학술논문
Correlations Between Endocardial Voltage Mapping, Diagnosis, and Genetics in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy.
Document Type
Academic Journal
Author
Delasnerie H; Department of Cardiology, Cardiology University Hospital Toulouse, Toulouse, France.; Gandjbakhch E; Department of Cardiology, Sorbonne Universités, AP-HP, Heart Institute, La Pitié-Salpêtrière University Hospital, Paris, France.; Sauve R; Biosense, Johnson & Johnson, Issy-les-Moulineaux, France.; Beneyto M; Department of Cardiology, Cardiology University Hospital Toulouse, Toulouse, France.; Domain G; Department of Cardiology, Cardiology University Hospital Toulouse, Toulouse, France.; Voglimacci-Stephanopoli Q; Department of Cardiology, Cardiology University Hospital Toulouse, Toulouse, France.; Mandel F; Department of Cardiology, Cardiology University Hospital Toulouse, Toulouse, France.; Badenco N; Department of Cardiology, Sorbonne Universités, AP-HP, Heart Institute, La Pitié-Salpêtrière University Hospital, Paris, France.; Waintraub X; Department of Cardiology, Sorbonne Universités, AP-HP, Heart Institute, La Pitié-Salpêtrière University Hospital, Paris, France.; Mondoly P; Department of Cardiology, Cardiology University Hospital Toulouse, Toulouse, France.; Fressart V; Service de Biochimie Métabolique, La Pitié-Salpêtrière University Hospital, Paris, France.; Rollin A; Department of Cardiology, Cardiology University Hospital Toulouse, Toulouse, France.; Maury P; Department of Cardiology, Cardiology University Hospital Toulouse, Toulouse, France; I2MC, Inserm UMR 1297, Toulouse, France. Electronic address: mauryjphil@hotmail.com.
Source
Publisher: Excerpta Medica Country of Publication: United States NLM ID: 0207277 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1879-1913 (Electronic) Linking ISSN: 00029149 NLM ISO Abbreviation: Am J Cardiol Subsets: MEDLINE
Subject
Language
English
Abstract
The relations between endocardial voltage mapping and the genetic background of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been investigated so far. A total of 97 patients with proved or suspected ARVC who underwent 3-dimensional endocardial mapping and genetic testing have been retrospectively included. Presence, localization, and size of scar areas were correlated to ARVC diagnosis and the presence of a pathogenic variant. A total of 78 patients (80%) presented with some bipolar or unipolar scar on endocardial voltage mapping, whereas 43 carried pathogenic variants (44%). Significant associations were observed between presence of endocardial scars on voltage mapping and previous or inducible ventricular tachycardia, right ventricular function and dimensions, or electrocardiogram features of ARVC. A total of 60 of the 78 patients (77%) with an endocardial scar fulfilled the criteria for a definitive arrhythmogenic right ventricular dysplasia diagnosis versus 8 of 19 patients (42%) without scar (p = 0.003). Patients with a definitive diagnosis of ARVC had more scars from any location and the scars were larger in patients with ARVC. In the 68 patients with a definitive diagnosis of ARVC, the presence of any endocardial scar was similar whether an ARVC-causal mutation was present or not. Only scar extent was significantly greater in patients with pathogenic variants. There was no difference in the presence and characteristics of scars in PKP2 mutated versus other mutated patients. The 3-dimensional endocardial mapping could have an important role for refining ARVC diagnosis and may be able to detect minor forms with otherwise insufficient criteria for diagnosis. The trend for larger scar extent were observed in mutated patients, without any difference according to the mutated genes.
Competing Interests: Disclosures Dr. Sauve is an employee of Biosense Webster. The remaining authors have no conflicts of interest to declare.
(Copyright © 2022 Elsevier Inc. All rights reserved.)
Competing Interests: Disclosures Dr. Sauve is an employee of Biosense Webster. The remaining authors have no conflicts of interest to declare.
(Copyright © 2022 Elsevier Inc. All rights reserved.)