Korean Circulation Journal最新文献

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Cost-effectiveness of Fractional Flow Reserve Versus Intravascular Ultrasound to Guide Percutaneous Coronary Intervention: Results From the FLAVOUR Study. 分数血流储备与血管内超声引导经皮冠状动脉介入治疗的成本效益:FLAVOUR 研究结果
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2025-01-01 Epub Date: 2024-10-11 DOI: 10.4070/kcj.2024.0156
Doyeon Hwang, Hea-Lim Kim, Jane Ko, HyunJin Choi, Hanna Jeong, Sun-Ae Jang, Xinyang Hu, Jeehoon Kang, Jinlong Zhang, Jun Jiang, Joo-Yong Hahn, Chang-Wook Nam, Joon-Hyung Doh, Bong-Ki Lee, Weon Kim, Jinyu Huang, Fan Jiang, Hao Zhou, Peng Chen, Lijiang Tang, Wenbing Jiang, Xiaomin Chen, Wenming He, Sung Gyun Ahn, Ung Kim, You-Jeong Ki, Eun-Seok Shin, Hyo-Soo Kim, Seung-Jea Tahk, JianAn Wang, Tae-Jin Lee, Bon-Kwon Koo
{"title":"Cost-effectiveness of Fractional Flow Reserve Versus Intravascular Ultrasound to Guide Percutaneous Coronary Intervention: Results From the FLAVOUR Study.","authors":"Doyeon Hwang, Hea-Lim Kim, Jane Ko, HyunJin Choi, Hanna Jeong, Sun-Ae Jang, Xinyang Hu, Jeehoon Kang, Jinlong Zhang, Jun Jiang, Joo-Yong Hahn, Chang-Wook Nam, Joon-Hyung Doh, Bong-Ki Lee, Weon Kim, Jinyu Huang, Fan Jiang, Hao Zhou, Peng Chen, Lijiang Tang, Wenbing Jiang, Xiaomin Chen, Wenming He, Sung Gyun Ahn, Ung Kim, You-Jeong Ki, Eun-Seok Shin, Hyo-Soo Kim, Seung-Jea Tahk, JianAn Wang, Tae-Jin Lee, Bon-Kwon Koo","doi":"10.4070/kcj.2024.0156","DOIUrl":"10.4070/kcj.2024.0156","url":null,"abstract":"<p><strong>Background and objectives: </strong>The Fractional Flow Reserve and Intravascular Ultrasound-Guided Intervention Strategy for Clinical Outcomes in Patients with Intermediate Stenosis (FLAVOUR) trial demonstrated non-inferiority of fractional flow reserve (FFR)-guided percutaneous coronary intervention (PCI) compared with intravascular ultrasound (IVUS)-guided PCI. We sought to investigate the cost-effectiveness of FFR-guided PCI compared to IVUS-guided PCI in Korea.</p><p><strong>Methods: </strong>A 2-part cost-effectiveness model, composed of a short-term decision tree model and a long-term Markov model, was developed for patients who underwent PCI to treat intermediate stenosis (40% to 70% stenosis by visual estimation on coronary angiography). The lifetime healthcare costs and quality-adjusted life-years (QALYs) were estimated from the healthcare system perspective. Transition probabilities were mainly referred from the FLAVOUR trial, and healthcare costs were mainly obtained through analysis of Korean National Health Insurance claims data. Health utilities were mainly obtained from the Seattle Angina Questionnaire responses of FLAVOUR trial participants mapped to EQ-5D.</p><p><strong>Results: </strong>From the Korean healthcare system perspective, the base-case analysis showed that FFR-guided PCI was 2,451 U.S. dollar lower in lifetime healthcare costs and 0.178 higher in QALYs compared to IVUS-guided PCI. FFR-guided PCI remained more likely to be cost-effective over a wide range of willingness-to-pay thresholds in the probabilistic sensitivity analysis.</p><p><strong>Conclusions: </strong>Based on the results from the FLAVOUR trial, FFR-guided PCI is projected to decrease lifetime healthcare costs and increase QALYs compared with IVUS-guided PCI in intermediate coronary lesion, and it is a dominant strategy in Korea.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov Identifier: NCT02673424.</p>","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":" ","pages":"34-46"},"PeriodicalIF":3.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11735157/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142729791","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Gender Is Not Predictor for Clinical Outcomes in Patients Undergoing Percutaneous Coronary Intervention for Coronary Bifurcation. 性别不是经皮冠状动脉介入治疗冠状动脉分叉患者临床结果的预测因子。
