Correlations Between Endocardial Voltage Mapping, Diagnosis, and Genetics in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy
Section snippets
Methods
This is a retrospective study performed at the University Hospitals of Toulouse and Paris La Pitié-Salpêtrière, including all successive patients with suspected or proved ARVC who have undergone both endocardial voltage mapping and genetic testing between 2005 and 2020.
The diagnosis of ARVC has been made according to the modified Task Force criteria.2 Familial and personal cardiac history, electrocardiogram (ECG), history of ventricular arrhythmias, signal-averaged ECG, cardiac magnetic
Results
A total of 97 successive unrelated patients with suspected or proved ARVC who underwent both genetic testing and endocardial voltage mapping over a 15-year period at the University Hospital Toulouse and Paris La Pitié were included. Most of them were men (n = 77, 79%) with a mean age of 49 ± 15 years at the time of investigation (47 ± 16 at the time of diagnosis). There was no case of competitive athletes in this population. The main characteristics of the patient population are given in Table 1
Discussion
In this study, we evaluated the presence, localization, and extent of endocardial scars using electroanatomic voltage mapping in a population of 97 patients with suspected or definitively proved ARVC, according to the Task Force criteria. Our findings are as follows:
- (1)
Significant associations were present between the presence of endocardial scars and previous or inducible VT, RV function and dimensions, or ECG features of ARVC in the whole population;
- (2)
Endocardial scars were more often present in
Disclosures
Dr. Sauve is an employee of Biosense Webster. The remaining authors have no conflicts of interest to declare.
References (27)
- et al.
Clinical diagnosis, imaging, and genetics of arrhythmogenic right ventricular cardiomyopathy/dysplasia: JACC state-of-the-art review
J Am Coll Cardiol
(2018) - et al.
Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the association for molecular pathology
Genet Med
(2015) - et al.
Three-dimensional electroanatomical voltage mapping and histologic evaluation of myocardial substrate in right ventricular outflow tract tachycardia
J Am Coll Cardiol
(2008) - et al.
Utility of cardiac magnetic resonance imaging to differentiate cardiac sarcoidosis from arrhythmogenic right ventricular cardiomyopathy
Am J Cardiol
(2012) - et al.
Endocardial unipolar voltage mapping to identify epicardial substrate in arrhythmogenic right ventricular cardiomyopathy/dysplasia
Heart Rhythm
(2011) - et al.
Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria
Circulation
(2010) - et al.
Right ventricular dysplasia: a report of 24 adult cases
Circulation
(1982) - et al.
Mutation-positive arrhythmogenic right ventricular dysplasia/cardiomyopathy: the triangle of dysplasia displaced
J Cardiovasc Electrophysiol
(2013) - et al.
Short- and long-term success of substrate-based mapping and ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia
Circulation
(2005) - et al.
Ablation of ventricular arrhythmias in arrhythmogenic right ventricular dysplasia/cardiomyopathy: arrhythmia-free survival after endo-epicardial substrate based mapping and ablation
Circ Arrhythm Electrophysiol
(2011)
Combined endocardial and epicardial catheter ablation in arrhythmogenic right ventricular dysplasia incorporating scar dechanneling technique
Circ Arrhythm Electrophysiol
Outcomes of catheter ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia/cardiomyopathy
Circ Arrhythm Electrophysiol
Characterization of endocardial electrophysiological substrate in patients with nonischemic cardiomyopathy and monomorphic ventricular tachycardia
Circulation
Cited by (1)
Ventricular Tachycardia Ablation in Patients With Desmoplakin Cardiomyopathy
2024, JACC: Clinical Electrophysiology
Funding: none.