Caring for the pregnant woman with an inherited arrhythmia syndrome

Pregnancy is a period of increased cardiovascular risk in a woman's life. In the setting of an inherited arrhythmia syndrome (IAS), cardiologists and obstetricians may be unfamiliar with cardiovascular optimization and risk stratification in pregnancy. Historically, there were little data addressing the safety of pregnancy in these rare disorders. Recent advances suggest that no type of IAS represents an absolute contraindication to pregnancy. However, it is imperative that obstetric and cardiovascular clinicians understand the major forms of IAS and how they affect the risks and course of pregnancy. This includes any disease-specific proarrhythmic triggers unique to pregnancy, such as the postpartum period in long QT syndrome (especially type 2), which poses the greatest risk of arrhythmias, and the adrenergic nature of labor and delivery, which is relevant to catecholaminergic polymorphic ventricular tachycardia. Fortunately, several effective antiarrhythmic options exist that pose little fetal risk. IAS-specific optimization of implantable cardioverter-defibrillator algorithms, drug therapy, and a maternal cardiac plan addressing the antepartum, labor, and delivery and postpartum periods reduces the risk. Where evidence does not exist, there are plausible mechanistic considerations to guide clinicians. To achieve optimal outcomes, early involvement of an expert pregnancy heart team comprising obstetrics, genetics, cardiology, and anesthesiology team members and a shared decision-making approach to IAS issues in pregnancy are needed.