Autoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome

Background: both myocarditis and mitral valve prolapse (MVP) are known uncommon causes of ventricular arrhythmias in young patients. Aim: to report the first clinical case of endomyocardial biopsy (EMB)-proven autoimmune myocarditis and associated arrhythmogenic MVP in a patient with recurrent ventr...

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Autores principales: Andrea Villatore, Simone Sala, Stefano Stella, Davide Vignale, Elena Busnardo, Antonio Esposito, Cristina Basso, Paolo Della Bella, Patrizio Mazzone, Giovanni Peretto
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Publicado: MDPI AG 2021
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spelling oai:doaj.org-article:f660035e6a734e55896f334173eff0b32021-11-25T18:00:23ZAutoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome10.3390/jcdd81101512308-3425https://doaj.org/article/f660035e6a734e55896f334173eff0b32021-11-01T00:00:00Zhttps://www.mdpi.com/2308-3425/8/11/151https://doaj.org/toc/2308-3425Background: both myocarditis and mitral valve prolapse (MVP) are known uncommon causes of ventricular arrhythmias in young patients. Aim: to report the first clinical case of endomyocardial biopsy (EMB)-proven autoimmune myocarditis and associated arrhythmogenic MVP in a patient with recurrent ventricular fibrillation (VF) episodes. Methods: myocarditis was diagnosed both by cardiac magnetic resonance (CMR) and EMB. Arrhythmogenic MVP was documented by transthoracic echocardiogram, CMR, and electroanatomical mapping of the trigger premature ventricular contractions (PVCs). Results: a 22-year-old woman underwent immunosuppressive therapy after EMB-proven diagnosis of autoimmune myocarditis with VF onset and early implantable cardioverter defibrillator (ICD) placement. Three years later, she experienced two VF recurrences and persistent PVCs, despite no signs of myocarditis recurrence. An echocardiogram revealed bileaflet MVP with high arrhythmic risk features. Finally, electroanatomical mapping and ablation of the trigger PVC were successfully performed. Conclusion: in patients with recurrent VF episodes despite evidence-based medical treatment for myocarditis, MVP should be considered as an alternative arrhythmogenic substrate, and warrants early ICD implant and PVC-targeted therapy.Andrea VillatoreSimone SalaStefano StellaDavide VignaleElena BusnardoAntonio EspositoCristina BassoPaolo Della BellaPatrizio MazzoneGiovanni PerettoMDPI AGarticlemyocarditisventricular arrhythmiasmitral valve prolapsearrhythmogenicsudden cardiac deathendomyocardial biopsyDiseases of the circulatory (Cardiovascular) systemRC666-701ENJournal of Cardiovascular Development and Disease, Vol 8, Iss 151, p 151 (2021)
institution DOAJ
collection DOAJ
language EN
topic myocarditis
ventricular arrhythmias
mitral valve prolapse
arrhythmogenic
sudden cardiac death
endomyocardial biopsy
Diseases of the circulatory (Cardiovascular) system
RC666-701
spellingShingle myocarditis
ventricular arrhythmias
mitral valve prolapse
arrhythmogenic
sudden cardiac death
endomyocardial biopsy
Diseases of the circulatory (Cardiovascular) system
RC666-701
Andrea Villatore
Simone Sala
Stefano Stella
Davide Vignale
Elena Busnardo
Antonio Esposito
Cristina Basso
Paolo Della Bella
Patrizio Mazzone
Giovanni Peretto
Autoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome
description Background: both myocarditis and mitral valve prolapse (MVP) are known uncommon causes of ventricular arrhythmias in young patients. Aim: to report the first clinical case of endomyocardial biopsy (EMB)-proven autoimmune myocarditis and associated arrhythmogenic MVP in a patient with recurrent ventricular fibrillation (VF) episodes. Methods: myocarditis was diagnosed both by cardiac magnetic resonance (CMR) and EMB. Arrhythmogenic MVP was documented by transthoracic echocardiogram, CMR, and electroanatomical mapping of the trigger premature ventricular contractions (PVCs). Results: a 22-year-old woman underwent immunosuppressive therapy after EMB-proven diagnosis of autoimmune myocarditis with VF onset and early implantable cardioverter defibrillator (ICD) placement. Three years later, she experienced two VF recurrences and persistent PVCs, despite no signs of myocarditis recurrence. An echocardiogram revealed bileaflet MVP with high arrhythmic risk features. Finally, electroanatomical mapping and ablation of the trigger PVC were successfully performed. Conclusion: in patients with recurrent VF episodes despite evidence-based medical treatment for myocarditis, MVP should be considered as an alternative arrhythmogenic substrate, and warrants early ICD implant and PVC-targeted therapy.
format article
author Andrea Villatore
Simone Sala
Stefano Stella
Davide Vignale
Elena Busnardo
Antonio Esposito
Cristina Basso
Paolo Della Bella
Patrizio Mazzone
Giovanni Peretto
author_facet Andrea Villatore
Simone Sala
Stefano Stella
Davide Vignale
Elena Busnardo
Antonio Esposito
Cristina Basso
Paolo Della Bella
Patrizio Mazzone
Giovanni Peretto
author_sort Andrea Villatore
title Autoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome
title_short Autoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome
title_full Autoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome
title_fullStr Autoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome
title_full_unstemmed Autoimmune Myocarditis and Arrhythmogenic Mitral Valve Prolapse: An Unexpected Overlap Syndrome
title_sort autoimmune myocarditis and arrhythmogenic mitral valve prolapse: an unexpected overlap syndrome
publisher MDPI AG
publishDate 2021
url https://doaj.org/article/f660035e6a734e55896f334173eff0b3
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