China Multi-Center Cohort Study on Risk Evaluation of Arrhythmogenic Cardiomyopathy: The ChinaCORE ACM Registry
Yuxiao Hu1, Zhongli Chen2, Anteng Shi3
1State Key Laboratory of Cardiovascular Disease, National Clinical Research Center for Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Insights
This study establishes the ChinaCORE ACM registry, a national cohort of Chinese patients with arrhythmogenic cardiomyopathy (ACM). The registry aims to improve understanding and risk prediction for ACM in this unique population.
Area of Science:
- Cardiology
- Genetics
- Public Health
Background:
- Arrhythmogenic cardiomyopathy (ACM) in China presents distinct genetic and clinical features.
- A significant gap exists in prognostic models tailored for Chinese ACM patients.
Purpose of the Study:
- To create a large, national ACM patient cohort with high-quality, standardized data.
- To facilitate future risk prediction models for Chinese ACM patients.
Main Methods:
- Enrollment of patients with definite/borderline ACM and genotype-positive relatives.
- Comprehensive baseline data collection: medical history, ECG, imaging, genetics, labs.
- Longitudinal outcome tracking for heart failure and ventricular arrhythmias.
Main Results:
- The ChinaCORE ACM registry includes 622 participants (552 probands).
- Preliminary analysis of 577 patients shows 495 diagnosed with arrhythmogenic right ventricular cardiomyopathy.
- High prevalence of malignant ventricular arrhythmias (40.1%) and end-stage heart failure (21.9%) observed.
Conclusions:
- The ChinaCORE ACM registry is a national, longitudinal, observational cohort study.
- This initiative enhances understanding of the ACM disease spectrum in Chinese populations.
- The registry will be instrumental in improving prognostic accuracy for ACM.
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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