parameters and electrolytes including Mg(2+), K(+), and Ca(2+) were analyzed. RESULTS: Although there was no association between serum K(+) or Ca(2+) and QTd, there was a strong negative correlation between serum Mg(2+) and QTd in J wave syndrome patients with a history of VF (r = -0.715, p = 0.006). Also, there was a tendency for a negative correlation between Mg(2+) and Tp-ed or ARI dispersion in J wave syndrome patients with a history of VF (r = -0.513, p = 0.072 and r = -0.53, p = 0.063, respectively). On the other hand, in 13 patients with a Brugada type ECG or ERP, no correlation was observed between serum Mg(2+) and the QTd, Tp-ed or ARI dispersion. CONCLUSION: Serum Mg(2+) may play an important role in the cardiac repolarization process in J wave syndromes.

DOI： 10.1684/mrh.2015.0379

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INTRODUCTION: We reported impaired QT-rate dependence in early repolarization syndrome (ERS); however, contemporary data have shown peak incidence of sudden cardiac death (SCD) in ERS and Brugada syndrome (BrS) at mid-night and early morning. Taken together, we analyzed the nocturnal QT-rate dependence in both syndromes. METHODS AND RESULTS: A total of 172 subjects were enrolled: 11 ERS, 11 BrS patients, 50 subjects with an uneventful ER pattern (ERP), and 100 non-J-wave control subjects. Ambulatory ECG-derived parameters (QT, QTc, and QT/RR slope) and day-night QT difference were analyzed and compared. Among the groups, there was no significant difference in the average QT or QTc; however, the 24-hour QT/RR slope was significantly smaller in ERS and BrS patients (0.103 +/- 0.01 and 0.106 +/- 0.01, respectively) than in the control group (0.156 +/- 0.03, P < 0.001). Detailed analysis showed a lower day-night QT difference in ERS and BrS patients (19 +/-18.7 and 24 +/-14 milliseconds, respectively) than in the controls (40 +/- 22 milliseconds, P = 0.007) with the lowest QT/RR slopes seen in the ERS and BrS groups from 0 to 3:00 am (QT/RR; 0.076 +/- 0.02 vs. 0.092 +/- 0.04 vs. 0.117 +/- 0.04, for the ERS, BrS, and controls, respectively, P = 0.004) and from 3 to 6 am (QT/RR 0.074 +/- 0.03 vs. 0.079 +/- 0.02 vs. 0.118 +/- 0.04, P < 0.001). CONCLUSION: In a large population of age- and gender-matched groups, both ERS and BrS patients showed attenuated QT-rate dependence and impaired QT day-night modulation that may provide a baseline reentrant substrate. Importantly, QT/RR maladaptation was most evident at mid-night and early morning, which may explain the propensity of such patients to develop SCD during this critical period.

DOI： 10.1111/jce.12566

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We describe three cases of J-wave syndrome in which ventricular fibrillation (VF) was probably induced by corticosteroid therapy. The patients involved were being treated with prednisolone for concomitant bronchial asthma. One of the three patients had only one episode of VF during her long follow-up period (14 years). Two patients had hypokalemia during their VF episodes. Corticosteroids have been shown to induce various types of arrhythmia and to modify cardiac potassium channels. We discuss the possible association between corticosteroid therapy and VF in J-wave syndrome based on the cases we have encountered.

