International Heart Journal
Online ISSN : 1349-3299
Print ISSN : 1349-2365
ISSN-L : 1349-2365
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Editorials
Clinical Studies
  • Masayuki Kubota, Kenichi Sakakura, Kei Yamamoto, Yousuke Taniguchi, Ta ...
    2020 Volume 61 Issue 5 Pages 865-871
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Bleeding complication has been considered as a serious problem in current percutaneous coronary interventions (PCI). Fortunately, several groups have already reported the effectiveness of protamine use just after PCI to immediately remove any arterial sheath. However, there is a concern that protamine reversal may increase non-occlusive thrombus and, in turn, lead to mid-term cardiovascular events such as target vessel revascularization (TVR) or stent thrombosis. Thus, the purpose of this study was to evaluate whether protamine use following elective PCI was associated with mid-term clinical outcomes. In total, 472 patients were included in this study; subsequently, they were divided into protamine group (n = 142) and non-protamine group (n = 330). The primary endpoint was the composite of ischemia-driven TVR and stent thrombosis. The median follow-up period was determined to be at 562 days. In total, 32 primary endpoints were observed during the study period, and the incidence of primary endpoints tended to be greater in the protamine group than in the non-protamine group (P = 0.056). However, the lesion length, the degree of calcification, and the prevalence of hemodialysis were significantly determined greater in the protamine group than in the non-protamine group. In the multivariate Cox proportional hazards model, the use of protamine (versus non-protamine: hazard ratio 0.542 and 95% confidence interval 0.217-1.355, P = 0.191) was deemed not to be associated with the primary endpoint after controlling legion length, calcification, and hemodialysis. In conclusion, immediate protamine use following elective PCI did not increase mid-term ischemia-driven TVR or stent thrombosis. However, immediate protamine use after PCI should be discussed further for the safety of the patient.

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  • Lingbo Meng, Xianglan Liu, Huai Yu, Guo Wei, Xia Gu, Xiaoshuang Chang, ...
    2020 Volume 61 Issue 5 Pages 872-878
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    In-stent restenosis (ISR) still exists after drug-eluting stent (DES) implantation, even up to one year. The incidence and risk factors for neoatherosclerosis in patients with early ISR have not yet been elucidated. Here, we used optical coherence tomography (OCT) to evaluate the incidence and predictors of neoatherosclerosis in patients with early ISRs.

    OCT was performed on ISR lesions in 185 patients in order to detect neoatherosclerosis. The median follow-up was 180 days, and neoatherosclerosis was detected in 37% of early ISR lesions. According to the presence of neoatherosclerosis, patients with ISR were divided into two groups: neoatherosclerosis (group A, n = 69) and non-neoatherosclerosis (group B, n = 116) groups.

    The risk factors were similar, except for hypercholesterolemia. Moreover, the tissue characteristics were not significantly different between patients with and without neoatherosclerosis. Follow-up low-density lipoprotein-cholesterol (LDL-C) levels were divided into three grades (LDL < 70 mg/dL, 70 mg/dL≤ LDL < 100 mg/dL, and LDL ≥ 100 mg/dL). The incidence of neoatherosclerosis was significantly lower (23% versus 57%, P < 0.0001) in the LDL < 70 mg/dL group. There was no significant difference in the incidence of neoatherosclerosis in patients with lipid levels between 70 and 100 mg/dL (P = 0.53). However, neoatherosclerosis was significantly more common in patients with a follow-up LDL-C level > 100 mg/dL (45% versus 15%, P < 0.0001).

    In patients with early ISR lesions, the LDL-C levels may be related to the formation and progression of early neoatherosclerosis, and poor LDL-C control may be a risk factor for the occurrence of early-stage neoatherosclerosis following DES implantation.

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  • Masaru Seguchi, Kenichi Sakakura, Takunori Tsukui, Kei Yamamoto, Yousu ...
    2020 Volume 61 Issue 5 Pages 879-887
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Since the number of elderly patients suffering from acute myocardial infarction (AMI) has been increasing in developed countries, primary percutaneous coronary intervention (PCI) for the very elderly aged ≥80 years old is already common. The study aimed to examine the determinants of in-hospital death among the very elderly with AMI in current PCI era. We included 412 consecutive AMI patients aged ≥ 80 years old who received PCI to the culprit lesion; however, 42 patients (10.2%) died during the index hospitalization. Thus, univariate and multivariate logistic regression analyses were performed to identify the determinants of in-hospital death. Of note, the modified KATZ index, which is a seven-point scale ranging from 0 to 6 (0 point indicating no dependence and six points indicating full dependence), was calculated to evaluate pre-admission activity of daily living (ADL). In multivariate analysis, cardiac arrest (OR 4.642, 95% CI 1.177-18.305, P = 0.028), Killip class IV (versus Killip class I: OR 5.732, 95% CI 1.076-16.630, P = 0.001), modified KATZ index (OR 1.212, 95% CI 1.001-1.469, P = 0.049), hemoglobin levels (OR 0.803, 95% CI 0.656-0.983, P = 0.033), use of temporary pacemaker (OR 2.603, 95% CI 1.010-6.709, P = 0.048), final Thrombolysis In Myocardial Infarction (TIMI) flow grade 3 (versus TIMI ≤ 2: OR 0.240, 95% CI 0.093-0.618, P = 0.003), and mechanical circulatory support (OR 4.264, 95% CI 1.818-10.005, P = 0.001) were found to be significantly associated with in-hospital death. In conclusion, in-hospital outcomes of the very elderly with AMI were still poor in the current PCI era. Poor pre-admission ADL as well as cardiogenic shock and anemia were determined to be strongly associated with in-hospital death.

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  • A Japanese registry of acute Myocardial INfarction diagnosed by Universal dEfiniTion (J-MINUET) Substudy
    Norihito Takahashi, Manabu Ogita, Satoru Suwa, Koichi Nakao, Yukio Oza ...
    2020 Volume 61 Issue 5 Pages 888-895
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Although B-type natriuretic peptide (BNP) has gradually gained recognition as an indicator in risk stratification for patients with acute myocardial infarction (AMI), the prognostic impact on long-term clinical outcomes in patients with non-ST-segment elevation acute myocardial infarction (NSTEMI) without creatine kinase (CK) elevation remains unclear.

