Author + information
- Received December 10, 2019
- Revision received March 24, 2020
- Accepted March 24, 2020
- Published online June 15, 2020.
- Michel Haïssaguerre, MDa,b,c,∗ (, )
- Josselin Duchateau, MDa,b,c,
- Remi Dubois, PhDa,b,c,
- Mélèze Hocini, MDa,b,c,
- Ghassen Cheniti, MDa,b,c,
- Frederic Sacher, MDa,b,c,
- Thomas Lavergne, MDa,b,c,
- Vincent Probst, MDd,
- Elodie Surget, MDa,b,c,
- Ed Vigmond, PhDa,b,c,
- Nicolas Welte, MDa,b,c,
- Remi Chauvel, MDa,b,c,
- Nicolas Derval, MDa,b,c,
- Thomas Pambrun, MDa,b,c,
- Pierre Jais, MDa,b,c,
- Wee Nademanee, MDe and
- Olivier Bernus, PhDb,c
- aDepartment of Electrophysiology and Cardiac Stimulation, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France
- bInstitut Hospitalo-Universitaire Electrophysiology and Heart Modeling Institute, Centre Hospitalier Universitaire de Bordeaux, France
- cCardiothoracic Research Center Bordeaux, Université de Bordeaux, Bordeaux, France
- dThorax Institute, Université de Nantes, Nantes, France
- eCardiology Department, Bumrungrad International Hospital, Bangkok, Thailand
- ↵∗Address for correspondence:
Dr. Michel Haïssaguerre, Institute of Rhythmology and Heart Modelling, Hôpital Cardiologique Haut-Leveque, Avenue de Magellan, 33600 Bordeaux-Pessac, France.
• VF can be unexplained despite extensive investigations, notably in the young.
• The use of high-density electrophysiological mapping detects causes in the great majority of victims.
• Microstructural cardiomyopathies are the main causes, likely underlain by multiple pathological processes.
• The phenotypic characterization of substrate is critical to develop therapy and interpret genetic results.
Idiopathic ventricular fibrillation is diagnosed in patients who survived a ventricular fibrillation episode without any identifiable structural or electrical cause after extensive investigations. It is a common cause of sudden death in young adults. The study reviews the diagnostic value of systematic investigations and the new insights provided by detailed electrophysiological mapping. Recent studies have shown the high incidence of microstructural cardiomyopathic areas, which act as the substrate of ventricular fibrillation re-entries. These subclinical alterations require high-density endo- and epicardial mapping to be identified using electrogram criteria. Small areas are involved and located individually in various sites (mostly epicardial). Their characteristics suggest a variety of genetic or acquired pathological processes affecting cellular connectivity or tissue structure, such as cardiomyopathies, myocarditis, or fatty infiltration. Purkinje abnormalities manifesting as triggering ectopy or providing a substrate for re-entry represent a second important cause. The documentation of ephemeral Purkinje ectopy requires continuous electrocardiography monitoring for diagnosis. A variety of diseases affecting Purkinje cell function or conduction are potentially at play in their pathogenesis. Comprehensive investigations can therefore allow the great majority of idiopathic ventricular fibrillation to ultimately receive diagnoses of a cardiac disease, likely underlain by a mosaic of pathologies. Precise phenotypic characterization has significant implications for interpretation of genetic variants, the risk assessment, and individual therapy. Future improvements in imaging or electrophysiological methods may hopefully allow the identification of the subjects at risk and the development of primary prevention strategies.
This work was supported by the National Research Agency (ANR-10-IAHU04-LIRYC) and the European Research Council (SYMPHONY). Dr. Haissaguerre has received research grants from Biosense Webster and Medtronic. Dr. Sacher has received honoraria and consulting fees from Biosense Webster. Dr. Jais has received speaker fees from Boston Scientific and Biosense Webster. Dr. Nademanee has received research grant support from Medtronic and Biosense Webster; and has received royalties from Biosense Webster. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Clinical Electrophysiology author instructions page.
- Received December 10, 2019.
- Revision received March 24, 2020.
- Accepted March 24, 2020.
- 2020 The Authors