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Prenatal Recommendations in Long QT Syndrome
Once pregnancy is confirmed in a woman with LQTS, a management plan for pregnancy and the 9-month postpartum period should be made in collaboration with the obstetrician and cardiologist with ongoing review and discussion throughout. Women should be educated and counseled on potential triggers of LQTS cardiac events including avoidance of hypokalemia and QT-prolonging drugs, which can be checked on the Credible Meds website. Several recommendations regarding the management of pregnant patients with LQTS have been included in other guidelines.18 An ECG should be performed at each visit to evaluate the corrected QT interval. Electrolytes and vitamin D levels should be monitored as mild hypomagnesemia and vitamin D deficiency are common during pregnancy and could put the mother and fetus at avoidable risk. Encouraging increased potassium and magnesium intake is reasonable. A maternal-fetal medicine specialist with expertise in the prenatal diagnosis and care of fetal LQTS can assist parents during the prenatal period. Likewise, early referral to a pediatric cardiologist familiar with LQTS will help expedite screening of the newborn and preventive therapy in the event that the baby is diagnosed with congenital LQTS.
Management of Women with Long QT Syndrome During Pregnancy
Treatment with a β-blocker is indicated to reduce risk of cardiac events and sudden cardiac death.14,18–20 Guidelines for management of ventricular arrhythmias and prevention of sudden cardiac death strongly recommend that in women with LQTS, a β-blocker should be continued during pregnancy and the postpartum period regardless of symptoms, including while breastfeeding.18 Arrhythmic events during pregnancy are not increased among women receiving β-blocker therapy.11,12,14,19 In contrast, in a case-control study, women with LQT1 who did not receive β-blockers during pregnancy were at increased risk of cardiac arrest or syncope.13 In at least one study, increased risk for cardiac events in the high-risk postpartum period was significantly reduced by β-blockers.14
Not all β-blockers are equally effective in LQTS (Table 1).21 In general, non-selective β-blockers, such as nadolol and propranolol, are preferred over the β1-selective agents, such as metoprolol. Documented evidence, however, is largely lacking comparing specific agents in LQTS during pregnancy and lactation. Most data are primarily limited to isolated small case reports. Nadolol titrated to a recommended dose of 1–1.5 mg/kg/day is prescribed for LQTS by the majority of electrophysiologists as it is the most effective β-blocker for the syndrome, especially in high-risk individuals.22,23 The longer half-life of nadolol compared with other β-blockers also gives it an advantage for LQTS. However, data supporting the use of nadolol in pregnancy are limited. Nadolol is highly excreted in breastmilk and infants exposed to nadolol via breast milk should be monitored for side-effects, such as bradycardia, lethargy, poor feeding or weight gain. Propranolol or bisoprolol appear to have acceptable efficacy and safety profiles in LQTS and during pregnancy and lactation.24,25
Management of Long QT Syndrome in Women Before, During, and After Pregnancy
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