J Obstet Gynaecol Res. 2026 Jan;52(1):e70181. doi: 10.1111/jog.70181.
ABSTRACT
Andersen-Tawil syndrome (ATS), also known as congenital long QT syndrome type 7 (LQT7), is a rare inherited channelopathy caused by KCNJ2 mutations and characterized by ventricular arrhythmias, periodic paralysis, and dysmorphic features. Pregnancy in women with ATS is extremely uncommon, and optimal perinatal management strategies are not well defined. We describe a 38-year-old primigravida with genetically confirmed ATS (p.R228X) who presented at 31 weeks of gestation without medication. During pregnancy, the burden of premature ventricular contractions and non-sustained ventricular tachycardia decreased compared with the pre-pregnancy period. At 36 weeks, atenolol and flecainide were introduced, and a wearable cardioverter-defibrillator (WCD) was applied, further reducing arrhythmias. A planned cesarean section at 37 weeks was uneventful, with favorable maternal and fetal outcomes. Postpartum, arrhythmias increased again, highlighting the importance of surveillance. This case demonstrates that combined pharmacological therapy and temporary WCD use may represent a safe and effective perinatal management strategy in high-risk pregnancies complicated by ATS.
PMID:41482511 | DOI:10.1111/jog.70181