Ventricular arrhythmias in children with mitral valve prolapse
P Laporte, G Vaksmann, M Wilkin, E Panaioti, A Richard, A E Baruteau, N Benbrik, P Lodewyckx, V Waldmann, A Sabbag, D Kraiche, M LaredoAbstract
Background
Mitral valve prolapse (MVP) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). However, specific data in children remain limited. We aimed to assess the prevalence, phenotype, and outcomes of VA in children with MVP.
Methods
This retrospective multicenter study included patients diagnosed with MVP before age 18 years from 1998 to 2025. Clinical, ECG, Holter, and imaging data were collected. Arrhythmogenic MVP (AMVP) was defined as MVP plus ≥1 of: upper-quartile PVC burden (>41/24 h) calculated from all available Holter ECGs, SCD, arrhythmic syncope, or complex VA.
Results
Among 100 children (30% male, median diagnosis age 9.7 [IQR 6.8–12.6] years), 17% had a family history of MVP and 18% had Marfan syndrome. At baseline, two had a history of arrhythmic syncope and one of SCD. Median PVC count was 2/24h (IQR 0-34, burden 0.4±2.1%) and four (4%) patients had non-sustained VT. T-wave inversion in ≥2 inferior leads was present in 20 (20%). During a median follow-up of 6.0 (IQR 2.7–8.8) years, two patients —not classified as AMVP at baseline— had SCD, three (3%) had non-sustained VT, and one had sustained VT. Median PVC count increased to 168/24h (IQR 15-825, burden 1.1±2.7%). Clinical, ECG, and imaging markers did not distinguish AMVP from non-AMVP.
Conclusions
In this cohort, children with MVP presented with a low burden of ventricular arrhythmia, but AMVP associated with severe arrhythmic events was observed. Adult risk markers were not predictive, highlighting the need for longitudinal follow-up and identification of specific risk-stratification factors.