Treatment Registry of Arrhythmias, Complications and Electrocardiograms in Arrhythmogenic CardioMyopathies

Treatment Registry of Arrhythmias, Complications and Electrocardiograms in Arrhythmogenic CardioMyopathies: A Multicenter Retrospective Observational Study

TRACE-ACM is a multicenter, retrospective, observational study of patients with arrhythmogenic cardiomyopathy who received an implantable cardioverter-defibrillator (ICD) and had documented ventricular tachyarrhythmias. The study aims to describe the prevalence and type of ICD-related complications, characterize ventricular arrhythmias documented by ICD electrograms and/or ECG recordings, and explore associations between clinical, device-related, and treatment-related factors and arrhythmic outcomes.

Patients with arrhythmogenic cardiomyopathy will be identified at participating centers with expertise in the diagnosis and management of arrhythmogenic cardiomyopathies. De-identified retrospective data will be collected, including demographics, arrhythmogenic cardiomyopathy phenotype, genetic data, ICD type and indication, clinical follow-up, ICD therapies, antiarrhythmic and heart failure therapies, catheter ablation, and ECG/ICD electrogram documentation of ventricular tachyarrhythmias.

ICD-related complications will include implant-related complications such as hematoma, perforation, pneumothorax, upper-limb deep vein thrombosis, lead failure, and infection, as well as non-implant-related complications such as inappropriate shocks. Ventricular arrhythmias will be classified as monomorphic ventricular tachycardia, polymorphic ventricular tachycardia/ventricular fibrillation, or transition patterns between these arrhythmia types. When available, arrhythmia initiation will be analyzed using pre-specified ECG/EGM criteria including the origin of the beats preceding arrhythmia onset, R1-R2 and R2-R3 intervals, pause dependency, coupling interval, and prematurity index.

The study will also describe atrial arrhythmias and medical, interventional, and device-based therapies adopted in this population, and will explore their relationship with ventricular arrhythmia recurrences and ICD interventions.

Inclusion Criteria:

• Diagnosis of arrhythmogenic cardiomyopathy, including right-dominant arrhythmogenic right ventricular cardiomyopathy, biventricular arrhythmogenic cardiomyopathy, or left-dominant arrhythmogenic left ventricular cardiomyopathy.
• ICD implantation.
• Documented sustained ventricular tachyarrhythmia, including polymorphic ventricular tachycardia, ventricular fibrillation, or monomorphic ventricular tachycardia.
• Periodic clinical and ICD follow-up.
• Arrhythmia onset available from ICD electrograms and/or ECG recordings.
• ECG/EGM tracings available for analysis by the steering ECG committee.

Exclusion Criteria:

• Incomplete ICD data or incomplete ICD follow-up.
• Significant coronary artery disease, defined as coronary plaque greater than 50% at coronary angiography or coronary computed tomography angiography.
• Primary valvular heart disease or congenital heart disease.
• Infiltrative or inflammatory cardiomyopathies, including sarcoidosis or amyloidosis.
• Previous exposure to therapies associated with cardiac toxicity, including chemotherapy.