Long QT Syndrome Management during and after Pregnancy

Long QT syndrome (LQTS) is majorly an autosomal dominantly inherited electrical dysfunction, but there are exceptions (Jervell and Lange-Nielsen syndrome is inherited in an autosomal recessive pattern). This disorder prolongs ventricular repolarization and increases the risk of ventricular arrhythmias, syncope, and even sudden cardiac death. The risk of fatal events is reduced during pregnancy, but dramatically increases during the 9 months after delivery, especially in patients with LQT2. In women with LQTS, treatment with β-blockers at appropriate doses is recommended throughout pregnancy and the high-risk postnatal period. In this review, we summarize the management of LQTS during pregnancy and beyond.