    This prospective multicenter study assessed 3,283 consecutive patients with AMI admitted to 28 institutions in Japan between 2012 and 2014. We analyzed 218 patients with NSTEMI without CK elevation (NSTEMI-CK) for whom BNP was available. In the NSTEMI-CK group, patients were assigned to high- and low-BNP groups according to BNP values (cut-off BNP, 100 pg/mL). The primary endpoint was defined as a composite of all-cause death, non-fatal myocardial infarction, non-fatal stroke, cardiac failure, and urgent revascularization for unstable angina up to 3 years. Primary endpoints were observed in 60 (33.3%) events among patients with NSTEMI-CK. Kaplan-Meier analysis revealed a significantly higher event rate for primary endpoints among patients with high BNP (log-rank P < 0.001). After adjusting for covariates, a higher BNP level was significantly associated with long-term clinical outcomes in NSTEMI-CK (adjusted hazard ratio, 4.86; 95% confidence interval, 2.18-12.44; P < 0.001).

    The BNP concentration is associated with adverse long-term clinical outcomes among patients with NSTEMI-CK who are considered low risk. Careful clinical management may be warranted for secondary prevention in patients with NSTEMI-CK with high BNP levels.

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  • Kojiro Ogawa, Miyako Igarashi, Akihiko Nogami, Masayoshi Yamamoto, Aki ...
    2020 Volume 61 Issue 5 Pages 896-904
    Published: September 29, 2020
    Released: September 29, 2020
    JOURNALS FREE ACCESS

    Identifying the optimal atrioventricular (AV) or interventricular (VV) delay is beneficial for patients using cardiac resynchronization therapy (CRT) devices. Ultrasonic echocardiography (UCG) has been the most commonly used method; however, it requires high technical knowledge. Impedance cardiography (ICG) can calculate stroke volume by measuring changes in transthoracic electric impedance. This study sought to assess the clinical utility of ICG in comparison with that of UCG for the optimization of CRT devices.

    Patients who underwent CRT device implantation were retrospectively analyzed. One week after implantation, optimization of AV delay (AVD) was performed in every patient with ICG (AVD-ICG) and UCG (AVD-UCG). VV delay (VVD) was then determined according to the optimal AVD using these two methods.

    Forty-two patients were enrolled. Average AVD-ICG was significantly shorter than AVD-UCG (128 ± 49 versus 146 ± 41 milliseconds, P = 0.018). Five patients (12%) had the same optimized AVD with two methods, and the difference between AVD-ICG and AVD-UCG was ≤ 20 milliseconds in 19 patients (45%). In the multivariate analysis, the presence of postoperative mitral regurgitation (MR) was an independent predictor of AVD-ICG/AVD-UCG mismatch, defined as a difference over 20 milliseconds (odds ratio = 10.71; 95% confidence interval = 1.72 to 66.72; P = 0.018). The results of optimized VVD were similar using both methods.

    ICG might be a promising tool for the rapid optimization of CRT devices. However, in patients with moderate-to-severe MR, ICG may not be able to optimize AVD.

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  • Mihoko Kawabata, Masahiko Goya, Shingo Maeda, Atsuhiko Yagishita, Yosh ...
    2020 Volume 61 Issue 5 Pages 905-912
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    There is little data on management and outcomes of atrial fibrillation (AF) patients on direct oral anticoagulants (DOAC) undergoing general surgery.

    We retrospectively assessed 98 surgeries in 85 nonvalvular AF patients aged 73 ± 8 (59 men) receiving DOACs. Cardiac, emergency, and minimally invasive surgeries were excluded.

    The CHA2DS2-VASc score ranged from 0 to 8. The DOACs being given were: dabigatran, 16; rivaroxaban, 25; apixaban, 28; and edoxaban, 16. While the DOACs were not suspended in 11 cases, they were interrupted for a median of 2.0 days before surgery and restarted at a median of 3.0 days after surgery. There were 9 complications (9.2%), 3 instances of thromboembolism and 6 bleeding. Thromboembolism occurred at a mean of 3.0 postoperative days, all of which occurred before resumption of DOACs, while bleeding events occurred at a mean of 4.0 postoperative days. Two of the 3 patients with thromboembolism went into cardiopulmonary arrest during the event, but were resuscitated. There were significantly more patients with congestive heart failure or combined antiplatelets in the patients with complications. The complication group had a significantly higher HAS-BLED score and lower preoperative hemoglobin level. There were no significant differences in the management of DOAC interruption between those with complications and without.

    The perioperative complication rate in nonvalvular AF patients undergoing elective surgery treating with DOACs was 9.2%. Patients with congestive heart failure, receiving combined therapy with antiplatelets, a higher HAS-BLED score, or lower preoperative hemoglobin level were at higher risk. Further studies evaluating the ideal perioperative DOAC protocol are warranted.

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  • Kenta Tsutsui, Ritsushi Kato, Sou Asano, Yoshifumi Ikeda, Hitoshi Mori ...
    2020 Volume 61 Issue 5 Pages 913-921
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Previous study has identified marked differences in patient characteristics and causes of inappropriate shock (IAS) between Japan and the Western societies in terms of subcutaneous implantable cardioverter-defibrillator (S-ICD). However, evidence of IAS in Asian populations including Japan has been limited to one observational study.

    Thus, we conducted a single-center registry study that tracks the postoperative course of 61 consecutive patients who received S-ICD from February 2016 to January 2020. Our findings showed that IAS occurred in 9.8% of the study population (6/61), which is comparable to the previously reported incidence. Remarkably, T-wave oversensing did not result in an IAS (0/6). Instead, myopotential oversensing was determined to have caused the most IAS events (4/6), while atrial fibrillation ranked second (2/6). A provocation maneuver (e.g., abdominal clench, push-ups, lifting a heavy item) reproduced myopotential noise disguised as R-waves, which should potentially trigger an IAS if uninterrupted. R-wave amplitude of the IAS group appeared relatively low compared to that of the non-IAS group although this finding was not tested significant. Furthermore, no temporal changes were noted in R-wave amplitude between the time of implantation and IAS events, suggesting that it is neither constantly low nor acutely dropped R-wave amplitude but a relatively high noise level that drives IAS. All the myopotential-IAS patients were found to be male. Right-sided lead implantation was associated with a higher incidence of IAS.