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2025-01-01 Epub Date: 2024-11-28 DOI: 10.4070/kcj.2024.0333
Cheol Ung Choi
{"title":"Gender Is Not Predictor for Clinical Outcomes in Patients Undergoing Percutaneous Coronary Intervention for Coronary Bifurcation.","authors":"Cheol Ung Choi","doi":"10.4070/kcj.2024.0333","DOIUrl":"10.4070/kcj.2024.0333","url":null,"abstract":"","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":" ","pages":"17-19"},"PeriodicalIF":3.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11735156/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142903048","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cost-Effectiveness Matters! FFR Versus IVUS-Guided PCI in Modern Clinical Practice: Insights From the FLAVOUR Trial. 成本效益至关重要!现代临床实践中的 FFR 与 IVUS 引导的 PCI:FLAVOUR 试验的启示。
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2025-01-01 Epub Date: 2024-10-28 DOI: 10.4070/kcj.2024.0327
Sun Oh Kim, Jong-Il Park, Ung Kim
{"title":"Cost-Effectiveness Matters! FFR Versus IVUS-Guided PCI in Modern Clinical Practice: Insights From the FLAVOUR Trial.","authors":"Sun Oh Kim, Jong-Il Park, Ung Kim","doi":"10.4070/kcj.2024.0327","DOIUrl":"10.4070/kcj.2024.0327","url":null,"abstract":"","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":" ","pages":"47-49"},"PeriodicalIF":3.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11735150/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142729788","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
UPF1 Alleviates Myocardial Ischemia-Reperfusion Injury by Regulating SMURF2-Mediated Ubiquitination Degradation of FOXA2.
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2024-12-10 DOI: 10.4070/kcj.2024.0190
Aixin Li, Peng Li, Chunling Mu, Dong Li, Keyan Chen, Zhaoguang Liang
{"title":"<i>UPF1</i> Alleviates Myocardial Ischemia-Reperfusion Injury by Regulating <i>SMURF2</i>-Mediated Ubiquitination Degradation of <i>FOXA2</i>.","authors":"Aixin Li, Peng Li, Chunling Mu, Dong Li, Keyan Chen, Zhaoguang Liang","doi":"10.4070/kcj.2024.0190","DOIUrl":"https://doi.org/10.4070/kcj.2024.0190","url":null,"abstract":"<p><strong>Background and objectives: </strong>Myocardial ischemia/reperfusion injury (MIRI) is an important factor affecting therapeutic effect and prognosis of acute myocardial infarction. Here, the effects of up-frameshift 1 (UPF1) on cardiomyocyte apoptosis in MIRI were evaluated.</p><p><strong>Methods: </strong>H9C2 cells were cultured under hypoxia/reoxygenation (H/R) condition. The expression of UPF1, SMAD-specific E3 ubiquitin ligase 2 (SMURF2), forkhead box A2 (FOXA2), protease-activated receptor 4 (PAR4), Bax, and Cleaved caspase-3 was assessed utilizing reverse transcription quantitative polymerase chain reaction and western blot. Cell viability and apoptosis were measured by Cell Counting Kit-8 and flow cytometry. Infarct area was examined by tetrazolium chloride staining in myocardial ischemia/reperfusion (I/R) rat model. HE and immunohistochemistry staining evaluated myocardial injury and UPF1 expression, respectively. Terminal deoxynucleotidyl transferase mediated dUTP nick end-labeling staining tested apoptosis. RNA immunoprecipitation, chromatin immunoprecipitation and dual luciferase assay verified molecular interactions. FOXA2 ubiquitination was detected by immunoprecipitation assay. SMURF2 mRNA stability was tested by actinomycin D treatment.</p><p><strong>Results: </strong>FOXA2 effectively suppressed cardiomyocyte apoptosis induced by H/R by inhibiting PAR4 at transcriptional level. Degradation of FOXA2 was facilitated through SMURF2-mediated ubiquitination. Increased expression of UPF1 resulted in a reduction of H/R-induced cardiomyocyte apoptosis, and improved myocardial dysfunction caused by I/R in vivo. UPF1 influenced the decay of SMURF2 mRNA, leading to a decrease in its expression. Through SMURF2/FOXA2/PAR4 axis, UPF1 effectively suppressed cardiomyocyte apoptosis triggered by H/R.