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

    Authors

    Keywords

    arrhythmias, echocardiography, mitral valve prolapse, sudden cardiac death

    DOI

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

    Full Text

    **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**Figure 2). 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. FIGURE 2. Cardiac magnetic resonance (2 chamber view) - separation between the ventricular myocardium and the mitral annulus. **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
    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
    Cardiologia Croatica
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    Mitral annular disjunction as a risk factor for sudden cardiac death – a clinical case of a sudden cardiac death survivor with mitral annular disjunction

    Extended Abstract
    Issue11-12
    Published
    Pages547
    PDF via DOIhttps://doi.org/10.15836/ccar2024.547
    arrhythmias
    echocardiography
    mitral valve prolapse
    sudden cardiac death

    Authors

    Helena JerkićORCIDUniversity Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
    Antun Lončarić*ORCIDGeneral Hospital ‘’dr. Ivo Pedišić’’, Sisak, Croatia
    Krešimir CrljenkoORCIDUniversity Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
    Iva KlobučarORCIDUniversity Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
    Zdravko BabićORCIDUniversity Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
    Vjekoslav RadeljićORCIDUniversity Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia
    Diana Delić-BrkljačićORCIDUniversity Hospital Centre “Sestre milosrdnice”, Zagreb, Croatia

    *Correspondence email: antun1412@gmail.com

    Full Text

    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 2Figure 2). 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.

    FIGURE 2. Cardiac magnetic resonance (2 chamber view) - separation between the ventricular myocardium and the mitral annulus.

    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. 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.DOI
    2. 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.DOI