Pregnancy in women with Brugada syndrome: Is there an increased arrhythmia risk? A case-series report

Introduction In some rare arrhythmia syndromes, arrhythmia risk in female patients increases during pregnancy, necessitating extra controls. We wanted to evaluate if the increased risk for arrhythmia during pregnancy applies in women with Brugada syndrome and their potentially affected fetuses. Methods A comprehensive literature search was performed on PubMed (MeSH search terms "Brugada syndrome," "pregnancy," "parturition," "labor," "delivery," "fetal death," and "stillbirth"). Results Overall, six case reports with a total of six patients were identified. Of these six patients (three carriers of an SCN5A variant, three not tested), two women (both with unknown SCN5A status), developed severe cardiac events during pregnancy. The first patient, with a previous history of aborted sudden cardiac arrest at the age of 12 years, developed ventricular fibrillation (VF), while the other was diagnosed with Brugada syndrome postpartum because of nocturnal agonal respiration during pregnancy. Conclusion These (limited, heterogenous) cases suggest that women with Brugada syndrome (and their possibly affected fetuses), might have an overall low tendency to develop arrhythmias during pregnancy, but important data on risk factors (SCN5A status) are lacking. Arrhythmia risk during pregnancy seems to increase in probands and those who have previously experienced cardiac events. We suggest the use of risk stratification in these women to improve patient care, lower the emotional stress and physical burden for the pregnant mother, and lower health costs. Furthermore, we plead for SCN5A analysis in all these women for use of risk stratification and to enable cascade screening especially for specialized care in children carrying an SCN5A mutation.