DOI： 10.1007/s00380-013-0443-x

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INTRODUCTION: Almost all current investigations on early repolarization syndrome (ERS) have focused on the J-wave characteristics and ST-segment configuration; however, few have reported on ventricular repolarization indexes in ERS. METHODS AND RESULTS: A total of 145 subjects were enrolled: 10 ERS patients, 45 uneventful ER pattern (ERP) subjects, and 90 healthy controls without J waves or ST-segment elevation. Ambulatory ECG-derived parameters (QT, QTc(B), QTc(F), T peak-Tend(Tpe), and QT/RR slope) were measured and statistically compared. Among the groups, there was no significant difference in the average QT and QTc(B); however, ERS patients had the shortest QTc(F) and longest Tpe (QTc(F): 396.2 +/- 19 vs 410.4 +/- 20 vs 419.2 +/- 19 milliseconds, P = 0.036, Tpe: 84.9 +/- 12 vs 70.4 +/- 11 vs 66.9 +/- 15 milliseconds, P < 0.001, for the ERS, ERP, and control groups, respectively). Importantly, the 24-hour QT/RR slope was significantly smaller in the ERS than ERP and control groups (QT/RR: 0.105 +/- 0.01 vs 0.154 +/- 0.02 vs 0.161 +/- 0.03, respectively; P < 0.001). When analyzing the diurnal and nocturnal QT/RR slopes, ERS patients had small diurnal and nocturnal QT/RR slopes while the ERP and control groups had large diurnal and small nocturnal QT/RR slopes (diurnal QT/RR: 0. 077 +/- 0.01 vs 0.132 +/- 0.03 vs 0.143 +/- 0.03, P < 0.001; nocturnal QT/RR: 0.093 +/- 0.02 vs 0.129 +/- 0.03 vs 0.130 +/- 0.04, P = 0.02 in the ERS, ERP, and control groups, respectively). CONCLUSION: ERS patients had a continuously depressed diurnal and nocturnal adaptation of the QT interval to the heart rate. Such abnormal repolarization dynamics might provide a substrate for reentry and be an important element for developing ventricular fibrillation in the ERS cohort.

DOI： 10.1111/jce.12074

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INTRODUCTION: Recently, great attention has been paid to the risk stratification of asymptomatic patients with an electrocardiographic early repolarization (ER) pattern. We investigated several repolarization parameters including the Tpeak-Tend interval and Tpeak-Tend/QT ratio in healthy individuals and patients with J wave syndrome who were aborted from sudden cardiac death. METHODS AND RESULTS: Ninety-two subjects were enrolled: 12 patients with ventricular fibrillation associated with J waves, 40 healthy subjects with an uneventful ER pattern and 40 healthy control subjects (C) without any evident J waves. Using ambulatory electrocardiogram recordings, the average QT interval, corrected QT interval (QTc), Tpeak-Tend (Tp-e) interval, which is the interval from the peak to the end of the T wave, and Tp-e/QT ratio were calculated. Using ANOVA and post hoc analysis, there was no significant difference in the average QT and QTc in all 3 groups (QT; 396 +/- 27 vs 405 +/- 27 vs 403 +/- 27 m, QTc; 420 +/- 26 vs 421 +/- 21 vs 403 +/- 19 milliseconds in the C, ER pattern and J groups, respectively). The Tp-e interval and Tp-e/QT ratio were significantly more increased in the J wave group than the ER Pattern group (Tp-e: 86.7 +/- 14 milliseconds vs 68 +/- 13.2 milliseconds, P < 0.001, Tp-e/QT; 0.209 +/- 0.04 vs 0.171 +/- 0.03, P < 0.001), but they did not significantly differ between the C and ER pattern groups (Tp-e: 68.6 +/- 7.5 vs 68 +/- 13.2, P = 0.97, Tp-e/QT 0.174 +/- 0.02 vs 0.171 +/- 0.03, P = 0.4). CONCLUSION: As novel markers of heterogeneity of ventricular repolarization, Tpeak-Tend interval and Tp-Te/QT ratio are significantly increased in patients with J wave syndromes compared to age and sex-matched uneventful ER.

DOI： 10.1111/j.1540-8167.2012.02363.x

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We describe a case of early repolarization syndrome in which augmented J waves were documented during an electrical storm associated with hypokalemia. The patient was referred to our hospital for therapy to treat recurrent ventricular fibrillation (VF). The 12-lead electrocardiogram showed giant J waves associated with hypokalemia during multiple episodes of VF. Although antiarrhythmic agents or deep sedation were not effective for the VF, an intravenous supplementation of potassium completely suppressed the VF with a reduction in the J-wave amplitude. Our report discusses the possible relationship between hypokalemia and VF in early repolarization syndrome.

