Preventing and Treating Torsades de Pointes in the Mother, Fetus and Newborn in the Highest Risk Pregnancies with Inherited Arrhythmia Syndromes

The number of women of childbearing age who have been diagnosed in childhood with ion channelopathy and effectively treated using beta blockers, cardiac sympathectomy, and life-saving cardiac pacemakers/defibrillators is increasing. Since many of these diseases are inherited as autosomal dominant, o...

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Main Authors: Annette Wacker-Gussmann, Gretchen K. Eckstein, Janette F. Strasburger
Format: Article
Language:English
Published: MDPI AG 2023-05-01
Series:Journal of Clinical Medicine
Subjects:
Online Access:https://www.mdpi.com/2077-0383/12/10/3379
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author Annette Wacker-Gussmann
Gretchen K. Eckstein
Janette F. Strasburger
author_facet Annette Wacker-Gussmann
Gretchen K. Eckstein
Janette F. Strasburger
author_sort Annette Wacker-Gussmann
collection DOAJ
description The number of women of childbearing age who have been diagnosed in childhood with ion channelopathy and effectively treated using beta blockers, cardiac sympathectomy, and life-saving cardiac pacemakers/defibrillators is increasing. Since many of these diseases are inherited as autosomal dominant, offspring have about a 50% risk of having the disease, though many will be only mildly impacted during fetal life. However, highly complex delivery room preparation is increasingly needed in pregnancies with inherited arrhythmia syndromes (IASs). However, specific Doppler techniques show meanwhile a better understanding of fetal electrophysiology. The advent of fetal magnetocardiography (FMCG) now allows the detection of fetal Torsades de Pointes (TdP) ventricular tachycardia and other LQT-associated arrhythmias (QTc prolongation, functional second AV block, T-wave alternans, sinus bradycardia, late-coupled ventricular ectopy and monomorphic VT) in susceptible fetuses during the second and third trimester. These types of arrhythmias can be due to either de novo or familial Long QT Syndrome (LQTS), Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT), or other IAS. It is imperative that the multiple specialists involved in the antenatal, peripartum, and neonatal care of these women and their fetuses/infants have the optimal knowledge, training and equipment in order to care for these highly specialized pregnancies and deliveries. In this review, we outline the steps to recognize symptomatic LQTS in either the mother, fetus or both, along with suggestions for evaluation and management of the pregnancy, delivery, or post-partum period impacted by LQTS.
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spelling doaj.art-a18d842b57de4b12840de86059687c4d2023-11-18T01:51:57ZengMDPI AGJournal of Clinical Medicine2077-03832023-05-011210337910.3390/jcm12103379Preventing and Treating Torsades de Pointes in the Mother, Fetus and Newborn in the Highest Risk Pregnancies with Inherited Arrhythmia SyndromesAnnette Wacker-Gussmann0Gretchen K. Eckstein1Janette F. Strasburger2Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich, 80636 Munich, GermanyDivision of Cardiology, Departments of Pediatrics and Biomedical Engineering, Children’s Wisconsin, Herma Heart Institute, Medical College of Wisconsin, Milwaukee, WI 53226, USADivision of Cardiology, Departments of Pediatrics and Biomedical Engineering, Children’s Wisconsin, Herma Heart Institute, Medical College of Wisconsin, Milwaukee, WI 53226, USAThe number of women of childbearing age who have been diagnosed in childhood with ion channelopathy and effectively treated using beta blockers, cardiac sympathectomy, and life-saving cardiac pacemakers/defibrillators is increasing. Since many of these diseases are inherited as autosomal dominant, offspring have about a 50% risk of having the disease, though many will be only mildly impacted during fetal life. However, highly complex delivery room preparation is increasingly needed in pregnancies with inherited arrhythmia syndromes (IASs). However, specific Doppler techniques show meanwhile a better understanding of fetal electrophysiology. The advent of fetal magnetocardiography (FMCG) now allows the detection of fetal Torsades de Pointes (TdP) ventricular tachycardia and other LQT-associated arrhythmias (QTc prolongation, functional second AV block, T-wave alternans, sinus bradycardia, late-coupled ventricular ectopy and monomorphic VT) in susceptible fetuses during the second and third trimester. These types of arrhythmias can be due to either de novo or familial Long QT Syndrome (LQTS), Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT), or other IAS. It is imperative that the multiple specialists involved in the antenatal, peripartum, and neonatal care of these women and their fetuses/infants have the optimal knowledge, training and equipment in order to care for these highly specialized pregnancies and deliveries. In this review, we outline the steps to recognize symptomatic LQTS in either the mother, fetus or both, along with suggestions for evaluation and management of the pregnancy, delivery, or post-partum period impacted by LQTS.https://www.mdpi.com/2077-0383/12/10/3379fetal arrhythmiaLong QTdelivery roomTorsades de pointes
spellingShingle Annette Wacker-Gussmann
Gretchen K. Eckstein
Janette F. Strasburger
Preventing and Treating Torsades de Pointes in the Mother, Fetus and Newborn in the Highest Risk Pregnancies with Inherited Arrhythmia Syndromes
Journal of Clinical Medicine
fetal arrhythmia
Long QT
delivery room
Torsades de pointes
title Preventing and Treating Torsades de Pointes in the Mother, Fetus and Newborn in the Highest Risk Pregnancies with Inherited Arrhythmia Syndromes
title_full Preventing and Treating Torsades de Pointes in the Mother, Fetus and Newborn in the Highest Risk Pregnancies with Inherited Arrhythmia Syndromes
title_fullStr Preventing and Treating Torsades de Pointes in the Mother, Fetus and Newborn in the Highest Risk Pregnancies with Inherited Arrhythmia Syndromes
title_full_unstemmed Preventing and Treating Torsades de Pointes in the Mother, Fetus and Newborn in the Highest Risk Pregnancies with Inherited Arrhythmia Syndromes
title_short Preventing and Treating Torsades de Pointes in the Mother, Fetus and Newborn in the Highest Risk Pregnancies with Inherited Arrhythmia Syndromes
title_sort preventing and treating torsades de pointes in the mother fetus and newborn in the highest risk pregnancies with inherited arrhythmia syndromes
topic fetal arrhythmia
Long QT
delivery room
Torsades de pointes
url https://www.mdpi.com/2077-0383/12/10/3379
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