</p><p><strong>Conclusions: </strong>By suppressing SMURF2 mRNA stability, UPF1 upregulated FOXA2 expression to inhibit PAR4, leading to inhibition of apoptosis during MIRI, which provides new therapeutic targets for MIRI treatment.</p>","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143441211","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical Characteristics and Outcomes of Acute Myocarditis: An Analysis of Korean Multicenter Registry. 急性心肌炎的临床特征和预后:韩国多中心登记分析。
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2024-12-10 DOI: 10.4070/kcj.2024.0229
Junho Hyun, Dayoung Pack, Minjung Bak, Hyukjin Park, Hyung Yoon Kim, Seonhwa Lee, In-Cheol Kim, So Ree Kim, Mi-Na Kim, Kyung-Hee Kim, Sang Eun Lee, Jeong Hoon Yang
{"title":"Clinical Characteristics and Outcomes of Acute Myocarditis: An Analysis of Korean Multicenter Registry.","authors":"Junho Hyun, Dayoung Pack, Minjung Bak, Hyukjin Park, Hyung Yoon Kim, Seonhwa Lee, In-Cheol Kim, So Ree Kim, Mi-Na Kim, Kyung-Hee Kim, Sang Eun Lee, Jeong Hoon Yang","doi":"10.4070/kcj.2024.0229","DOIUrl":"https://doi.org/10.4070/kcj.2024.0229","url":null,"abstract":"<p><strong>Background and objectives: </strong>Data are limited on the clinical manifestations and outcomes of acute myocarditis from a large-scale registry. We investigated acute myocarditis's clinical characteristics and prognosis from a large-scale, multi-center registry in the Republic of Korea.</p><p><strong>Methods: </strong>We collected data from seven hospitals between 2001 and 2021. Clinical variables and outcomes during the index hospitalization and follow-up periods were analyzed. We also evaluated inter-center and temporal differences in diagnostic and treatment patterns.</p><p><strong>Results: </strong>Eight hundred forty-one patients diagnosed with acute myocarditis were included. Common symptoms included chest pain (60.4%), followed by fever or myalgia (46.3%), and dyspnea (45.7%). Fulminant myocarditis occurred in 421 (50.1%), with 217 requiring extracorporeal membrane oxygenation (ECMO) support. Endomyocardial biopsy (EMB) was performed in 276 (32.8%) patients, and biopsy-proven diagnosis was made in 234 (27.8%). Based on the EMB results, lymphocytic myocarditis was the predominant form (69.6%), followed by eosinophilic (13.8%) and giant cell myocarditis (1.4%). Eighty-three in-hospital (9.9%) and 16 (1.9%) additional mortality during the follow-up occurred. An increase in the use of EMB, cardiac imaging, and immunosuppressive therapy was noted over time, but in-hospital mortality remained unchanged. Remarkable variations in diagnosis and treatment were observed across different centers.</p><p><strong>Conclusions: </strong>This study unveiled clinical features of acute myocarditis in the Republic of Korea, including a high incidence of fulminant myocarditis and complex cases requiring ECMO. Given the considerable inter-center variation in diagnostic and treatment patterns and prognosis, protocolized future trials are needed to clarify diagnosis and treatment in patients with acute myocarditis.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov Identifier: NCT05933902.</p>","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142979076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Exercise Preferences and Barriers Among Patients With Heart Failure With Reduced Ejection Fraction or Myocardial Infarction. 射血分数降低型心力衰竭或心肌梗死患者的运动偏好和障碍。
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2024-12-01 Epub Date: 2024-07-30 DOI: 10.4070/kcj.2024.0097
