Arrhythmic Mitral Valve Prolapse with Features of Mitral Annular Disjunction and Myocardial Tissue Changes as Assessed with Cardiac Magnetic Resonance

  • Elen Sahara Department of Cardiology and Vascular Medicine, Faculty Medicine Universitas Indonesia, National Cardiovascular Center Harapan Kita
  • Swandito Wicaksono Department of Cardiology and Vascular Medicine, Faculty Medicine Universitas Indonesia, National Cardiovascular Center Harapan Kita
  • Celly Atmadikoesoemah Department of Cardiology and Vascular Medicine, Faculty Medicine Universitas Indonesia, National Cardiovascular Center Harapan Kita
DOI: https://doi.org/10.30701/ijc.1681
Keywords: arrhythmic mitral valve prolapse, mitral annulus disjunction, endomyocardial fibrosis

Abstract

Background. Mitral valve prolapse (MVP) is a rare disorder linked to abrupt cardiac mortality and malignant ventricular arrhythmias. Beyond conventional prognostic indicators, risk stratification may have a promising function in MVP patients, as cardiac magnetic resonance imaging (CMR) can identify tissue alterations in these patients.

Case Illustration. A 36-year-old female with palpitation, dyspnea on effort, and episode of near syncope had multifocal premature ventricular complex (PVC) with right bundle branch block patern. Bileaflet MVP with multifocal benign infrequent PVC from posteromedial papillary muscle was diagnosed in this patient based on echocardiography and holter monitoring. Mitral annular disjuction (MAD), mitral regurgitation (MR), tricuspid regurgitation (TR) were also noticed. CMR examination confirmed moderate MR ec AML-PML prolapse with MAD at PML (P1, P2, P3), moderate TR ec anterior tricuspid leaflet prolapse, myocardial inflammation and myocardial fibrosis.

Conclusion. We present case report of a young woman diagnosed with PVC predominant from posteromedial papillary muscle and bileaflet with features of MAD, myocardial inflammation and fibrosis. The arrhythmogenesis in MVP involves the development of a substrate for arrhythmias combined with a trigger for arrhythmias. Future prospective research is needed to further delineate optimal methods for risk stratification and treatment

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References

1. Haugaa KH, Aabel EW, Tani T, Konda T, Kitai T, Ota M, et al. Varied Extent of Mitral Annular Disjunction Among Cases With Different Phenotypes of Mitral Valve Prolapse. JACC Cardiovasc Imaging. 2021;3:1251-1257.
2. Dejgaard LA, Skjølsvik ET, Lie ØH, Ribe M, Stokke MK, Hegbom F, Scheirlynck ES, Gjertsen E, Andresen K, Helle-Valle TM, Hopp E, Edvardsen T, Haugaa KH. The Mitral Annulus Disjunction Arrhythmic Syndrome. J Am Coll Cardiol. 2018;72:1600-1609.
3. Freed LA, Benjamin EJ, Levy D, Larson MG, Evans JC, Fuller DL, Lehman B, Levine RA. Mitral valve prolapse in the general population: The benign nature of echocardiographic features in the Framingham Heart Study. J Am Coll Cardiol. 2002;40:1298-1304.
4. Chakrabarti AK, Bogun F, Liang JJ. Arrhythmic Mitral Valve Prolapse and Mitral Annular Disjunction: Clinical Features, Pathophysiology, Risk Stratification, and Management. J Cardiovasc Dev Dis. 2022;9.
5. Basso C, Perazzolo Marra M, Rizzo S, De Lazzari M, Giorgi B, Cipriani A, Frigo AC, Rigato I, Migliore F, Pilichou K, Bertaglia E, Cacciavillani L, Bauce B, Corrado D, Thiene G, Iliceto S. Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death. Circulation. 2015;132:556-566.
6. Garg P, Swift AJ, Zhong L, Carlhäll CJ, Ebbers T, Westenberg J, Hope MD, Bucciarelli-Ducci C, Bax JJ, Myerson SG. Assessment of mitral valve regurgitation by cardiovascular magnetic resonance imaging. Nat Rev Cardiol. 2020;17:298-312.
7. Carmo P, Andrade MJ, Aguiar C, Rodrigues R, Gouveia R, Silva JA. Mitral annular disjunction in myxomatous mitral valve disease: a relevant abnormality recognizable by transthoracic echocardiography. Cardiovasc Ultrasound. 2010;8:53.
8. Kitkungvan D, Nabi F, Kim RJ, Bonow RO, Khan MA, Xu J, Little SH, Quinones MA, Lawrie GM, Zoghbi WA, Shah DJ. Myocardial Fibrosis in Patients With Primary Mitral Regurgitation With and Without Prolapse. J Am Coll Cardiol. 2018;72:823-834.
9. Oliveri F, Kakargias F, Panday P, Arcia Franchini AP, Iskander B, Anwer F, Hamid P. Arrhythmic mitral valve prolapse: Diagnostic parameters for high-risk patients: A systematic review and meta-analysis. Pacing Clin Electrophysiol PACE. 2021;44:1746-1755.
Published
2025-01-02
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How to Cite
Sahara, E., Wicaksono, S., & Atmadikoesoemah, C. (2025). Arrhythmic Mitral Valve Prolapse with Features of Mitral Annular Disjunction and Myocardial Tissue Changes as Assessed with Cardiac Magnetic Resonance. Indonesian Journal of Cardiology, 45(1), 27-32. https://doi.org/10.30701/ijc.1681
Issue
Vol 45 No 1 (2024): January - March, 2024
Section
Case Reports

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