DOI： 10.1111/j.1540-8159.2012.03460.x

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Large infarcts are associated with a terminal QRS-distortion in ST-elevation myocardial infarction (STEMI) patients. Late gadolinium enhancement (LGE) on the cardiac MRI (CMR) can depict an infarct distribution. However, less is known about the relationship between the LGE findings and QRS-distortion on admission, including the best ECG-lead location to reveal the QRS-distortion (DIS-lead) in STEMI patients. Fifty STEMI patients successfully treated with percutaneous coronary intervention were classified into two groups according to whether the QRS-distortion was positive (+) or negative (-). The LGE on a recent CMR was classified into 12 left ventricular segments (Basal-Middle-Apical x Anterior-Septal-Inferior-Lateral). The coincidences between the segmental LGE scores and DIS-lead were investigated. All patients were divided into 23 QRS-distortion (+) and 27 QRS-distortion (-) groups. The total LGE score was significantly greater in the QRS-distortion (+) group (14.7 +/- 6.8 versus 9.6 +/- 6.2, P < 0.01). The highest LGE score in 96% of QRS-distortion (+) patients was 4, and a score 4 segment indicated a good selection of the DIS-lead (86.4%). QRS-distortion in the ECG on admission represents severe transmural infarction in the LGE using CMR, which represents large infarcts in STEMI patients.

DOI： 10.1536/ihj.53.270

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We describe a case of advanced atrioventricular (AV) block, in which treatment with cilostazol was effective in recovering the AV conduction. The patient was referred to our hospital for close examination of the advanced AV block and permanent pacemaker implantation. Although the patient had experienced third-degree AV block with occasional AV synchrony for more than two days, the AV conduction completely recovered after treatment with oral cilostazol at 200 mg/day. Here we discuss the possible mechanism of the improvement in the AV conduction by cilostazol.

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Fabry disease is an X-linked lysosomal storage disorder caused by mutations of the alpha-galactosidase A gene (GLA), and the disease is a relatively prevalent cause of left ventricular hypertrophy mimicking idiopathic hypertrophic cardiomyopathy. We assessed clinically 5 patients of a three-generation family and also searched for GLA mutations in 10 family members. The proband had left ventricular hypertrophy with localized thinning in the basal posterior wall and late gadolinium enhancement (LGE) in the near-circumferential wall in cardiovascular magnetic resonance images and her sister had vasospastic angina pectoris without organic stenosis of the coronary arteries. LGE notably appeared in parallel with decreased alpha-galactosidase A activity and increased NT-pro BNP in our patients. We detected a new GLA missense mutation (G195V) in exon 4, resulting in a glycine-to-valine substitution. Of the 10 family members, 5 family members each were positive and negative for this mutation. These new data extend our clinical and molecular knowledge of GLA gene mutations and confirm that a novel missense mutation in the GLA gene is important not only for a precise diagnosis of heterozygous status, but also for confirming relatives who are negative for this mutation.

DOI： 10.1536/ihj.52.308

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Systemic capillary leak syndrome (SCLS) is a life-threatening disorder which presents with periodic episodes of hypovolemic shock, due to plasma leakage to the extra-vascular space reflected by accompanying hypoalbuminemia, hemoconcentration and edema often with associated monoclonal gammopathy. We describe a 28-year-old woman with SCLS who required aggressive fluid resuscitation and was successfully treated with corticosteroid, terbutaline, and theophylline. At exacerbation, the levels of serum granulocyte colony-stimulating factor (G-CSF) were increased. Thus, G-CSF might play an important role and can be a useful biomarker for the severity of attacks in SCLS.

