Catheter ablation of ventricular arrhythmias in left ventricular noncompaction cardiomyopathy
Juan José Sánchez Muñoz1, Carmen Muñoz-Esparza1, Pablo Peñafiel Verdú1
1Arrhythmia Unit and Electrophysiology, Department of Cardiology, Virgen de la Arrixaca University Hospital, Murcia, Spain; Inherited Cardiac Disease Unit, Department of Cardiology, Virgen de la Arrixaca University Hospital, European Reference Networks (Guard-Heart), European Commission, Brusels, Belgium; Instituto Murciano de Investigación Biosanitaria (IMIB), Murcia, Spain; Red de Investigación Cardiovascular (CIBERCV) Instituto de Salud Carlos III, Spain.
Insights
Ventricular arrhythmias (VAs) in left ventricular noncompaction (LVNC) cardiomyopathy have a varied origin, including outflow tracts and Purkinje fibers. Ablation strategies are tailored to these diverse substrates, offering new insights into managing VAs in LVNC.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Limited data exist on ventricular arrhythmias (VAs) in left ventricular noncompaction (LVNC) cardiomyopathy.
- LVNC is a rare congenital heart defect characterized by abnormal myocardial development.
Purpose of the Study:
- To analyze the clinical and electrocardiographic characteristics of VAs in patients with LVNC.
- To understand the arrhythmogenic substrate in LVNC.
Main Methods:
- Retrospective analysis of 42 patients with LVNC and VAs.
- Evaluation of clinical presentation, electrocardiography, and electrophysiological study findings.
- Endocardial and epicardial mapping and ablation were performed in selected patients.
Main Results:
- VAs in LVNC were associated with isolated LVNC, dilated cardiomyopathy, or hypertrophic cardiomyopathy.
- Premature ventricular complexes (PVCs), non-sustained and sustained ventricular tachycardias (VTs), and ventricular fibrillation were observed.
- Arrhythmia origins included ventricular outflow tracts, Purkinje system, and endocardial scar patterns.
Conclusions:
- The substrate for VAs in LVNC cardiomyopathy is heterogeneous.
- Origins include ventricular outflow tracts, Purkinje system, and scar-like areas.
- These findings aid in guiding ablation strategies for VAs in LVNC.
Background:
There are limited data on ventricular arrhythmias (VAs) associated with left ventricular noncompaction (LVNC) cardiomyopathy.
Objectives:
This study aims to analyze the clinical and electrocardiographic characteristics of VAs in a group of patients with LVNC.
Methods:
Forty-two nonrelated patients with LVNC and VAs were included that were evaluated at the Inherited Cardiac Disease Unit of the University Hospital Virgen Arrixaca (Murcia-Spain) (ERN Guard-Heart Centre, European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart).
Results:
Thirteen patients (30.9%) had isolated LVNC, 27 (64.3%) had LVNC associated with dilated cardiomyopathy, and 2 (4.8%) had LVNC associated with hypertrophic cardiomyopathy. Among isolated LVNC individuals, 9 (69.2%) had premature ventricular complexes (PVCs)/nonsustained ventricular tachycardias (VTs), and 4 (30.8%) VTs (1 VT degenerating in ventricular fibrillation). In the dilated cardiomyopathy group, 11 (40.7%) patients had PVCs, 14 (51.9%) VTs, and 2 (7.4%) ventricular fibrillation. In the hypertrophic cardiomyopathy group, one patient had PVCs and the other VTs. Endocardial mapping and ablation were performed in 19 patients (45.2%): 7 ventricular outflow tracts (4 right ventricular outflow tract, 1 left coronary cusp, and 2 right coronary cusp), 2 in the left ventricular summit, 5 related to Purkinje potentials at the mid inferoseptal area, and 5 associated with endocardial scar localized in the basal anterolateral and inferolateral segments. Epicardial ablation was performed in 3 cases.
Conclusion:
The substrate of VAs in LVNC cardiomyopathy is heterogeneous, with origin in ventricular outflow tracts, Purkinje system related, and resembling scar patterns in nonischemic cardiomyopathy.
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