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Prenatal diagnosis and In Utero treatment of Torsades de Pointes associated with congenital long QT syndrome

      Congenital long QT syndrome (LQTS) is an inherited disorder of myocardial repolarization resulting from gene mutations in potassium and sodium ion channels.
      • Keating M.T.
      • Sanguinetti M.C.
      Molecular and cellular mechanisms of cardiac arrhythmias.
      The prenatal diagnosis of LQTS is often retrospective for several reasons. First, fetal echocardiography identifies only the mechanical consequences of electrophysiologic events,
      • Silverman N.H.
      • Enderlein M.A.
      • Stanger P.
      • Teitel D.F.
      • Heymann M.A.
      • Golbus M.S.
      Recognition of fetal arrhythmias by echocardiography.
      and fetal electrocardiograms do not provide tracings of adequate quality for clinical application. Second, mothers with the LQTS mutation whose offspring are at increased risk for LQTS may have normal QTc intervals on the baseline electrocardiogram.
      • Vincent G.M.
      • Timothy K.W.
      • Leppert M.
      • Keating M.
      The spectrum of symptoms and QT intervals in carriers of the gene for the long-QT syndrome.
      Finally, the typical presentation of fetal LQTS is asymptomatic and mild sinus bradycardia at rates at or barely below normal.
      • Hofbeck M.
      • Ulmir H.
      • Beinder E.
      • Sieber E.
      • Singer H.
      Prenatal findings with prolonged QT interval in the neonatal period.
      ,
      • Beinder E.
      • Grancay T.
      • Menendez T.
      • Singer H.
      • Hofbeck M.
      Fetal sinus bradycardia and the long QT syndrome.
      ,
      • Vigliani M.
      Romano-Ward syndrome diagnosed as moderate fetal bradycardia. A case report.
      ,
      • Hamada H.
      • Horigome H.
      • Asaka M.
      • Shigemitsu S.
      • Mitsui T.
      • Kubo T.
      • Kandori A.
      • Tsukada K.
      Prenatal diagnosis of long QT syndrome using fetal magnetocardiography.
      ,
      • Donofrio M.T.
      • Gallquist S.D.
      • O’Connell N.G.
      • Redwine F.O.
      Fetal presentation of congenital long QT syndrome.
      A rare presentation is second-degree atrioventricular block and ventricular tachycardia leading to congestive heart failure.
      • Hofbeck M.
      • Ulmir H.
      • Beinder E.
      • Sieber E.
      • Singer H.
      Prenatal findings with prolonged QT interval in the neonatal period.
      ,
      • Yamada M.
      • Nakazawa M.
      • Momma K.
      Fetal ventricular tachycardia in the long QT syndrome.
      ,
      • Ohkuchi A.
      • Shiraishi H.
      • Minakami H.
      • Eguchi Y.
      • Izumi A.
      • Sato I.
      Fetus with long QT syndrome manifested by tachyarrhythmia a case report.
      ,
      • Wu M.H.
      • Hsieh F.C.
      • Wang J.K.
      • Kau M.L.
      A variant of long QT syndrome manifested as fetal tachycardia associated with ventricular septal defect.
      We describe the diagnosis and treatment of a hydropic fetus with sustained ventricular tachycardia and atrioventricular block. LQTS was diagnosed by fetal magnetocardiography based on QTc prolongation and Torsades de Pointes.
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