BACKGROUND AND AIMS: Arrhythmic mitral valve prolapse (AMVP) is linked to life-threatening ventricular arrhythmias (VAs), and young women are considered at high risk. Cases of AMVP in women with malignant VA during pregnancy have emerged, but the arrhythmic risk during pregnancy is unknown. The authors aimed to describe features of women with high-risk AMVP who developed malignant VA during the perinatal period and to assess if pregnancy and the postpartum period were associated with a higher risk of malignant VA. METHODS: This retrospective international multi-centre case series included high-risk women with AMVP who experienced malignant VA and at least one pregnancy. Malignant VA included ventricular fibrillation, sustained ventricular tachycardia, or appropriate shock from an implantable cardioverter defibrillator. The authors compared the incidence of malignant VA in non-pregnant periods and perinatal period; the latter defined as occurring during pregnancy and within 6 months after delivery. RESULTS: The authors included 18 women with AMVP from 11 centres. During 7.5 (interquartile range 5.8-16.6) years of follow-up, 37 malignant VAs occurred, of which 18 were pregnancy related occurring in 13 (72%) unique patients. Pregnancy and 6 months after delivery showed increased incidence rate of malignant VA compared to the non-pregnancy period (univariate incidence rate ratio 2.66, 95% confidence interval 1.23-5.76). CONCLUSIONS: The perinatal period could impose increased risk of malignant VA in women with high-risk AMVP. The data may provide general guidance for pre-conception counselling and for nuanced shared decision-making between patients and clinicians.

• Keywords
• Arrhythmic mitral valve prolapse, Cardiomyopathy, Mitral annular disjunction, Pregnancy, Ventricular arrhythmia,
• MeSH
• Defibrillators, Implantable MeSH
• Adult MeSH
• Ventricular Fibrillation epidemiology   etiology   MeSH
• Incidence MeSH
• Pregnancy Complications, Cardiovascular * epidemiology   MeSH
• Tachycardia, Ventricular epidemiology   etiology   MeSH
• Humans MeSH
• Postpartum Period MeSH
• Puerperal Disorders epidemiology   etiology   MeSH
• Mitral Valve Prolapse * complications   epidemiology   MeSH
• Retrospective Studies MeSH
• Risk Factors MeSH
• Arrhythmias, Cardiac epidemiology   etiology   MeSH
• Pregnancy MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Pregnancy MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study MeSH