Arrhythmogenic Mitral Valve Prolapse Presenting with Syncope and Ventricular Tachycardia

Abstract

Mitral valve prolapse is the most common mitral valve disease in Western countries. Its two main etiologies are myxomatous degeneration (Barlow’s disease) and fibroelastic degeneration. Although generally benign, this prolapse can be associated with an increased risk of ventricular arrhythmias, ranging from simple asymptomatic extrasystoles to sudden cardiac death. Its management remains a challenge for clinicians. We report the case of a 56-year-old man with no significant medical history, admitted for a sudden syncope without prodrome, preceded by progressive dyspnea over a week. Clinical examination revealed an intense holosystolic murmur (grade 3/6) at the mitral focus (apical area). The electrocardiogram showed ventricular extrasystoles likely originating from the posterior papillary muscle. Echocardiography revealed severe mitral regurgitation due to a prolapse of the P1-P2 leaflets with chordal rupture. Given the severity of the valvular damage, an emergency surgical mitral valve repair was performed. Postoperatively, the patient experienced an episode of sustained ventricular tachycardia, requiring the implantation of an implantable cardioverter-defibrillator (ICD) for secondary prevention. This case highlights the importance of screening for arrhythmogenic mitral valve prolapse, which can present with initial syncope and severe ventricular arrhythmias. Close monitoring and a multidisciplinary approach are crucial to prevent serious complications, including sudden cardiac death. Early diagnosis and regular follow-up of patients with Mitral Valve Prolapse, especially those at risk for arrhythmias, are essential. Identifying Mitral Annular Disjunction in these patients is also key for proper management. Lastly, transesophageal echocardiography and magnetic resonance imaging are valuable tools for diagnosing and monitoring these patients. These aspects should encourage further research to enhance management strategies and reduce complications associated with MVP.