DOI： 10.2169/internalmedicine.50.4857

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Recent evidence suggests an association between vasospastic angina and Brugada syndrome. Here we present two cases of coronary artery disease who presented with ECG abnormalities which might have been provoked or enhanced by ischemia of the conus branch of the right coronary artery. The 12-lead ECGs demonstrated normal sinus rhythm in these two cases. Interestingly, a saddle back or coved type ST segment elevation in leads V1-V3 was documented either in the percutaneous transluminal angioplasty procedure of the proximal right coronary artery or with an intracoronary acetylcholine (Ach) administration into the right coronary artery. These Brugada type ECG changes were restored to the baseline ECG waveform after improvement in the ischemia. In the second case, vasospasms of the conus branch of the right coronary artery were associated with a coved type ST segment elevation in leads V1 to V2. We discuss the possible interaction between ischemia caused by conus branch lesions and Brugada type electrocardiographic changes.

DOI： 10.1536/ihj.51.68

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We describe a thought-provoking case of Brugada syndrome in which a relationship between the diurnal electrocardiogram (ECG) changes and sex hormone levels was observed. A 36-year-old man who experienced cardiac arrest was referred to our hospital. He had a family history of sudden cardiac death. The 12-lead ECG exhibited a mild coved type ST-segment elevation in leads V1 and V2, which was enhanced by intravenous pilsicainide injection. Hence, this case was diagnosed as Brugada syndrome. The circadian rhythm of the serum testosterone level revealed low levels in the daytime (1.66-1.99 ng/mL) and high levels (2.52-3.42 ng/mL) in the nighttime. Interestingly, augmentation of the ST segment elevation and widening of the P wave were observed at around 2:00 AM, when the serum testosterone was recorded at its highest. Our report discusses the influence of the circadian rhythms of sex hormones on the ECG changes in Brugada syndrome.

DOI： 10.1536/ihj.50.669

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The purpose of this study was to investigate whether the ideal control of atrial fibrillation (AF) associated with hypertensive patients depends on the usage of renin-angiotensin system (RAS) inhibitors or whether it occurs regardless of the kind of antihypertensive agents used. The control of AF was compared in 112 outpatients between 1) those with or without the administration of RAS inhibitors, and 2) those with an ideal or poor control of the blood pressure (BP) regardless of the kind of antihypertensive therapy used. The therapies with or without RAS inhibitors did not yield any significant difference in the AF control states, even though RAS inhibitors had been administered to the patient group with a high proportion of organic heart disease. The ideal BP control group exhibited a significantly better AF control in comparison to the poor BP control group. The former group had a significantly smaller left atrial diameter determined by ultrasonic echocardiography. BP control itself may essentially be important for preventing AF in the general patient population. Poor BP control seemed to have an affect on worsening AF possibly via left ventricular diastolic dysfunction, followed by left atrial overload.

DOI： 10.1536/ihj.50.445

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We describe a case of Brugada syndrome, in which recurrent syncope with convulsive seizures was induced after antidepressant treatment. The patient had been treated with five kinds of psychotropic drugs. The twelve-lead ECG after the syncope exhibited an RSR'-pattern in the precordial leads, however, a coved type ST-segment elevation was induced by a pilsicainide test. Although ventricular fibrillation was not induced in the electrophysiologic study, an ICD implantation was considered as the recommended therapy since Brugada syndrome unmasked by antidepressants could not be ruled out. The possible contribution of antidepressants to Brugada type ST-segment changes is discussed.

DOI： 10.2169/internalmedicine.48.2370

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We describe a case of Brugada syndrome, in which a coved type ST-segment elevation was enhanced by antihistamines and antiallergenic drugs. The patient had been treated with four kinds of antihistamines and antiallergenic drugs. The twelve-lead ECG exhibited a coved type ST-segment elevation in leads V(1) and V(2), and their enhancement was induced by pilsicainide. After discontinuing those drugs, the ST segment elevation in leads V(1) and V(2) became reduced. An ICD implantation was selected for the therapy since ventricular fibrillation was induced. Our report discusses the possible contribution of antihistamines and antiallergenic drugs to the Brugada type ST-segment changes.

