Peripartum Management of Patient with Long QT3 after Successful ICD Device Discharge Resulting in Device Failure: A Case Report

Background Long QT3 syndrome (LQT3) is a gain of function mutation of the SCN5A gene that is inherited in an autosomal dominant fashion. LQT3 results in an increase in arrhythmic events during rest, sleep, and bradycardia by extending the QT interval and inducing Torsades de pointes and sudden cardiac death. Attempting to block the sodium channel with Class I anti-arrhythmics or blocking adrenergic tone with beta blockers especially in women, have shown to be beneficial. There have been few large-scale studies on treating patients with LQT3 due to its lethality and underreported number of cases. Specifically, the safety and efficacy of pharmacologic treatment in pregnant LQT3 patients is unknown. Case summary This case demonstrates the safe use of Mexiletine and Propranolol in a third trimester pregnant LQT3 patient after a presumed ventricular arrhythmia and device-lead electrical short from therapy rendered her Implantable Cardioverter Defibrillator (ICD) inoperable in a VVI mode. With appropriate medications, the patient was safely monitored through the remainder of her pregnancy and safely delivered at 36 weeks of pregnancy a healthy baby girl. The daughter, heterozygous for LQT3, showed no evidence of intrauterine growth restriction or other side effects from the medications. Discussion There are many variants of the SCN5A gene mutations that can lead to different phenotypes and not all mutations are responsive to the same medications. In this case, Mexiletine and Propranolol, both of which have only recently shown to benefit certain variants or LQT3 respectively, were safely started during the third trimester of pregnancy without harming the fetus.