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.

中文翻译：

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二尖瓣脱垂：怀孕和产后心律失常的风险

背景和目的心律失常性二尖瓣脱垂 (AMVP) 与危及生命的室性心律失常 (VA) 相关，年轻女性被认为是高危人群。妊娠期间患有恶性 VA 的女性发生 AMVP 的病例已经出现，但妊娠期间心律失常的风险尚不清楚。作者旨在描述在围产期发生恶性 VA 的高危 AMVP 女性的特征，并评估妊娠和产后期是否与恶性 VA 的较高风险相关。方法 这项回顾性国际多中心病例系列包括患有 AMVP 且经历过恶性 VA 且至少有过一次妊娠的高危女性。恶性 VA 包括心室颤动、持续性室性心动过速或植入式心律转复除颤器引起的适当电击。作者比较了非妊娠期和围产期恶性VA的发生率；后者定义为在怀孕期间和产后6个月内发生。结果 作者纳入了来自 11 个中心的 18 名 AMVP 女性。在 7.5 年（四分位距 5.8-16.6）年的随访期间，发生了 37 例恶性 VA，其中 18 例与妊娠相关，发生在 13 名 (72%) 独特患者中。与非妊娠期相比，妊娠期和产后 6 个月恶性 VA 的发生率增加（单变量发生率比 2.66，95% 置信区间 1.23-5.76）。结论 围产期可能会增加高危 AMVP 女性发生恶性 VA 的风险。这些数据可以为孕前咨询以及患者和临床医生之间细致入微的共同决策提供一般指导。