DOI： 10.2169/internalmedicine.48.2067

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A 47-year-old man was admitted to our hospital complaining of chest pain at rest in the early morning. Electrocardiography showed ST segment elevation in leads II, III and aVF. Emergency coronary angiography revealed total occlusion of the right coronary artery at the proximal portion. Intracoronary administration of isosorbide dinitrate successfully recanalized the right coronary artery. However, there was a thrombus image at the culprit lesion. Intracoronary administration of urokinase caused the residual thrombus to disappear completely. Follow-up coronary angiography at 1 week and 3 months revealed no organic stenotic lesion. Intravascular ultrasound showed only a little plaque without signs of ruptured plaque in the right coronary artery. Provocation coronary angiography revealed remarkable spasm causing total occlusion at the proximal portion of the right coronary artery. This case suggests that only severe coronary spasm without plaque rupture could form a thrombus causing acute coronary syndrome.

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Language：Japanese   Publishing type：Research paper (scientific journal)  

BACKGROUND: Delayed-enhancement MRI is a technique that has significant clinical usefulness, particularly for myocardial viability determination in ischemic heart disease. Delayed enhanced images have been acquired by using the inversion recovery(IR)method. It is necessary for the IR method to select optimal inversion time(TI). Recently, the phase-sensitive inversion recovery(PSIR)method has been developed to detect Gd-DTPA enhanced myocardium. PURPOSE: To compare the IR method with the PSIR method by acquiring Gd-DTPA solution phantoms A(0.05 mmol/l)and B(0.04 mmol/l)in various parameters. METHOD: Images were acquired using a turbo-fast low angle shot(t-flash)sequence in each method. RESULTS: The null point of signal intensity(SI)shortened as the flip angle(FA)and segments increased in the IR method. Excess segments also caused the duration of breath holding to be extended. The IR method might cause reversed SI between normal and Gd-DTPA enhanced myocardium if the optimal TI was not carefully selected. On the other hand, PSIR had no problem in obtaining phase-sensitive images that were converted into positive SI keeping inverse longitudinal magnetization. The PSIR method avoided the need to select optimal TI and loss of contrast. CONCLUSION: The PSIR method was a useful sequence for delayed-enhancement MRI to detect Gd-DTPA enhanced myocardium.

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Language：Japanese   Publishing type：Research paper (scientific journal)  

OBJECTIVES: This clinical study investigated the prevalence of cervical and cerebral atherosclerosis and silent brain infarction in patients with coronary artery disease. METHODS: Cervical and cerebral magnetic resonance angiography(MRA) was performed in 133 patients (98 males, 35 females, mean age 65.3 years) with suspected coronary artery disease, who were divided into a zero- and one-vessel disease group(n = 71) and a two- and three-vessel disease group(n = 62) depending on the number of major coronary branches with 75% or more stenosis. The MRA lesion was defined as more than 50% stenosis. Magnetic resonance imaging(MRI) of the brain was performed within 1 week of MRA in 78 patients without symptomatic stroke and atrial fibrillation. Silent brain infarction on MRI was defined as a focal high intensity area on T2-weighted images larger than 3 mm. RESULTS: The prevalence of MRA lesions was significantly greater in the two- and three-vessel group than in the zero- and one-vessel group(53% vs 14%, p < 0.01). The prevalence of MRI lesion was significantly higher in the two- and three-vessel group than in the zero- and one-vessel group(77% vs 36%, p < 0.01). The size and number of the MRI lesions were also significantly greater in the two- and three-vessel group than in the zero- and one-vessel group(p < 0.01). Neither age nor percentage of male gender was different between the groups. Diabetes mellitus was the common risk factor for coronary artery disease, MRA lesion and MRI lesion. CONCLUSIONS: Cervical and cerebral atherosclerosis and silent brain infarction are frequently observed in patients with multivessel coronary artery disease.

