Background: Mitral valve prolapse (MVP) is commonly benign, but an arrhythmic phenotype (AMVP) with increased risk for ventricular arrhythmia (VA) and sudden cardiac death has been described. The significance of programmed ventricular stimulation (PVS) for invasive risk stratification in this entity has not yet been established.
Methods and results: 42 patients with suspected AMVP syndrome underwent programmed ventricular stimulation between 2016 and 2024 for risk stratification. Sustained VA was induced in 20 patients (47.6 %). An ICD was implanted in 14 patients with pathological risk stratification (70 %) and in 6 patients with a normal PVS (27.3 %) following shared decision making, while the remaining patients received an implantable loop recorder (ILR). During a median follow-up of 37.9 months, five patients (11.9%) experienced in total 10 appropriate ICD therapies (ATP or ICD shock for sustained VA). Secondary ICD implantation was performed in six patients based on ILR-documented significant VA (sustained VT or VT resulting in syncope). The inducibility of VA during PVS was significantly associated with either the occurrence of appropriate ICD therapies or the detection of high-risk VT on the ILR (approximate hazard ratio 4.95, p = 0.02).
Conclusion: Induction of VT/VF during invasive risk stratification in patients with AMVP was associated with the occurrence of appropriate ICD-therapies and clinically significant VA on ILR.
