Sudden cardiac death unveiling a mitral annular disjunction in a young man: The role of multimodal cardiac imaging for diagnosis

Non-ischemic structural heart diseases, such as mitral annular disjunction (MAD) and/or mitral valve prolapse (MVP), are known to increase the risk of arrhythmias and sudden cardiac death (SCD). We report a rare case of SCD in a young patient, secondary to ventricular fibrillation, revealing a MAD. A 20-year-old man was admitted to our hospital after a sudden cardiac arrest at home. Immediate cardiopulmonary resuscitation was initiated with chest compressions. Semi-automatic defibrillator tracings demonstrated ventricular fibrillation, and three rounds of defibrillation were performed with return of spontaneous circulation within approximately 15 min. Electrocardiogram showed T-wave inversion in inferior-lateral leads. Transthoracic echocardiography and cardiac magnetic resonance imaging revealed a MAD and a bileaflet MVP without obvious valve regurgitation. The patient was diagnosed with ventricular fibrillation, likely resulting from MAD. He was initiated on a daily regimen of bisoprolol and received a subcutaneous implantable cardioverter-defibrillator. In-hospital evolution was favorable. Cognitive impairment was noted, leading to a referral for rehabilitation. In conclusion, this case involves a cardiac arrest likely caused by valvular heart disease. Multimodal cardiac imaging contribution is fundamental in the diagnosis of MAD with MVP, which is an uncommon cause of cardiac arrest.

Learning objectives

This case highlights the utility of multimodal cardiac imaging in mitral annular disjunction (MAD) diagnosis and the management of emergency care. Isolated MAD is not an indication for implantable cardioverter-defibrillator (ICD) placement, but the sudden death due to ventricular fibrillation that was recovered in this patient makes the implantation of a prophylactic ICD worth considering.