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Language：English   Publishing type：Research paper (scientific journal)  

The authors investigated bipolar electrograms recorded from the catheter tip at the actual successful ablation sites in 22 consecutive patients with concealed Wolff-Parkinson-White syndrome to clarify the characteristics of the potentials indicating the optimal site for catheter ablation. In all patients the retrograde transaortic approach to their left-sided accessory pathways, and a temperature-controlled (60 degrees C) energy delivery, were performed. The authors assumed that a shorter dissociation time (time from energy delivery to ventriculoatrial conduction dissociation) indicated more accurate catheter mapping. A significant negative correlation (r = 0.527, p < 0.05) between the AV ratio (ratio of the amplitudes of the atrial to ventricular potentials) recorded at the ablation catheter tip and the dissociation time was observed. When the AV ratio and the dissociation time were compared among the groups classified according to the corresponding Npeak (the number of positive potential peaks in the electrogram obtained from the ablation catheter tip during right ventricular apical pacing) value, they differed significantly (p < 0.05 and p < 0.01, respectively), ie, a higher AV ratio and a shorter dissociation time related to a multipeak electrogram from the ablation catheter tip. The authors conclude that the atrial insertion site of the accessory pathway, exhibiting a multipeak complex electrogram that may represent nonuniform anisotropic characteristics, is an adequate ablation site.

DOI： 10.1177/000331979905000807

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Authorship：Corresponding author   Language：Japanese   Publishing type：Research paper (scientific journal)  

We have studied function of the corpus callosum in a patient with spastic paraparesis with mental deterioration and markedly thin corpus callosum using magnetic stimulation methods. In a 21-year-old woman with slowly progressive gait disturbance, neurological examination showed mental deterioration, euphoria, spastic paraparesis, bilateral Babinski's sign, and hyperesthesia caudal to the eighth thoracic level. No abnormalities were observed in electroencephalograms. Magnetic resonance imaging (MRI) studies of the brain showed cerebral cortical atrophy, markedly thin corpus callosum, and dilated cavum septum pellucidum and cavum Vergae, but spinal cord MRIs showed no abnormalities. The lysosomal enzyme activities, whose reduction was known to cause leukodystrophy, were all normal. Very long chain fatty acid was not increased in her blood, which is against adrenoleukodystrophy. She had no anti-HTLV-1 virus antibody. Based on these clinical features and the results of biochemical analyses, we diagnosed this patient as having spastic paraplegia associated with hypoplasia of the corpus callosum (Nojima and Iwabuchi). We performed three studies on the central motor pathways in this patient. The latencies of responses recorded from upper or lower limb muscles were all within the normal range, despite that the thresholds were slightly increased. This suggests that axonal degeneration occurs in the central motor pathways, which is consistent with the autopsy findings of a patient with hereditary spastic paraplegia associated with hypoplasia of the corpus callosum. Connection between the bilateral motor cortices was investigated by magnetic stimulation of both motor cortices. The suppression of the motor cortex evoked by stimulation of the contralateral motor cortex through the corpus callosum was absent in this patient. Intracortical inhibition within the motor cortex was demonstrated to be normal by a paired-magnetic stimulation technique. Based on the results of these results of these two experiments, we conclude that the function of the corpus callosum was disturbed in the present patient. This report first shows the functional abnormality of the extremely thin corpus callosum in a patient with hereditary spastic paraplegia associated with hypoplasia of the corpus callosum.

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Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (trade magazine, newspaper, online media)   Publisher：科学評論社  

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Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (trade magazine, newspaper, online media)   Publisher：メジカルビュー社  

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Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (trade magazine, newspaper, online media)   Publisher：自然科学社  

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Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (trade magazine, newspaper, online media)   Publisher：科学評論社  

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Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (trade magazine, newspaper, online media)   Publisher：最新医学社  

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Event date： 2015.6

Language：English   Presentation type：Poster presentation  

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Event date： 2014.9

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Event date： 2006.11

Language：English   Presentation type：Oral presentation (general)  

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Event date： 2006.5

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Event date： 2005.11

Language：English   Presentation type：Poster presentation  

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