Mitral annular disjunction arrhythmic syndrome in a woman in her 40s with bileaflet prolapse and focal myocardial fibrosis presented as ventricular fibrillation arrest requiring ICD implantation.
Case Report (n=1)
This case highlights the risk of malignant ventricular arrhythmia and sudden cardiac arrest in patients with mitral annular disjunction and bileaflet mitral valve prolapse, even when graded as intermediate risk.
Abstract Background Mitral annular disjunction with bileaflet mitral valve prolapse is increasingly recognized as a substrate for malignant ventricular arrhythmia and sudden cardiac arrest. Prior myocardial fibrosis adjacent to papillary muscles and frequent ventricular ectopy increase arrhythmic risk. Case Summary We describe the case of a woman in her 40s who suffered an out-of-hospital ventricular fibrillation cardiac arrest with immediate bystander CPR and ambulance arrival within 5 min. Return of spontaneous circulation occurred approximately 30 minutes after multiple shocks. She had a background of bileaflet mitral valve prolapse discovered on routine investigation following a syncopal episode in her youth. She was known to have significant mitral annular disjunction and focal mid-wall/subepicardial late gadolinium enhancement on serial cardiac MRI. An implantable loop recorder previously demonstrated frequent multifocal ventricular ectopy but had reached end-of-life by the time of the case. Subsequent testing excluded coronary disease, pulmonary embolus, and long QT as causes for her arrest. A transvenous implantable cardioverter defibrillator was inserted for secondary prevention and pacing capability should she require it, with mitral valve replacement following this. Discussion This case exemplifies mitral annular disjunction arrhythmic syndrome presenting as ventricular fibrillation arrest in a patient with bileaflet prolapse, focal myocardial fibrosis, and frequent ventricular ectopy, managed with ICD implantation and eventual surgical valve replacement. Current consensus would have graded her arrhythmic risk as intermediate.
Smman et al. (Thu,) conducted a case report in Mitral annular disjunction arrhythmic syndrome (n=1). ICD implantation and mitral valve replacement was evaluated. Mitral annular disjunction arrhythmic syndrome in a woman in her 40s with bileaflet prolapse and focal myocardial fibrosis presented as ventricular fibrillation arrest requiring ICD implantation.