    This study highlights the fact that IAS continues to occur due to myopotential noise oversensing instead of T-wave oversensing. To minimize the risk of IAS, it is desirable to search and secure high R-wave voltage.

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  • Junya Hosoda, Toshiyuki Ishikawa, Katsumi Matsumoto, Masayoshi Kiyokun ...
    2020 Volume 61 Issue 5 Pages 922-926
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    The incidence of ventricular arrhythmia in patients with an implanted pacemaker is not yet known. The aim of this study was to analyze non-sustained ventricular tachycardia (NSVT) episodes based on stored electrograms (EGM) and determine the occurrence rate and risk factors for NSVT in a pacemaker population.

    This study included 302 consecutive patients with a dual-chamber pacemaker. A total of 1024 EGMs stored in pacemakers as ventricular high-rate episodes were analyzed. The definition of NSVT was ≥ 5 consecutive ventricular beats at ≥ 150 bpm lasting < 30 seconds.

    In baseline, most patients (94.8%) had ≥ 60% left ventricular ejection fraction. Of 1024 EGMs, 420 (41.0%) showed appropriate NSVT episodes, as well as premature atrial contractions, atrial tachyarrhythmia, or atrial fibrillation with a rapid ventricular response, whereas other EGMs did not show an actual ventricular arrhythmia. On EGM analysis, during a mean follow-up period of 46.1 months, NSVT occurred one or more times in 82 patients (33.1%). On multivariate analysis, ≥ 50% right ventricular pacing was an independent risk factor for NSVT (odds ratios, 4.519; P < 0.001), but NSVT was not associated with increased all-cause mortality.

    Moreover, in the pacemaker population, ≥ 50% right ventricular pacing is an independent risk factor for NSVT; however, NSVT was not associated with increased all-cause mortality because of the preserved left ventricular function.

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  • Yuki Shirakawa, Shinichi Niwano, Jun Oikawa, Daiki Saito, Tetsuro Sato ...
    2020 Volume 61 Issue 5 Pages 927-935
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 02, 2020
    JOURNALS FREE ACCESS

    We prospectively collected device and heart rate data through remote monitoring (RM) of patients with an implantable cardioverter defibrillator (ICD). The objective was to identify the predictors of lethal arrhythmic events (VT/VF).

    Thirty-three patients (mean age: 50 years) with ICDs [with functionality of heart rate variability (HRV) analysis] were divided into two groups [VT/VF (+), VT/VF (−) ]. Clinical, device (ventricular lead impedance; amplitude of ventricular electrogram), and HRV data were compared between the two groups. The NN interval-index (SDNNi) was calculated for every 5 minutes, and the mean, maximum, minimum, and standard deviation of SDNNi during the 24-hour period were used.

    During the observation period of 13 ± 10 months, 10 patients experienced VT/VF events. Total mean, max, and min SDNNi were higher in the VT/VF (+) than the VT/VF (−) group (132.9 ± 9.3 versus 93.5 ± 6.1, P = 0.0013; 214.6 ± 10.6 versus 167.0 ± 7.0, P = 0.0007; 71.2 ± 7.5 versus 43.9 ± 4.9, P = 0.0047). On logistic regression analysis, a total mean SDNNi of 100.1, max SDNNi of 185.0 and min SDNNi of 52.0 as cut-off values for prediction of a VT/VF event demonstrated significant receiver operating characteristic (ROC) curves (AUC = 0.86, P = 0.0007; AUC = 0.84, P = 0.0005; AUC = 0.78, P = 0.0030). The max ΔSDNNi, i.e., difference from baseline SDNNi, and min ΔSDNNi in 7 and 28 days preceding VT/VF events were significant predictors of VT/VF events.

    Time-domain HRV analysis through a RM system may help identify patients at high risk of lethal arrhythmic events; in addition, it may help predict the occurrence of lethal arrhythmic events in specific cases.

    Editor’s picks

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  • Yu Xia, Jun Liu, Yuhe Jia, Hao Zhang, Miao Yu, Xiaofeng Li, Pihua Fang
    2020 Volume 61 Issue 5 Pages 936-943
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 02, 2020
    JOURNALS FREE ACCESS

    On the basis of radiofrequency ablation of atrial fibrillation (AF), some studies suggested that early recurrences of atrial tachyarrhythmia (ERATs) were associated with late AF recurrence (LAFR), and some also suspected and challenged the current recommended 90 day blanking period. We aim to evaluate the impact of ERAT on long-term success and to determine the optimum blanking period after AF ablation using second-generation cryoballoon (sg-CB). From August 2016 to October 2018, 369 consecutive patients who successfully underwent initial AF ablation using sg-CB at the Fuwai Hospital were finally enrolled. All patients were followed up no less than 12 months. Receiver operating characteristic curve analysis was used to determine the optimum blanking period after AF ablation. There were 62 (16.8%) who experienced ERAT. After a median follow-up of 615 days, 74.5% were free of LAFR after the 90 day blanking period. Incidence of freedom from LAFR during the long-term follow-up was markedly lower in patients with ERAT than in those without ERAT (27.4% versus 84.0%; log-rank P < 0.001). Furthermore, only ERAT (HR 8.579; 95% CI 5.604-13.133; P < 0.001) was significantly associated with an increased risk of LAFR after adjusting for other factors. The optimum cut-off time point for the blanking period was 21.5 days (sensitivity: 71.1%, specificity: 94.1%). In conclusion, ERAT was an independent predictor of LAFR after AF ablation using sg-CB. Based on our findings, blanking period was advised to be shorten to 21.5 days or about 3 weeks instead of 90 days after CB ablation.

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  • Masaya Shinohara, Ryou Wada, Kensuke Yano, Katsuya Akitsu, Hideki Koik ...
    2020 Volume 61 Issue 5 Pages 944-950
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Clinical experience with landiolol use in patients with atrial fibrillation (AF) and a severely depressed left ventricular (LV) function is limited. We compared the efficacy and safety of landiolol with that of digoxin as an intravenous drug in controlling the heart rate (HR) during AF associated with a very low LV ejection fraction (LVEF).

    We retrospectively analyzed 53 patients treated with landiolol (n = 34) or digoxin (n = 19) for AF tachycardias with an LVEF ≤ 25. The landiolol dose was adjusted between 0.5 and 10 μg/kg/minute according to the patient's condition. The response to treatment was defined as a decrease in the HR of ≤ 110/minute, and that decreased by ≥ 20% from baseline.

