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Observational Study
. 2023 Aug;9(8 Pt 3):1709-1716.
doi: 10.1016/j.jacep.2023.04.011. Epub 2023 May 24.

Arrhythmic Mitral Valve Prolapse With Only Mild or Moderate Mitral Regurgitation: Characterization of Myocardial Substrate

Marc A Miller  1 , Ana Devesa  2 , Philip M Robson  2 , Steve L Liao  3 , Renata Pyzik  2 , Ahmed El-Eshmawi  4 , Percy Boateng  4 , Dimosthenis Pandis  4 , Srinivas R Dukkipati  5 , Vivek Y Reddy  5 , David H Adams  4 , Zahi A Fayad  2 , Maria G Trivieri  2
Affiliations
Free article
Observational Study

Arrhythmic Mitral Valve Prolapse With Only Mild or Moderate Mitral Regurgitation: Characterization of Myocardial Substrate

Marc A Miller et al. JACC Clin Electrophysiol. 2023 Aug.
Free article

Abstract

Background: Sustained ventricular tachycardia and sudden cardiac death due to degenerative mitral valve prolapse (MVP) can occur in the absence of severe mitral regurgitation (MR). A significant percentage of patients with MVP-related sudden death do not have any evidence of replacement fibrosis, suggesting other unrecognized proarrhythmic factors may place these patients at risk.

Objectives: This study aims to characterize myocardial fibrosis/inflammation and ventricular arrhythmia complexity in patients with MVP and only mild or moderate MR.

Methods: Prospective observational study of patients with MVP and only mild or moderate MR underwent ventricular arrhythmia characterization and hybrid positron emission tomography (PET)/magnetic resonance imaging (MRI). Coregistered hybrid 18F-fluorodeoxyglucose (18F-FDG)-PET and MRI late gadolinium enhancement images were assessed and categorized. Recruitment occurred in the cardiac electrophysiology clinic.

Results: In 12 patients with degenerative MVP with only mild or moderate MR, of which a majority had complex ventricular ectopy (n = 10, 83%), focal (or focal-on-diffuse) uptake of 18F-FDG (PET-positive) was detected in 83% (n = 10) of patients. Three-quarters of the patients (n = 9, 75%) had FDG uptake that coexisted with areas of late gadolinium enhancement (PET/MRI-positive). Abnormal T1, T2 and extracellular volume (ECV) values were observed in 58% (n = 7), 25% (n = 3), and 16% (n = 2), respectively.

Conclusions: Most patients with degenerative MVP, ventricular ectopy, and mild or moderate MR show myocardial inflammation that is concordant with myocardial scar. Further study is needed to determine whether these findings contribute to the observation that most MVP-related sudden deaths occur in patients with less than severe MR.

Keywords: magnetic resonance imaging; mitral regurgitation; mitral valve prolapse; positron emission tomography; ventricular arrhythmia.

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Conflict of interest statement

Funding Support and Author Disclosures This work was supported by National Institutes of Health grant R01 HL071021 (Dr Fayad) and the KL2 TR001435 (Dr Trivieri). Dr Devesa is recipient of the “Alfonso Martin Escudero” grant. Dr Miller has served as a consultant to Boston Scientific. Dr Dukkipati has received research grant support from Biosense Webster. Dr Reddy has received research grants from and served as a consultant for Biosense Webster, Boston Scientific, Biotronik, and Abbott. Dr Adams declares that The Icahn School of Medicine at Mount Sinai receives royalty payments from Edwards Lifesciences for intellectual property related to the development of 2 mitral valve repair rings, and from Medtronic for intellectual property related to the development of two tricuspid valve repair rings; is the National Co-Principal Investigator for the Medtronic CoreValve, NeoChord System, Medtronic Apollo, and Triluminate-II U.S. Pivotal Trials, respectively. Icahn School of Medicine receives royalties for Dr Adam’s intellectual property from Edwards Lifesciences and Medtronic, related to valve repair rings. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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