Mi Kyung Lee, Chan Joo Lee, Seon Young Goo, Jin Young Moon, Tae Ho Lee, Seok-Min Kang, Ick-Mo Chung, Justin Y Jeon
{"title":"Exercise Preferences and Barriers Among Patients With Heart Failure With Reduced Ejection Fraction or Myocardial Infarction.","authors":"Mi Kyung Lee, Chan Joo Lee, Seon Young Goo, Jin Young Moon, Tae Ho Lee, Seok-Min Kang, Ick-Mo Chung, Justin Y Jeon","doi":"10.4070/kcj.2024.0097","DOIUrl":"10.4070/kcj.2024.0097","url":null,"abstract":"<p><strong>Background and objectives: </strong>Although cardiac rehabilitation (CR) is highly recommended in patients with cardiovascular disease (CVD), participation in CR is low mainly due to access barriers. Home-based CR (HBCR) has been recommended to overcome access barriers. Exercise is a core component of CR and should be developed and implemented based on individual characteristics. We aimed to assess physical activity behaviors, exercise preferences, and exercise barriers to understand physical activity characteristics of CVD patients.</p><p><strong>Methods: </strong>Participants were patients between the ages 19 to 75 years with a history of heart failure with reduced ejection fraction (HFrEF) or myocardial infarction (MI). They completed a cross-sectional survey at a tertiary hospital's outpatient clinic from April to June 2021. Survey data included physical activity levels, patterns, preference, and barriers of exercise.</p><p><strong>Results: </strong>Participants (n=189; 143 males, 46 females, 62.1±12.0 years) were diagnosed as either HFrEF (n=160, 84.7%) or a history of MI (n=97, 51.3%). Only 26.5% of patients engaged in moderate to vigorous exercise for more than 150 minutes per week. Participants preferred exercising alone or with families. Walking (65.6%) and resistance exercises (35.4%) were favored, with outdoor (37%) and home-based (30.2%) settings preferred over fitness centers (10.6%) and hospitals (0.5%). Barriers to exercise included fatigue (34.4%), poor health perception (31.7%), and low fitness levels (30.7%).</p><p><strong>Conclusions: </strong>The results of this study can be used to develop tailored HBCR programs that consider individual preferences and address specific barriers, facilitating adequate physical activity engagement.</p>","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":" ","pages":"825-835"},"PeriodicalIF":3.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11685343/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142036227","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Navigating the Efficacy and Safety of Oral Anticoagulation Use for Stroke Prevention in Very Elderly Patients With Atrial Fibrillation. 老年心房颤动患者使用口服抗凝药预防脑卒中的有效性和安全性导航。
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2024-12-01 Epub Date: 2024-10-17 DOI: 10.4070/kcj.2024.0309
Seung Yong Shin, Woo Hyuk Song, Gregory Y H Lip
{"title":"Navigating the Efficacy and Safety of Oral Anticoagulation Use for Stroke Prevention in Very Elderly Patients With Atrial Fibrillation.","authors":"Seung Yong Shin, Woo Hyuk Song, Gregory Y H Lip","doi":"10.4070/kcj.2024.0309","DOIUrl":"10.4070/kcj.2024.0309","url":null,"abstract":"","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":" ","pages":"822-824"},"PeriodicalIF":3.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11685349/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142590815","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Catecholaminergic Polymorphic Ventricular Tachycardia in Children: Insights and Challenges From the Current Study. 儿茶酚胺能多形性室性心动过速儿童:当前研究的见解和挑战。
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2024-12-01 DOI: 10.4070/kcj.2024.0417
Min-Jung Cho