    There were no significant differences between the two groups regarding the clinical characteristics. The responder rate to landiolol at 24 hours was significantly higher than that to digoxin (71.0% versus 41.2%; odds ratio: 4.65, 95% confidence interval: 1.47-31.0, P = 0.048). The percent decrease in the HR from baseline at 1, 2, 12, and 24 hours was greater in the landiolol group than in the digoxin group (P < 0.01, P = 0.071, P = 0.036, and P = 0.016, respectively). The systolic blood pressure (SBP) from baseline within 24 hours after administering landiolol was significantly reduced, whereas digoxin did not decrease the SBP over time. Hypotension (< 80 mmHg) occurred in two patients in the landiolol group and 0 in the digoxin group (P = 0.53).

    Landiolol could be more effective in controlling the AF HR than digoxin even in patients with severely depressed LV function. However, careful hemodynamic monitoring is necessary when administering landiolol.

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  • A Meta-Analysis
    Yi Lu, Jianfang Wang, Zexin Chen, Jia Wei, Fei Li, Zhejun Cai
    2020 Volume 61 Issue 5 Pages 951-960
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 02, 2020
    JOURNALS FREE ACCESS
    Supplementary material

    The aim of this meta-analysis was to compare the clinical outcomes in patients who underwent rapid deployment aortic valve replacement (RDAVR) and conventional bio prosthetic aortic valve replacement (CAVR).

    We performed a literature search by August 2018. The primary outcomes were hospital and 1-year mortality, and the secondary endpoints included the aortic cross-clamp (ACC), cardiopulmonary bypass (CPB) time, and postoperative and valve-related complications.

    Two randomized controlled trials and 13 propensity score-matched studies were included. There was no difference between RDAVR and CAVR in hospital mortality (2.5% versus 2.1%; risk ratio (RR) 1.16 [95% confidence interval (CI) 0.80-1.68]) or 1-year mortality (2.9% versus 4.1%; RR 0.69 [95% CI 0.34-1.34]). RDAVR significantly reduced the ACC time ( (mean difference (MD) −24.33 [95% CI −28.35 to −20.32]) and CPB time (MD −21.51 [95% CI −22.83 to −20.20]). The pooled analysis showed that RDAVR doubled the occurrence of permanent pacemaker implantation (8.6% versus 4.3%; RR 2.05 [95% CI 1.62-2.60]). Meanwhile, the blood transfusion amount (MD −1.54 [95% CI −2.22 to −0.86]) and postoperative atrial fibrillation (POAF) occurrence (RR 0.83 [95% CI 0.69-0.99]) was reduced. The difference of paravalvular leakage frequency between RDAVR and CAVR was marginal (RR 1.77 [95% CI 1.00-3.17]; P = 0.05). Furthermore, RDAVR was related to larger valves (MD 0.70 cm [95% CI 0.33-1.07]) and lower mean pressure gradients (MD −1.93 mmHg [95% CI −3.58 to −0.28]).

    The hospital and 1-year survival rates between RDAVR and CAVR are comparable. RDAVR reduces POAF occurrence and blood transfusion but is associated with a higher occurrence of pacemaker implantation.

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  • Ryo Ninomiya, Makoto Orii, Jumpei Fujiwara, Michiko Yoshizawa, Yoshifu ...
    2020 Volume 61 Issue 5 Pages 961-969
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Left ventricular (LV) remodeling with aortic stenosis (AS) appears to differ according to sex, but reverse remodeling after transcatheter aortic valve implantation (TAVI) has not been elucidated in a Japanese population. This study aims to determine whether any sex-related differences in LV or reverse remodeling after TAVI exist in the context of severe AS.

    Of 208 patients who received TAVI for severe AS in our institution, 100 (men, 42; mean age, 83.0 ± 4.9 years) underwent transthoracic echocardiography before and 3 months after TAVI. Despite similar valvular gradients, women with severe AS had lower indexed LV mass (LVMi) than did men (152.3 ± 35.4 versus 173.2 ± 44.6 g/m2, P = 0.005), with smaller indexed LV end-diastolic (LVEDVi) (50.2 ± 13.3 versus 61.4 ± 20.7 mL/m2, P = 0.001) and end-systolic (LVESVi; 17.9 ± 8.7 versus 24.3 ± 13.8 mL/m2, P = 0.006) volumes. After TAVI, women (−6.0% ± 14.4%) had higher reduction in the rate of change of relative wall thickness (RWT) than did men (4.4% ± 19.0%, P = 0.003). Men (−8.9% ± 3.9%) had higher reduction in the rate of change of LVEDVi than did women (1.5% ± 3.3%, P = 0.045). Incidence of LV reverse remodeling defined as a reduction in LVESV of >15% was significantly higher in men (50%) than in women (26%, P = 0.013).

    In addition to sex differences in the pattern of LV remodeling with AS, reverse LV remodeling after TAVI also differed between sexes.

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  • Soshi Hei, Mai Iwataki, Jeong-Yoon Jang, Hiroshi Kuwaki, Shota Fukuda, ...
    2020 Volume 61 Issue 5 Pages 970-978
    Published: September 29, 2020
    Released: September 29, 2020
    JOURNALS FREE ACCESS

    The mechanism of systolic annular expansion in mitral valve prolapse (MVP) is not clarified. Since annular expansion is systolic outward shift of MV leaflet/chorda tissue complex at superior and outer ends, annular expansion could be related to inward (superior) shift of the complex at another inferior and inner end of the papillary muscle (PM) tip and/or systolic lengthening of the tissue complex, especially MV leaflets.

    MV annulus systolic expansion, PMs' systolic superior shift, and MV leaflets' systolic lengthening were evaluated by echocardiography with a speckle tracking analysis in 25 normal subjects, 25 subjects with holo-systolic MVP and 20 subjects with late-systolic MVP.

    PMs' superior shift, MV leaflets' lengthening, MV annular area at the onset of systole and subsequent MV annulus expansion were significantly greater in late-systolic MVP than in holo-systolic MVP (4.6 ± 1.6 versus 1.5 ± 0.7 mm/m2, 2.5 ± 1.4 versus 0.6 ± 2.0 mm/m2, 6.8 ± 2.5 versus 5.7 ± 1.0 cm2/m2 and 1.6 ± 0.8 versus 0.1 ± 0.5 cm2/m2, P < 0.001, respectively). Multivariate analysis identified MV leaflets' lengthening and PMs' superior shift as independent factors associated with MV annular expansion.

