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Patients with congenital long QT syndrome (LQTS) exhibit prolongation of the QT interval on electrocardiograms (see Table 1) and are at risk for sudden cardiac death, especially during exercise or emotional distress (particularly fear). This genetic abnormality prevents the normal shortening of ventricular repolarization, leads to elevated heart rate during sympathetic activity, and can be treated with beta-blockers. To determine whether LQTS is associated with higher risk for cardiac events during pregnancy, investigators compared 115 pregnancies in 36 LQT1 mutation carriers with 67 pregnancies in 24 unaffected sisters. LQT1 is one of the most common of three variations of genetic defects responsible for the syndrome.
Most (75%) of the LQTS…