Incidence and type of recurrent ventricular arrhythmia in patients with arrhythmic mitral valve prolapse and implantable cardioverter defibrillator for secondary prevention

Background Mitral valve prolapse is a benign in the majority of cases, however, life-threatening ventricular arrhythmia (VA), requiring the implantation of an implantable cardioverter-defibrillator (ICD) for secondary prevention, is reported in a subset of patients. Data on the recurrence of life-threatening VA following ICD implantation are not available. Purpose This study aimed to evaluate the incidence of appropriate ICD therapies and type of arrythmia recurrence in arrhythmic mitral valve prolapse (AMVP) patients with an ICD implanted for secondary prevention. Methods A systematic literature search was conducted in PubMed/MEDLINE and EMBASE databases until January 2025. Studies reporting the incidence of appropriate ICD therapy in AMVP patients were included. Results A total of 9 studies met the inclusion criteria resulting in 163 AMVP patients with ICDs for secondary prevention. The mean age was 41.8 years, with 55.8% of patients being female. Over a weighted mean follow-up of 4.9±3.1 years, 33.4% (95%CI: 20.2-46.4) of the patients experienced recurrence of VA. The pooled incidence of appropriate ICD therapies during follow-up was 7.8 per person-year (95% CI: 5.0–10.6, I2: 30.1%). Regarding the type of arrhythmic recurrence, the majority of arrhythmias (53.3%, 95% CI: 43.8%–62.9%) were in the form of ventricular fibrillation or polymorphic ventricular tachycardia (VT), while the remaining recurrent arrhythmias were reported as monomorphic VT Conclusion AMVP patients with ICDs for secondary prevention exhibit a high risk of recurrent arrhythmias, with recurrences in the form of polymorphic VA occurring in more than half of the cases.