    Conclusions: These results suggest that systolic MV annular expansion in MVP is related to abnormal MV leaflets' lengthening and PMs' superior shift.

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  • Norihiko Oka, Takashi Miyamoto, Takahiro Tomoyasu, Hidenori Hayashi, K ...
    2020 Volume 61 Issue 5 Pages 979-983
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    The Fontan procedure is a palliative surgery performed for patients with complex congenital heart disease who exhibit functional single ventricular physiology. Although clinical outcomes of the Fontan procedure have improved in recent years and most patients who undergo the procedure reach adulthood, Fontan-associated liver disease (FALD) is a noncardiovascular complication that has become increasingly common; its risk factors remain unknown.

    A total of 95 patients who underwent the Fontan procedure and who were followed up for at least three years at Gunma Children's Medical Center and Kitasato University Hospital between 1996 and 2015 were retrospectively enrolled in this study.

    The mean age of the patients at the time of Fontan procedure was 2.3 ± 1.4 years. Overall, 21 patients (23.1%) experienced FALD. All Fontan procedures were performed with extracardiac total cavopulmonary connection using 16-mm expanded polytetrafluoroethylene grafts. The presence of systemic right ventricle, requirement of pulmonary vasodilator, application of a non-fenestrated Fontan procedure, and absence of fenestration flow at the time of follow-up catheter examination were identified as predictors of FALD using univariate analysis. All these factors, except the requirement of pulmonary vasodilator, remained significant predictors of FALD in multivariate logistic regression analysis.

    Patients with a systemic right ventricle who undergo the Fontan procedure are at a high risk of FALD in the mid-term. Creating fenestration at the time of Fontan and maintaining the fenestration flow may reduce the mid-term risk of FALD.

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  • Tomoo Nakata, Satoshi Ikeda, Seiji Koga, Tsuyoshi Yonekura, Akira Tsun ...
    2020 Volume 61 Issue 5 Pages 984-992
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Uric acid is generated with reactive oxygen species via xanthine oxidase (XO), and hyperuricemia, which is identified as the excess of uric acid in the blood, has been associated with vascular endothelial dysfunction. However, the effects of urate-lowering medicines on endothelial function have not been fully elucidated. Thus this study determined and compared the effects of benzbromarone (urate transporter 1 inhibitor) and febuxostat (XO inhibitor) on endothelial function.

    This randomized, cross-over, open-label study initially recruited 30 patients with hyperuricemia. They were divided into two groups, treated initially with benzbromarone or febuxostat for three months and then were switched for the next three months. Endothelial function was defined as reactive hyperemia indexes (RHI) determined using Endo-PAT 2000 before and at three and six months after medication using the two agents. Blood levels of asymmetric dimethylarginine (ADMA) and high-molecular-weight (HMW) adiponectin were also compared. We finally analyzed data from 24 patients whose endothelial function was assessed as described above.

    Our findings show that levels of uric acid significantly decreased, whereas those of HMW adiponectin and the RHI have significantly increased after treatment with benzbromarone. Meanwhile, in patients administered with febuxostat, uric acid levels tended to decrease and RHI significantly decreased. Neither of the two agents altered ADMA levels. The changes in RHI (P = 0.026) and HMW adiponectin levels (P = 0.001) were found to be significantly greater in patients treated with benzbromarone than febuxostat. Changes in the levels of HMW adiponectin and of uric acid were significantly correlated (r = −0.424, P = 0.039).

    Benzbromarone has increased adiponectin besides reducing uric acid levels, and thus, this might confer more benefits on endothelial function than febuxostat.

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  • A Novel Protocol to Prevent Trauma-Related Venous Thromboembolism
    Keita Iyama, Satoshi Ikeda, Takamitsu Inokuma, Shuntaro Sato, Shuhei Y ...
    2020 Volume 61 Issue 5 Pages 993-998
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Venous thromboembolism (VTE) is a life-threatening complication after trauma. Several studies have reported VTE prophylaxis using low-molecular-weight heparin; however, there is no consensus for prophylaxis after trauma. This study aimed to assess the efficacy and safety of our new anticoagulation therapy protocol using unfractionated heparin (UFH) plus intermittent pneumatic compression (IPC) to prevent post-traumatic VTE in high-risk trauma patients.

    This study enrolled 70 trauma patients who were admitted to the emergency medical center of Nagasaki University Hospital and had Risk Assessment Profile (RAP) scores ≥ 5. After stopping bleeding at the trauma site, all patients received intravenous UFH (10,000 U/day) plus IPC, which was continued for 14 days or until the patients could walk. On days 7 and 14, all patients underwent lower extremity sonography for deep-vein thrombosis screening. VTE incidences between patients with the above intervention and historical controls with IPC alone were compared.

    No significant differences in age, sex, and the RAP score were observed between the 105 controls and intervention patients. VTE occurrence was fewer in patients with the intervention (14.3%) than in the controls (28.6%; P = 0.029). No hemorrhagic complications occurred after UFH administration. Multivariable logistic analysis revealed a significant association between the intervention and low incidence of VTE (odds ratio: 0.390; 95% confidence interval: 0.163-0.913; P = 0.030).

    Routine UFH administration with IPC may prevent post-traumatic VTE without adverse events.

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  • Shun Minatsuki, Arihiro Kiyosue, Satoshi Kodera, Kazutoshi Hirose, Aki ...
    2020 Volume 61 Issue 5 Pages 999-1004
    Published: September 29, 2020
    Released: September 29, 2020
    JOURNALS FREE ACCESS

    This study aimed to clarify the usefulness of the Ikari-curve left (IL) guiding catheter for balloon pulmonary angioplasty (BPA).

    The current BPA strategy for chronic thromboembolic pulmonary hypertension is dilation of as many branches as possible to normalize hemodynamics and oxygenation. The shape of the guiding catheter is a major factor in achieving this. However, conventional guiding catheters are difficult to introduce into particular branches. The IL guiding catheter may be suitable; however, its utility remains unclear.

