{"title":"China Multi-Center Cohort Study on Risk Evaluation of Arrhythmogenic Cardiomyopathy (ChinaCORE ACM) Registry.","authors":"Yuxiao Hu, Zhongli Chen, Anteng Shi, Zemeng Li, Zixian Chen, Yingying Zheng, Xi Zhao, Shimo Dai, Yubi Lin, Yifei Li, Bing Yang, Xiaoyan Zhao, Guoliang Li, Xianliang Zhou, Shengshou Hu, Lingmin Wu, Liang Chen","doi":"10.1016/j.jacasi.2025.04.005","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Arrhythmogenic cardiomyopathy (ACM) patients in China exhibit unique genetic and clinical characteristics. There is a lack of prognostic models specific to Chinese ACM patients.</p><p><strong>Objectives: </strong>This study aims to establish a large, national ACM patient cohort with uniformly collected, high-quality data for future risk prediction.</p><p><strong>Methods: </strong>This study includes patients with definite or borderline ACM diagnoses, along with their genotype-positive relatives. At baseline, comprehensive data collection includes medical history, electrocardiograms, imaging data, genetic testing, and laboratory evaluations. Outcome data include heart failure events and malignant ventricular arrhythmias.</p><p><strong>Results: </strong>As of September 2024, the registry has enrolled 622 participants, including 552 probands (88.7%) and 70 family members (11.3%) carrying ACM-related variants. Preliminary cohort includes 577 patients (92.8%), of whom 495 were diagnosed with definite arrhythmogenic right ventricular cardiomyopathy. The median age of symptom onset was 33.0 years (Q1-Q3: 22.0-45.0 years), with 41.6% experiencing arrhythmia-related symptoms. Abnormal electrocardiogram findings included T-wave inversion (72.7%) and epsilon waves (24.8%) in leads V<sub>1</sub> to V<sub>3</sub>. Imaging evaluation revealed RV dilatation in 44.6% and left ventricular dilatation in 29.8%, with a mean left ventricular ejection fraction of 53.0 ± 14.5%. Regarding outcomes, malignant ventricular arrhythmias occurred in 255 (40.1%) individuals, while 21.9% developed end-stage heart failure, including 35 individuals who died of heart failure and 101 patients who underwent heart transplantation.</p><p><strong>Conclusions: </strong>The ChinaCORE ACM (China Multi-Center Cohort Study on Risk Evaluation of Arrhythmogenic Cardiomyopathy) registry is a national, longitudinal, observational cohort study. This study contributes to expanding the understanding of the disease spectrum of Chinese ACM patients and improving prognostic predictions.</p>","PeriodicalId":73529,"journal":{"name":"JACC. Asia","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2025-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"JACC. Asia","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1016/j.jacasi.2025.04.005","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Arrhythmogenic cardiomyopathy (ACM) patients in China exhibit unique genetic and clinical characteristics. There is a lack of prognostic models specific to Chinese ACM patients.
Objectives: This study aims to establish a large, national ACM patient cohort with uniformly collected, high-quality data for future risk prediction.
Methods: This study includes patients with definite or borderline ACM diagnoses, along with their genotype-positive relatives. At baseline, comprehensive data collection includes medical history, electrocardiograms, imaging data, genetic testing, and laboratory evaluations. Outcome data include heart failure events and malignant ventricular arrhythmias.
Results: As of September 2024, the registry has enrolled 622 participants, including 552 probands (88.7%) and 70 family members (11.3%) carrying ACM-related variants. Preliminary cohort includes 577 patients (92.8%), of whom 495 were diagnosed with definite arrhythmogenic right ventricular cardiomyopathy. The median age of symptom onset was 33.0 years (Q1-Q3: 22.0-45.0 years), with 41.6% experiencing arrhythmia-related symptoms. Abnormal electrocardiogram findings included T-wave inversion (72.7%) and epsilon waves (24.8%) in leads V1 to V3. Imaging evaluation revealed RV dilatation in 44.6% and left ventricular dilatation in 29.8%, with a mean left ventricular ejection fraction of 53.0 ± 14.5%. Regarding outcomes, malignant ventricular arrhythmias occurred in 255 (40.1%) individuals, while 21.9% developed end-stage heart failure, including 35 individuals who died of heart failure and 101 patients who underwent heart transplantation.
Conclusions: The ChinaCORE ACM (China Multi-Center Cohort Study on Risk Evaluation of Arrhythmogenic Cardiomyopathy) registry is a national, longitudinal, observational cohort study. This study contributes to expanding the understanding of the disease spectrum of Chinese ACM patients and improving prognostic predictions.
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