Congenital LQTS is an often undetected inherited cardiac channel dysfunction and
can be a reason for intrauterine fetal demise. It can present in utero as CTG
and ultrasound abnormalities, i. e., bradycardia, ventricular tachycardia, or
fetal hydrops. Diagnosis is made by CTG, echocardiography, or fMCG. Intrauterine
therapy with a ß blocker and i. v. magnesium should be started. Our objective
was to examine the current knowledge about diagnosis and treatment of LQTS and
in particular to highlight the opportunity of vaginal birth under continuous
intravenous magnesium therapy. Therefore, a thorough MEDLINE and Google Scholar
search was conducted. Randomized controlled trials, meta-analyses, prospective
and retrospective cohort trials, and case reports were considered. We showed the
possibility of vaginal delivery under continuous magnesium therapy in a case of
suspected fetal LQTS. A stepwise concept for diagnosis, monitoring, and
peripartum management in low, intermediate, and high risk cases of fetal LQTS is
presented. If risk is low or intermediate, a vaginal delivery under continuous
monitoring is reasonable. Induction of labor at term should be evaluated.