    We retrospectively analyzed 202 consecutive BPA sessions of 40 patients from November 2016 to October 2019 and divided these sessions into two groups: the IL group where the IL guiding catheter was used and the non-IL group where other catheters were utilized. The occurrence of lung injury was determined by the presence of bloody sputum. We compared the rates of successful introduction into target vessels and assessed for the occurrence of lung injury.

    The average age of enrolled patients was 60.3 ± 14.4 years, with females comprising 65%. There were 99 sessions in the IL group. The median treated branches per session differed between the 2 groups (IL group: 15 versus non-IL group: 10, P < 0.05). The occurrence of lung injury was lower in the IL group (4.0% versus 11.7%, P = 0.07). The IL group had more successful vessel insertions than the non-IL group (78.8% versus 42.7%, P < 0.01).

    The IL guiding catheter may be introduced into branches that cannot be accessed by conventional guiding catheters.

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  • A Retrospective Single-Center Study
    Yingxian Liu, Ligang Fang, Wei Chen, Xue Lin, Qian Wang, Yanlin Zhu, H ...
    2020 Volume 61 Issue 5 Pages 1005-1013
    Published: September 29, 2020
    Released: September 29, 2020
    JOURNALS FREE ACCESS
    Supplementary material

    This study sought to evaluate clinical features, treatment patterns, and outcomes of patients with idiopathic inflammatory myopathy (IIM) complicated by heart failure (HF). Thirty-two patients with IIM-HF admitted to the Peking Union Medical College Hospital between January 1999 and January 2018 were retrospectively reviewed, including 14 patients with polymyositis, 11 with dermatomyositis, and 7 with overlap syndrome. Survivors and no-survivors were compared on clinical characteristics and treatment. Although systemic symptoms were variable, all patients presented with elevated troponin I. Rapid atrial arrhythmia was the most frequent arrhythmia. Systolic dysfunction and restrictive diastolic dysfunction were typical presentations in echocardiography. Twenty-nine patients were followed up for a median of 2.8 years (0.1 month to 11 years). We recorded 13 deaths of cardiogenic cause, 1 of serious IIM, and 3 of infective complications. The median survival time from diagnosis of IIM-HF to all-cause mortality was 8.4 months (range from 1 month to 5 years). Both all-cause deaths and cardiogenic deaths were more reported in the methotrexate-alone group than in the combination therapy group (6/7 versus 3/10, P = 0.050; 5/6 versus 2/9, P = 0.041). Combination therapy including methotrexate (HR = 0.188, 95%CI 0.040-0.871, P = 0.033) and taking β-receptor blockers (HR = 0.249, 95%CI 0.086-0.719, P = 0.010) was associated with reduced risk of all-cause deaths. In conclusion, elevated troponin I, atrial arrhythmia, and systolic and restrictive diastolic dysfunction are typical characteristics of IIM-HF. Combined immunosuppression that includes methotrexate and β-receptor blockers seems to be important to improve survival.

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  • Wei-Siang Chen, Min-Hui Liu, Mei-Ling Cheng, Chao-Hung Wang
    2020 Volume 61 Issue 5 Pages 1014-1021
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 02, 2020
    JOURNALS FREE ACCESS

    Impaired fatty acid metabolism is associated with heart failure (HF) prognosis. However, specific changes in acylcarnitine profiles and their potential clinical value have not been well explored in patients recovering from acute decompensation.

    This study recruited 79 HF patients hospitalized because of acute decompensation with a left ventricular ejection fraction (LVEF) of < 40% and 51 normal controls. Patients were dichotomized into two groups, namely, the "improved (IMP) " and the "non-improved (NIMP) " groups, as defined by the changes in LVEF from baseline to 12 months after discharge. Mass spectrometry was used to quantify the acylcarnitine concentrations at baseline and 6 and 12 months after discharge. The IMP and NIMP groups contained 42 and 37 patients, respectively. At baseline, HF patients had higher plasma concentrations of specific long-, medium-, and short-chain acylcarnitines compared to normal controls. From baseline to 12 months post-discharge, the IMP group showed significant decreases in long- and short-chain acylcarnitine concentrations, but significant increases in medium-chain acylcarnitines. In the NIMP group, none of the acylcarnitines significantly decreased, and significant increases were noted in long-, medium-, and short-chain acylcarnitines. Generalized estimating equations demonstrated that nine acylcarnitines could discriminate the IMP group from the NIMP group, including three long-chain (C18:1, C16, and C16:1) and six short-chain acylcarnitines (C5, C5-OH, C4, C4:1-DC, C3, and C2). After adjusting for age, the six short-chain acylcarnitines remained significant. Changes in short-chain acylcarnitine profiles are independently associated with the improvement in cardiac systolic function after acute decompensation.

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Experimental Studies
  • Zhichao Ma, Jie Qi, Li Gao, Jun Zhang
    2020 Volume 61 Issue 5 Pages 1022-1033
    Published: September 29, 2020
    Released: September 29, 2020
    JOURNALS FREE ACCESS

    Cardiac hypertrophy is one of the significant risk factors that result in maladaptive cardiac remodeling and heart failure, and exercise is known to exert cardioprotection. In this research, the cardioprotective function and exercise mechanisms were explored.

    The rats underwent transverse aortic constriction (TAC) or a sham operation. The rats that received TAC were randomly assigned to five groups: (1) rats subjected to a sham operation as control group (SC), (2) rats that underwent TAC group (TC), (3) TAC and moderate-intensity exercise group (TE), (4) TE plus 3-MA group (TEM), and (5) TE plus Compound C group (TEC). The heart function was measured via echocardiography. Histological analysis and relative protein testing were conducted to analyze collagen deposition and apoptosis. Furthermore, western blot was employed to measure the protein expression of relevant signaling pathways. Impaired cardiac function, interstitial fibrosis, enhanced apoptosis, and ER stress were observed in the TAC-induced left ventricular hypertrophy. Exercise attenuated TAC-induced cardiac dysfunction, interstitial fibrosis, and ER stress-related apoptosis. In addition, exercise significantly improved autophagy and upregulated AMPK phosphorylation. Furthermore, AMPK inhibitor Compound C repressed the activation of AMPK, and autophagy inhibitor 3-methyladenine reversed exercise-induced autophagy. All of these abolished the protection of exercise against cardiac dysfunction and fibrosis induced by TAC.

