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Meeting abstract

https://doi.org/10.15836/ccar2024.547

Mitral annular disjunction as a risk factor for sudden cardiac death – a clinical case of a sudden cardiac death survivor with mitral annular disjunction

Helena Jerkić orcid id orcid.org/0000-0002-1650-4735 ; University Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
Antun Lončarić orcid id orcid.org/0000-0001-7771-2549 ; General Hospital ‘’dr. Ivo Pedišić’’, Sisak, Croatia
Krešimir Crljenko orcid id orcid.org/0000-0002-5753-9475 ; University Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
Iva Klobučar orcid id orcid.org/0000-0002-0640-7149 ; University Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
Zdravko Babić orcid id orcid.org/0000-0002-7060-8375 ; University Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
Vjekoslav Radeljić orcid id orcid.org/0000-0003-2471-4035 ; University Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
Diana Delić-Brkljačić orcid id orcid.org/0000-0002-7116-2360 ; University Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia


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Abstract

Keywords

arrhythmias; echocardiography; mitral valve prolapse; sudden cardiac death

Hrčak ID:

328440

URI

https://hrcak.srce.hr/328440

Publication date:

13.12.2024.

Visits: 330 *



Introduction: Mitral annular disjunction is a rare and poorly recognized condition which involves the separation between the ventricular myocardium and the mitral annulus during systole (1,2). Mitral annular disjunction is a risk marker for ventricular arrhythmias and sudden cardiac death and is often associated with mitral valve prolapse.

Case report: 32-year-old female presented to the hospital after successful resuscitation of out-of-hospital cardiac arrest. She has been followed up by a cardiologist, due to mitral valve prolapse and palpitations since age of 15. Echocardiography at admission showed dilated, globally hypokinetic left ventricle, with severely reduced systolic function, thickened mitral valve cusps, bileaflet mitral valve prolapse, with mild mitral regurgitation and without pericardial effusion. Coronary angiography excluded coronary artery disease. Targeted temperature management was maintained. Heart failure therapy have been administrated, as well as antiarrhythmic therapy with amiodarone. Blood samples were sent for genetic analysis were negative for arrhythmias and cardiomyopathies. Cardiac magnetic resonance imaging revealed normal left ventricular dimensions, basal inferoseptal wall hypertrophy, with mildly reduced systolic function of left ventricle. Additionally, mild mitral regurgitation, bileaflet mitral valve prolapse and insertion point of posterolateral annulus 6 mm out of left ventricular myocardium, suggestive for mitral annular disjunction were shown (Figures 1 and 2{ label needed for fig[@id='f2'] }). There was no late postcontrast imbibition. Furthermore, patient received implantable cardioverter defibrillator for secondary prevention of sudden cardiac death and was discharged with bisoprolol and amiodarone. No neurological deficits remained after the neurorehabilitation.

FIGURE 1 Cardiac magnetic resonance (4 chamber view) - separation between the ventricular myocardium and the mitral annulus.
CC202419_11-12_547-f1
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FIGURE 2 Cardiac magnetic resonance (2 chamber view) - separation between the ventricular myocardium and the mitral annulus.
CC202419_11-12_547-f2

Conclusion: This case report emphasizes the importance of awareness and diagnosis of mitral annular disjunction, particularly in patients presenting with ventricular arrhythmias, syncope or cardiac arrest. Recognition and diagnosis of mitral annular disjunction, with or without mitral valve prolapse, should be routinely done in practice.

LITERATURE

1 

Sabbag A, Essayagh B, Barrera JDR, Basso C, Berni A, Cosyns B, et al. EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction complex. Europace. 2022 December 9;24(12):1981–2003. https://doi.org/10.1093/europace/euac125 PubMed: http://www.ncbi.nlm.nih.gov/pubmed/35951656

2 

Essayagh B, Sabbag A, Antoine C, Benfari G, Batista R, Yang LT, et al. The mitral annular disjunction of mitral valve prolapse: presentation and outcome. JACC Cardiovasc Imaging. 2021;14:2073–87. https://doi.org/10.1016/j.jcmg.2021.04.029 PubMed: http://www.ncbi.nlm.nih.gov/pubmed/34147457


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