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ABSTRACT: Aims
Familial ST-segment Depression Syndrome (Fam-STD) is a novel inherited cardiac disease associated with arrhythmias and sudden cardiac death. This study aimed at investigating the cardiac activation pathway in patients with Fam-STD, modelling the electrocardiogram (ECG) phenotype, and performing in-depth ST-segment analyses.Methods and results
CineECG analysis of patients with Fam-STD and age- and sex-matched controls. The groups were compared using the CineECG software which included the trans-cardiac ratio and the electrical activation pathway. We simulated the Fam-STD ECG phenotype by adjusting action potential duration (APD) and action potential amplitude (APA) in specific cardiac regions. High-resolution ST-segment analyses were performed per lead by dividing the ST-segment into nine 10 ms subintervals. Twenty-seven Fam-STD patients (74% females, mean age 51.6 ± 6.2 years) and 83 matched controls were included. Among Fam-STD patients, electrical activation pathway analysis in the anterior-basal orientation showed significantly abnormal direction toward the basal areas of the heart starting from QRS 60-89 ms until Tpeak-Tend (all P < 0.001). Simulations with shortened APD and reduced APA in the left ventricle basal regions recapitulated the Fam-STD ECG phenotype. Detailed ST-segment analyses showed significant differences in all nine 10 ms subintervals (all P < 0.01), with the most prominent findings during the 70-79/80-89 ms intervals.Conclusion
CineECG analyses indicated abnormal repolarization with basal directions, and the Fam-STD ECG phenotype was simulated by reducing APD and APA in the left ventricle basal regions. Detailed ST-analysis showed amplitudes consistent with the proposed diagnostic criteria for Fam-STD patients. Our findings provide new insight into the electrophysiological abnormalities of Fam-STD.
SUBMITTER: Frosted R
PROVIDER: S-EPMC10228618 | biostudies-literature | 2023 May
REPOSITORIES: biostudies-literature
Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology 20230501 5
<h4>Aims</h4>Familial ST-segment Depression Syndrome (Fam-STD) is a novel inherited cardiac disease associated with arrhythmias and sudden cardiac death. This study aimed at investigating the cardiac activation pathway in patients with Fam-STD, modelling the electrocardiogram (ECG) phenotype, and performing in-depth ST-segment analyses.<h4>Methods and results</h4>CineECG analysis of patients with Fam-STD and age- and sex-matched controls. The groups were compared using the CineECG software which ...[more]