    Our results indicated that 4 weeks of treadmill exercise could alleviate pressure overload-induced LV dysfunction and remodeling via an autophagy-dependent mechanism, which was induced by enhancing autophagy through the activation of AMPK.

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  • Yongsheng Li, Yanbiao Rao, Hongtao Zhu, Bingyuan Jiang, Maoshu Zhu
    2020 Volume 61 Issue 5 Pages 1034-1040
    Published: September 29, 2020
    Released: September 29, 2020
    JOURNALS FREE ACCESS

    Low-density lipoprotein (LDL) particles are known to be atherogenic agents in coronary artery diseases. They adjust to other electronegative forms and can be the subject for the enhancement of inflammatory events in vessel subendothelial spaces. The LDL uptake is related to the membrane scavenger receptors, including LDL receptor (LDLR). The LDLR expression is closely associated with LDL uptake and occurrence of diseases, such as atherosclerotic cardiovascular diseases. Our findings identified USP16 as a novel regulator of LDLR due to its ability to prevent ubiquitylation-dependent LDLR degradation, further promoting the uptake of LDL. The enhancement of USP16-mediated deubiquitination andthe suppressive degradation of the LDLR cause the presentation of a potential strategy to increase LDL cholesterol clearance.

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Case Reports
  • A Case Report of Radiation-Induced Coronary Artery Disease
    Junqin Bai, Zhichao Dong, Lifei Pan, Xunlong Xia, Bo Zhang
    2020 Volume 61 Issue 5 Pages 1041-1043
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 02, 2020
    JOURNALS FREE ACCESS

    The current treatment of radiation-induced coronary artery disease (RCAD) is comparable to that of generic coronary artery disease (CAD); however, the outcomes of these treatment measures have not been fully examined in RCAD. A 33-year-old woman, without conventional cardiovascular risk factors, presented with left main coronary artery (LMCA) lesions. At the age of 26, she received mediastinal radiation therapy (RT) to treat mixed cellularity Hodgkin lymphoma. One BiodivYsio 3.5 × 18 mm stent was implanted at the LMCA site. At the age of 38, the patient was treated by balloon dilatation because of approximately 50% in-stent stenosis. At the last follow-up in February 2018, when the patient was 51 years old, she no longer complained of chest pain. Coronary angiography showed no de novo or in-stenosis lesions, although optical coherence tomography showed mild neointimal proliferation, calcific plaque, small ruptured intima, and several uncovered struts. The experience of treating this case may shed some light on coronary stenting in coronary lesions caused by RCAD.

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  • Ji-Hun Jang, Sung Woo Kwon, Man-Jong Lee, Kyu-Yong Ko, Jin-Hee Park, G ...
    2020 Volume 61 Issue 5 Pages 1044-1048
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Ivabradine is a selective inhibitor of the sinoatrial node "funny" current, prolonging the slow diastolic depolarization. As it has the ability to block the heart rate selectively, it is more effective at a faster heart rate. It is recommended for the treatment of heart failure reduced ejection fraction in the presence of beta-blocker therapy for the further reduction of the heart rate. However, previous reports have shown the association of Torsade de pointes (TdP) with concurrent use of ivabradine and drugs resulting in QT prolongation or blockage of the metabolic breakdown of ivabradine. In this article, we report two cases of patients with heart failure reduced ejection fraction who developed TdP after ivabradine use. Our report highlights the need to exercise caution with the administration of ivabradine in the presence of a reduced repolarization reserve, such as QT prolongation or metabolic insufficiency.

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  • Tadashi Nakajima, Reika Kawabata-Iwakawa, Yoshiaki Kaneko, Shin-ichiro ...
    2020 Volume 61 Issue 5 Pages 1049-1055
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    While a KCND3 V392I mutation uniquely displays a mixed electrophysiological phenotype of Kv4.3, only limited clinical information on the mutation carriers is available. We report two teenage siblings exhibiting both cardiac (early repolarization syndrome and paroxysmal atrial fibrillation) and cerebral phenotypes (epilepsy and intellectual disability), in whom we identified the KCND3 V392I mutation. We propose a link between the KCND3 mutation with a mixed electrophysiological phenotype and cardiocerebral phenotypes, which may be defined as a novel cardiocerebral channelopathy.

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  • Yu Kang, Hui Wang, Hong Chen, Bo Wang, Yingying Yang, Xuan Zhao, Qihui ...
    2020 Volume 61 Issue 5 Pages 1056-1058
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    An 84-year-old woman with hypertension, Alzheimer's disease, and chronic kidney disease presented with fever and was diagnosed with corona virus disease 2019 (COVID-19). During the hospitalization, she experienced unexpected sinus bradycardia with prolonged QTc, which was thought to be closely related to the short-term use of hydroxychloroquine (HCQ), an old drug used to treat malaria and autoimmune diseases, but now used against COVID-19. The cardiac side effects of HCQ were rare, seen with short-term and low-dose use. With the COVID-19 pandemic, this case alerts clinicians to be aware of the arrhythmogenic effects of HCQ when it is used as an antiviral drug, especially in patients with preexisting cardiovascular diseases.

    Editor’s picks

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  • Technical Aspects and Clinical Outcomes
    Umihiko Kaneko, Daisuke Hachinohe, Ken Kobayashi, Hidemasa Shitan, Kei ...
    2020 Volume 61 Issue 5 Pages 1059-1069
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Because of its rigidity and non-steerability, the presence of a horizontal aortic root poses a major anatomical issue during transcatheter aortic valve replacement (TAVR) with Evolut self-expanding valve. Previous studies have elucidated the difficulties of coaxial implantation of the self-expanding valve in patients with horizontal aorta, often resulting in increased complications and a lower device success rate. To date, most patients with extremely horizontal aorta (aortic root angle ≥ 70°) have been excluded from major TAVR clinical trials. Therefore, available data on TAVR with Evolut in this challenging anatomy are limited, and standardized treatment strategies and clinical results remain unknown. Herein, we report a clinical case series of TAVR with Evolut in extremely horizontal aorta. Among seven patients (aged 80-92 years; STS score, 12.6% ± 7.9%) who underwent TAVR with Evolut system, aortic root angle ranged from 71° to 83° (mean, 75.1°± 4.5°). All patients achieved device success with dedicated strategies and were clinically stable at 3-month follow-up. None of the patients had more than mild paravalvular leakage (PVL) at any point during follow-up.

