Alexandros Protonotarios1, Eleanor Wicks2, Michael Ashworth3, Edward Stephenson4, Oliver Guttmann5, Kostas Savvatis4, Neha Sekhri6, Saidi A Mohiddin4, Petros Syrris7, Leon Menezes8, Perry Elliott9. 1. Institute for Cardiovascular Science, University College London, London, UK; Barts Heart Centre, St Bartholomew's Hospital, London, UK. Electronic address: alexanderproton@gmail.com. 2. Institute for Cardiovascular Science, University College London, London, UK; Barts Heart Centre, St Bartholomew's Hospital, London, UK; John Radcliffe Hospital, Oxford University Hospitals, Oxford, UK. 3. Department of Pathology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. 4. Barts Heart Centre, St Bartholomew's Hospital, London, UK; William Harvey Research Institute, London, UK. 5. Institute for Cardiovascular Science, University College London, London, UK; Barts Heart Centre, St Bartholomew's Hospital, London, UK. 6. Barts Heart Centre, St Bartholomew's Hospital, London, UK. 7. Institute for Cardiovascular Science, University College London, London, UK. 8. Barts Heart Centre, St Bartholomew's Hospital, London, UK; Institute of Nuclear Medicine, University College London, London, UK; NIHR University College London Hospitals Biomedical Research Centre, UK. 9. Institute for Cardiovascular Science, University College London, London, UK; Barts Heart Centre, St Bartholomew's Hospital, London, UK; NIHR University College London Hospitals Biomedical Research Centre, UK.
Abstract
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable heart muscle disease that causes sudden cardiac death in the young. Inflammatory myocardial infiltrates have been described at autopsy and on biopsy, but there are few data on the presence of myocarditis in living patients with ARVC using non-invasive imaging techniques. FDG-PET is a validated technique for detecting myocardial inflammation in clinically suspected myocarditis. We aimed to determine the prevalence of myocardial inflammation in patients with ARVC using 18F-fluorodeoxyglucose positron emission tomography (FDG-PET). METHODS AND RESULTS: We performed a retrospective analysis of a single centre cohort of patients with ARVC referred for FDG-PET scans between 2012 and 2017 for investigation of symptoms or suspected device infection. Sixteen patients (12 male; age 42 ± 13 years) with a definite diagnosis of ARVC were identified. Seven had positive FDG-PET scans, two of whom had cardiac sarcoidosis on endomyocardial biopsy. Of the remaining five, two carried pathogenic desmoplakin mutations. FDG uptake was found in the left ventricular myocardium in all cases. One patient also had right ventricular uptake. CONCLUSION: In this exploratory study, we show that some patients with ARVC have evidence for myocardial inflammation on FDG-PET, suggesting that myocarditis plays a role in disease pathogenesis.
BACKGROUND:Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable heart muscle disease that causes sudden cardiac death in the young. Inflammatory myocardial infiltrates have been described at autopsy and on biopsy, but there are few data on the presence of myocarditis in living patients with ARVC using non-invasive imaging techniques. FDG-PET is a validated technique for detecting myocardial inflammation in clinically suspected myocarditis. We aimed to determine the prevalence of myocardial inflammation in patients with ARVC using 18F-fluorodeoxyglucose positron emission tomography (FDG-PET). METHODS AND RESULTS: We performed a retrospective analysis of a single centre cohort of patients with ARVC referred for FDG-PET scans between 2012 and 2017 for investigation of symptoms or suspected device infection. Sixteen patients (12 male; age 42 ± 13 years) with a definite diagnosis of ARVC were identified. Seven had positive FDG-PET scans, two of whom had cardiac sarcoidosis on endomyocardial biopsy. Of the remaining five, two carried pathogenic desmoplakin mutations. FDG uptake was found in the left ventricular myocardium in all cases. One patient also had right ventricular uptake. CONCLUSION: In this exploratory study, we show that some patients with ARVC have evidence for myocardial inflammation on FDG-PET, suggesting that myocarditis plays a role in disease pathogenesis.
Authors: Sanjay Divakaran; Garrick C Stewart; Neal K Lakdawala; Robert F Padera; Wunan Zhou; Akshay S Desai; Michael M Givertz; Mandeep R Mehra; Raymond Y Kwong; Sandeep S Hedgire; Brian B Ghoshhajra; Viviany R Taqueti; Hicham Skali; Sharmila Dorbala; Ron Blankstein; Marcelo F Di Carli Journal: Circ Cardiovasc Imaging Date: 2019-06-10 Impact factor: 7.792
Authors: J Lukas Laws; Megan C Lancaster; M Ben Shoemaker; William G Stevenson; Rebecca R Hung; Quinn Wells; D Marshall Brinkley; Sean Hughes; Katherine Anderson; Dan Roden; Lynne W Stevenson Journal: Circ Res Date: 2022-05-26 Impact factor: 23.213
Authors: Babken Asatryan; Angeliki Asimaki; Andrew P Landstrom; Mohammed Y Khanji; Katja E Odening; Leslie T Cooper; Francis E Marchlinski; Anna R Gelzer; Christopher Semsarian; Tobias Reichlin; Anjali T Owens; C Anwar A Chahal Journal: Circulation Date: 2021-11-15 Impact factor: 39.918
Authors: Eric D Smith; Neal K Lakdawala; Nikolaos Papoutsidakis; Gregory Aubert; Andrea Mazzanti; Anthony C McCanta; Prachi P Agarwal; Patricia Arscott; Lisa M Dellefave-Castillo; Esther E Vorovich; Kavitha Nutakki; Lisa D Wilsbacher; Silvia G Priori; Daniel L Jacoby; Elizabeth M McNally; Adam S Helms Journal: Circulation Date: 2020-05-06 Impact factor: 29.690
Authors: Viraj Patel; Babken Asatryan; Bhurint Siripanthong; Patricia B Munroe; Anjali Tiku-Owens; Luis R Lopes; Mohammed Y Khanji; Alexandros Protonotarios; Pasquale Santangeli; Daniele Muser; Francis E Marchlinski; Peter A Brady; C Anwar A Chahal Journal: Int J Mol Sci Date: 2020-09-10 Impact factor: 5.923
Authors: Carsten Tschöpe; Enrico Ammirati; Biykem Bozkurt; Alida L P Caforio; Leslie T Cooper; Stephan B Felix; Joshua M Hare; Bettina Heidecker; Stephane Heymans; Norbert Hübner; Sebastian Kelle; Karin Klingel; Henrike Maatz; Abdul S Parwani; Frank Spillmann; Randall C Starling; Hiroyuki Tsutsui; Petar Seferovic; Sophie Van Linthout Journal: Nat Rev Cardiol Date: 2020-10-12 Impact factor: 49.421