The Many Faces of Arrhythmic Mitral Valve Prolapse: Case Series

Abstract

Background:

Although it is frequently a benign condition, mitral valve prolapse (MVP) may be associated with an increased risk of ventricular arrhythmias (VA), a condition known as arrhythmic mitral valve prolapse (AMVP).

Objectives:

To present diverse manifestations of AMVP by means of clinical cases that illustrate these patients’ symptoms and electrocardiogram (ECG), 24-hour Holter, transthoracic echocardiogram (TTE), and cardiac magnetic resonance imaging (CMRI) findings and to discuss the decisions made in relation to different clinical outcomes.

Methods:

This retrospective, descriptive, and observational study analyzed 5 patients with AMVP treated between 2019 and 2024, investigating markers of elevated risk for severe clinical outcomes, especially sudden cardiac death (SCD).

Results:

Among the 5 patients evaluated, the most common clinical presentation was palpitations (100% of cases), followed by syncope (40%). Three patients (60%) had severe or very severe arrhythmia on 24-hour Holter monitoring, while 4 (80%) had mitral annular disjunction (MAD). Positive delayed enhancement was observed in 2 (40%) patients. In 2 cases (40%), implantable cardioverter-defibrillator (ICD) was indicated. In 1 case, mitral valve replacement was chosen, but the arrhythmias were not resolved; consequently, radiofrequency ablation was indicated. Although SCD has been described in the literature, no cases were observed in our sample.

Conclusion:

AMVP may present distinct clinical manifestations, including severe outcomes. It is essential to identify risk markers for early diagnosis and treatment of this condition, with the goal of reducing SCD-related mortality in these patients.