    Complications in three patients included complete atrioventricular block requiring a permanent pacemaker implantation, cerebral infarction because of atrial fibrillation 3 days after TAVR, and cardiac tamponade requiring pericardiocentesis. In this case series, Evolut self-expanding TAVR in extremely horizontal aorta was effective and feasible with a high device success rate. Based on anatomical features, some dedicated strategies majorly contribute to the success of this procedure. Large-scale multicenter studies are required to confirm our findings.

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  • Hotaka Matsui, Taketo Kawai, Yusuke Sato, Junichi Ishida, Hiroshi Kado ...
    2020 Volume 61 Issue 5 Pages 1070-1074
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    We report a case of lethal myocarditis and myositis after pembrolizumab treatment for advanced upper urinary tract urothelial carcinoma. A 69-year-old man underwent pembrolizumab therapy as a second-line treatment. He had myalgia and a slightly elevated creatinine kinase (CK) on the day of the second administration of pembrolizumab. Five days later, the patient was admitted with severe fatigue and an abnormal gait. Physical examination revealed reduced muscle reflexes and proximal muscle weakness. An electrocardiogram (ECG) demonstrated a wide QRS complex ventricular rhythm. A marked elevation of cardiac enzymes, including CK, myoglobin, and cardiac troponin I, was detected. Myocardial biopsy revealed inflammatory cell infiltration and the partial impairment of myocardial tissue. The electromyogram was normal, but inflammation in myofibers was noted in a muscle biopsy. Myocarditis and myositis as immune-related adverse events (irAEs) were suspected, and the patient began intravenous steroid therapy and plasma exchange. However, the patient underwent cardiac arrest three days after admission and began extracorporeal membrane oxygenation and intra-aortic balloon pumping therapy. Despite steroid pulse therapy, the patient demonstrated no sign of improvement and subsequently died 17 days after admission. Immune-mediated myocarditis is a rare but fatal irAE of an immune checkpoint inhibitor (ICI). The present case suggests that myositis precedes myocarditis. Therefore, if myositis is suspected, subsequent myocarditis may need attention. In conclusion, we found that myositis and myocarditis developed in a patient with advanced urothelial carcinoma after pembrolizumab treatment. A routine follow-up of CK and cardiac troponin I, as well as an ECG, should be performed to identify any possible ICI-induced myocarditis and myositis quickly.

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  • Satoshi Ishii, Takayuki Fujiwara, Jiro Ando, Norifumi Takeda, Toshiro ...
    2020 Volume 61 Issue 5 Pages 1075-1078
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Pulmonary valve stenosis (PVS) accounts for approximately 10% of all congenital heart defects. Echocardiography and right heart catheterization are the gold standards for diagnosis of PVS and for assessing disease severity and responsiveness to treatment.

    Recently, cardiac magnetic resonance imaging (cMRI) has been established as an important tool to comprehensively evaluate cardiac structure and function; however, research into the usefulness of cMRI for PVS management is limited. Here, we describe a case of a 59-year-old female with isolated, severe PVS who was successfully treated with balloon pulmonary valvuloplasty (BPV) followed by sequential cMRI at 1 and 12 months. Exertional dyspnea and elevated plasma BNP concentration were observed 1 month after BPV; however, echocardiographic findings did not indicate recurrent stenosis or increased pulmonary valve regurgitation but an increase in mitral E/e'. cMRI demonstrated improved systolic forward flow and RV function with enlargement of LV volume, and the rapid increase in LV preload might be associated with the transient deterioration in symptoms and BNP level, which both gradually improved within 3 months after BPV. cMRI further depicted that a reduced RV mass index and increased RV cardiac output were achieved gradually during the follow-up period.

    In conclusion, cMRI in combination with echocardiography was sufficiently informative to follow-up this PVS patient both before and after BPV. cMRI is easily reproducible in adult patients; therefore, cMRI should be recommended for long-term follow-up in adult PVS patients.

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  • Aya Miura, Hidehito Kondo, Takuma Yamamoto, Yasuko Okumura, Hajime Nis ...
    2020 Volume 61 Issue 5 Pages 1079-1083
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 02, 2020
    JOURNALS FREE ACCESS
    Supplementary material

    A Japanese girl with polycystic kidney disease (PKD) developed normally, but at 8 months of age, she was hospitalized for acute onset dyspnea. On the day after admission to hospital, her general condition suddenly became worse. An echocardiogram showed left ventricular dilatation with thin walls, severe mitral valve regurgitation, and a reduced ejection fraction. She died of acute cardiac failure 3 hours after the sudden change. Postmortem analysis with light microscopy showed disarray of cardiomyocytes without obvious infiltration of lymphocytes, and we diagnosed her heart failure as idiopathic dilated cardiomyopathy (DCM). Clinical exome sequencing showed compound heterozygous variants in JPH2 (p.T237A/p.I414L) and a heterozygous nonsense mutation in PKD1 (p.Q4193*). To date, several variants in the JPH2 gene have been reported to be pathogenic for adult-onset hypertrophic cardiomyopathy or DCM in an autosomal dominant manner and infantile-onset DCM in an autosomal recessive manner. Additionally, autosomal dominant polycystic kidney disease is a systemic disease associated with several extrarenal manifestations, such as cardiomyopathy. Here we report a sudden infant death case of DCM and discuss the genetic variants of DCM and PKD.

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  • Yuji Ohnishi, Seigo Okada, Hiroki Yasudo, Sasagu Kimura, Yasuo Suzuki, ...
    2020 Volume 61 Issue 5 Pages 1084-1087
    Published: September 29, 2020
    Released: September 29, 2020
    [Advance publication] Released: September 12, 2020
    JOURNALS FREE ACCESS

    Diazoxide, a drug used to treat hyperinsulinemic hypoglycemia (HH), is associated with pulmonary hypertension (PH), as reported by the US Food and Drug Administration. However, no report has detailed the association between diazoxide dose and PH development. We report a case of an infant with HH, subsequently complicated by diazoxide-induced PH. When diazoxide was introduced, PH did not appear initially, but it developed during increased dosing. We monitored PH via regular echocardiography examinations. PH gradually improved with tapering of the diazoxide dose and disappeared after drug discontinuation. Our case suggests a diazoxide dose threshold might induce PH. Therefore, close echocardiography examinations should accompany diazoxide treatment.

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