https://orcid.org/0000-0001-6391-7244
Despite considerable therapeutic advances, ventricular arrhythmias remain responsible for sudden cardiac death in thousands of patients. Mitral annular disjunction (MAD), atrial displacement of mitral valve away from atrioventricular junction, is an under-recognized cause of arrhythmogenic sudden death. Clinical risk factors for sudden death due to MAD include female gender, young age, low ejection fraction, papillary muscle fibrosis, and high premature ventricular contraction burden. Multimodality imaging is crucial to making this diagnosis as demonstrated in a 46-year-old previously healthy woman who had a sudden ventricular fibrillation cardiac arrest. Early cardiopulmonary resuscitation and defibrillation restored a normal cardiac rhythm further treated with targeted temperature management. Echocardiography (Panel A) showed normal left ventricular (LV) ejection fraction (50%) with a curling motion of the basolateral LV wall at the posterior mitral valve leaflet insertion, concerning for MAD. Curling motion is due to paradoxical widening of the mitral annulus (solid arrow = mitral valve insertion; dashed arrow = atrioventricular junction) during the systolic phase of the cardiac cycle. A coronary computed tomography angiography (Panel B) showed no coronary disease or anomalies. A cardiac MRI confirmed MAD of 8.5 mm of the posterior mitral leaflet (Panel C) and late gadolinium enhancement (LGE) in the basal (arrow) inferolateral mid-wall distribution suggesting scar, not typical of coronary disease (Panel D). Our investigation concluded that ventricular fibrillation was due to MAD. A single-chamber implantable cardioverter-defibrillator was placed. This case emphasizes the importance of using multimodality imaging including coronary CTA, echocardiogram, and cardiac MRI in reaching appropriate diagnosis and treatment of MAD induced ventricular arrhythmias.