{"title":"Catecholaminergic Polymorphic Ventricular Tachycardia in Children: Insights and Challenges From the Current Study.","authors":"Min-Jung Cho","doi":"10.4070/kcj.2024.0417","DOIUrl":"10.4070/kcj.2024.0417","url":null,"abstract":"","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":"54 12","pages":"865-867"},"PeriodicalIF":3.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11685348/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142903249","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
New Suggestion for Congenital Heart Disease Prevalence Studies. 先天性心脏病患病率研究的新建议
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2024-12-01 DOI: 10.4070/kcj.2024.0329
Sin Weon Yun
{"title":"New Suggestion for Congenital Heart Disease Prevalence Studies.","authors":"Sin Weon Yun","doi":"10.4070/kcj.2024.0329","DOIUrl":"10.4070/kcj.2024.0329","url":null,"abstract":"","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":"54 12","pages":"851-852"},"PeriodicalIF":3.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11685347/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142903321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Treatment Outcomes in Children With Catecholaminergic Polymorphic Ventricular Tachycardia: A Single Institutional Experience. 儿茶酚胺能多形性室性心动过速儿童的治疗结果:单一机构经验。
IF 3 3区 医学
Korean Circulation Journal Pub Date : 2024-12-01 DOI: 10.4070/kcj.2024.0183
Joowon Lee, Bo Sang Kwon, Mi Kyoung Song, Sang-Yun Lee, Jung Min Ko, Gi Beom Kim, Eun Jung Bae
{"title":"Treatment Outcomes in Children With Catecholaminergic Polymorphic Ventricular Tachycardia: A Single Institutional Experience.","authors":"Joowon Lee, Bo Sang Kwon, Mi Kyoung Song, Sang-Yun Lee, Jung Min Ko, Gi Beom Kim, Eun Jung Bae","doi":"10.4070/kcj.2024.0183","DOIUrl":"10.4070/kcj.2024.0183","url":null,"abstract":"<p><strong>Background and objectives: </strong>Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening inherited arrhythmogenic disorder. Recently, <i>RYR2</i>, the major CPVT-causative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT.</p><p><strong>Methods: </strong>We retrospectively reviewed 23 patients diagnosed with CPVT before 19 years of age. Genetic analysis, history of neuropsychiatric manifestations, changes in ventricular arrhythmia burden before and after treatment, occurrence of cardiac events, and overall survival (OS) were investigated.</p><p><strong>Results: </strong><i>RYR2</i> variants were identified in 17 patients, and 14 were classified as pathogenic or likely pathogenic. Neuropsychiatric manifestations, including intellectual disability and attention deficit hyperactivity disorder, were identified in 10 patients (43.5%). The 5-year cardiac event-free survival rate was 31.2%, and the 10-year OS rate was 73.1%. Patients diagnosed since 2009 had a higher cardiac event-free survival rate than those diagnosed before 2009 (p=0.0028). Combined beta-blocker and flecainide therapy demonstrated a lower risk of cardiac events than beta-blocker monotherapy (hazard ratio [HR], 0.08; 95% confidence interval [CI], 0.02-0.38; p=0.002). Left cardiac sympathetic denervation (LCSD) reduced the ventricular arrhythmia burden in Holter monitoring. Occurrence of near-fatal cardiac events after diagnosis was an independent predictor of death (HR, 33.40; 95% CI, 6.23-179.95; p<0.001).</p><p><strong>Conclusions: </strong>Neuropsychiatric manifestations are common in children with CPVT. Flecainide and/or LCSD, when added to beta-blocker therapy, reduce the ventricular arrhythmia burden and cardiac events, thereby improving treatment outcomes in recent years.</p>","PeriodicalId":17850,"journal":{"name":"Korean Circulation Journal","volume":"54 12","pages":"853-864"},"PeriodicalIF":3.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11685342/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142903322","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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