BACKGROUND:

Andersen Tawil syndrome is a very rare genetic multisystemic channelopathy without structural cardiac alteration, inherited in an autosomal dominant manner and caused by mutation in the KCNJ2 gene. This syndrome is characterised by a triad of periodic muscle paralysis, electrocardiogram and body structural changes. The hallmark is bidirectional ventricular tachycardia, premature ventricular contractions and rarely polymorphic torsade de pointes tachycardia. Currently there is a lack of guidelines for peri- and postpartum and arrhythmia prevention.

CLINICAL CASE:

21-year-old pregnant patient with Andersen-Tawil syndrome diagnosed at this age, based on a history of repeated syncope and weakness in the extremities since the age of 11. She was being treated with a beta-blocker and an implantable cardioverter defibrillator. Fetal echocardiography at 23 and 33 weeks gestation reported an apical ventricular septal defect of 1.6 mm. Elective caesarean section was performed at 39 weeks of pregnancy, with satisfactory postoperative evolution. Molecular study of the newborn ruled out congenital Andersen-Tawil syndrome.

CONCLUSION:

In patients with congenital arrhythmia syndromes, pregnancy can be safe as long as it is managed by a group of experts to make decisions and optimise care throughout the pregnancy and postpartum period.

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