title: Comprehensive multi-modality imaging approach in arrhythmogenic cardiomyopathy-an expert consensus document of the European Association of Cardiovascular Imaging.
abstract: Arrhythmogenic cardiomyopathy (AC) is a progressive disease with high risk of life-threatening ventricular arrhythmias. A genetic mutation is found in up to 50-60% of probands, mostly affecting desmosomal genes. Diagnosis of AC is made by a combination of data from different modalities including imaging, electrocardiogram, Holter monitoring, family history, genetic testing, and tissue properties. Being a progressive cardiomyopathy, repeated cardiac imaging is needed in AC patients. Repeated imaging is important also for risk assessment of ventricular arrhythmias. This expert consensus document gives clinical recommendations for how to use multi-modality imaging in the different aspects of AC disease, including diagnosis, family screening, follow-up, risk assessment, and differential diagnosis.
authors.affiliation: Department of Cardiology, Center for Cardiological Innovation and Institute for Surgical Research, Oslo University Hospital, Oslo and University of Oslo, Oslo, Norway.
authors.forename: Kristina H
authors.initials: KH
authors.lastname: Haugaa
authors.affiliation: Cardiovascular Pathology, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Basso
authors.affiliation: Cardiology, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy.
authors.forename: Luigi P
authors.initials: LP
authors.lastname: Badano
authors.affiliation: Department of Cardiology, Bristol Heart Institute, University Hospitals Bristol NHS Trust and University of Bristol and Bristol NIRH Cardiovascular Biomedical Research Unit, Bristol, UK.
authors.forename: Chiara
authors.initials: C
authors.lastname: Bucciarelli-Ducci
authors.affiliation: Department of Cardiology, Multimodality Cardiac Imaging Center, Sports Cardiology and Cardiomyopathies Center, Hospital da Luz, Lisbon, Portugal.
authors.forename: Nuno
authors.initials: N
authors.lastname: Cardim
authors.affiliation: Interventional Cardiology and Cardiac Imaging, University Heart Center Zurich, Zurich, Switzerland.
authors.forename: Oliver
authors.initials: O
authors.lastname: Gaemperli
authors.affiliation: Department of Advanced Biomedical Sciences, Federico II University Hospital, Naples, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Galderisi
authors.affiliation: Aix-Marseille Université, Marseille and Cardiology Department, APHM, La Timone Hospital, Marseille, France.
authors.forename: Gilbert
authors.initials: G
authors.lastname: Habib
authors.affiliation: Turku PET Centre, Turku University Hospital and University of Turku, Kiinamyllynkatu, Turku, Finland.
authors.forename: Juhani
authors.initials: J
authors.lastname: Knuuti
authors.affiliation: GIGA Cardiovascular Sciences, Department of Cardiology, University of Liège Hospital, Heart Valve Clinic, CHU Sart Tilman, Liège, Belgium and Gruppo Villa Maria Care and Research, Anthea Hospital, Bari, Italy.
authors.forename: Patrizio
authors.initials: P
authors.lastname: Lancellotti
authors.affiliation: Heart Hospital, Hamad Medical Corporation, Doha, Qatar and Imperial College, London, UK.
authors.forename: William
authors.initials: W
authors.lastname: McKenna
authors.affiliation: Cardiovascular Department at Fondazione Toscana G. Monasterio, CNR Institute of Clinical Physiology and Scuola Superiore San'Anna, Pisa, Italy.
authors.forename: Danilo
authors.initials: D
authors.lastname: Neglia
authors.affiliation: University of Medicine and Pharmacy "Carol Davila"-Euroecolab, Institute of Cardiovascular Diseases "Prof. Dr. C. C. Iliescu," Bucharest, Romania.
authors.forename: Bogdan A
authors.initials: BA
authors.lastname: Popescu
authors.affiliation: Department of Cardiology, Center for Cardiological Innovation and Institute for Surgical Research, Oslo University Hospital, Oslo and University of Oslo, Oslo, Norway.
authors.forename: Thor
authors.initials: T
authors.lastname: Edvardsen
mesh_terms: D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000000981:diagnostic imaging* / Q000473:pathology*; D032921:Consensus; D004452:Echocardiography / Q000379:methods*; D017548:Echocardiography, Transesophageal / Q000379:methods; D004562:Electrocardiography / Q000379:methods; D005060:Europe; D005260:Female; D006801:Humans; D007090:Image Interpretation, Computer-Assisted; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods; D008297:Male; D064847:Multimodal Imaging / Q000379:methods; D017410:Practice Guidelines as Topic; D012680:Sensitivity and Specificity; D012955:Societies, Medical; D014057:Tomography, X-Ray Computed / Q000379:methods
keywords: arrhythmogenic cardiomyopathy; cardiac magnetic resonance; echocardiography; late gadolinium enhancement; multi-modality imaging; multidetector computed tomography; speckle tracking echocardiography