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Published on: December 22, 2023
[Early repolarization syndrome and recurrent syncope in two Chinese pedigrees]
Zhen-ni TAN1, Wei DENG, Yuan-yuan LUO
1Department of Cardiology, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China.
Early repolarization syndrome (ERS) can lead to serious cardiac events like syncope and ventricular arrhythmias, not always a benign condition. Family screening is crucial for identifying at-risk individuals.
Area of Science:
- Cardiology
- Clinical Electrophysiology
- Genetics
Background:
- Early repolarization syndrome (ERS) is an electrocardiographic finding often considered benign.
- However, its association with cardiac events, particularly in familial contexts, requires further investigation.
Purpose of the Study:
- To investigate the clinical characteristics of Early Repolarization Syndrome (ERS) in two families.
- To understand the link between ERS, syncope, and potentially life-threatening arrhythmias within these families.
Main Methods:
- Comprehensive clinical screening of all family members, including probands.
- Utilized electrocardiography (ECG), echocardiography, Holter monitoring, chest X-ray, head-up tilt test, and blood biochemistry.
Main Results:
- No organic heart disease was found in either family.
- Proband 1 experienced recurrent syncope, ERS, and documented torsade de pointes (TdP) ventricular tachycardia and ventricular fibrillation.
- Proband 2 presented with recurrent syncope and ERS; his father had sudden cardiac death, and a nephew had asymptomatic ERS.
Conclusions:
- Early Repolarization Syndrome (ERS) is not invariably benign and can be associated with severe outcomes.
- Recurrent syncope, TdP, and ventricular fibrillation are potential complications of ERS.
- Pedigree research is essential for understanding the clinical implications and inheritance patterns of ERS.
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