Diagnosis and Management of Fetal Arrhythmias in the Current Era.

Diagnosis and management of fetal arrhythmias have changed over the past 40-50 years since propranolol was first used to treat fetal tachycardia in 1975 and when first attempts were made at in utero pacing for complete heart block in 1986. Ongoing clinical trials, including the FAST therapy trial for fetal tachycardia and the STOP-BLOQ trial for anti-Ro-mediated fetal heart block, are working to improve diagnosis and management of fetal arrhythmias for both mother and fetus. We are also learning more about how "silent arrhythmias", like long QT syndrome and other inherited channelopathies, may be identified by recognizing "subtle" abnormalities in fetal heart rate, and while echocardiography yet remains the primary tool for diagnosing fetal arrhythmias, research efforts continue to advance the clinical envelope for fetal electrocardiography and fetal magnetocardiography.

Low Baseline Fetal Heart Rate Leads to Diagnosis of Long QT Syndrome Type 1.

A low baseline fetal heart rate at 20 weeks' gestation was detected in a fetus without cardiac structural anomalies. Fetal echocardiography and magnetocardiography were used to diagnose congenital long QT syndrome. It was confirmed in the neonate, and the same pathogenic variant in was subsequently identified in the mother.

Fetal Congenital Complete Heart Block: A Rare Case with an Extremely Low Ventricular Rate and Review of Current Management Strategies.

Congenital complete heart block (CCHB) is associated with high intrauterine and post-natal mortality. Prenatal detection and management, as well as appropriate delivery planning, may improve the outcomes in CCHB. We describe a rare case of CCHB that initially presented with fetal ascites and high-grade second-degree heart block noted on fetal echocardiography.

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, 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).

Ferrite Shield to Enhance the Performance of Optically Pumped Magnetometers for Fetal Magnetocardiography.

Fetal magnetocardiography (fMCG) has proven to be an important tool for the prenatal monitoring of electrical cardiac activity; however, the high cost of superconducting quantum instrumentation (SQUID) poses a limitation for the dissemination of fMCG as a routine clinical technique. Recently, optically pumped magnetometers (OPMs) operating within person-sized, cylindrical shields have made fMCG more practical, but environmental magnetic interference entering through the shield opening substantially degrades the quality of fMCG signals. The goal of this study was to further attenuate these interferences by placing the OPM array within a small ferrite shield.

Fetal Arrhythmia Diagnosis and Pharmacologic Management.

One of the most successful achievements of fetal intervention is the pharmacologic management of fetal arrhythmias. This management usually takes place during the second or third trimester. While most arrhythmias in the fetus are benign, both tachy- and bradyarrhythmias can lead to fetal hydrops or cardiac dysfunction and require treatment under certain conditions.

Contribution of Fetal Magnetocardiography to Diagnosis, Risk Assessment, and Treatment of Fetal Arrhythmia.

Background Fetal echocardiography has been the mainstay of fetal arrhythmia diagnosis; however, fetal magnetocardiography (fMCG) has recently become clinically available. We sought to determine to what extent fMCG contributed to the precision and accuracy of fetal arrhythmia diagnosis and risk assessment, and in turn, how this altered pregnancy management. Methods and Results We reviewed fMCG tracings and medical records of 215 pregnancies referred to the Biomagnetism Laboratory, UW-Madison, over the last 10 years, because of fetal arrhythmia or risk of arrhythmia.

Fetal Unguarded Mitral Valve Orifice, Aortic Atresia, and Severe Left Heart Enlargement.

Unguarded mitral valve orifice is a rare disease with only 7 described cases in the literature. We describe the first known case of unguarded mitral valve orifice with normal segmental cardiac anatomy, severe left ventricular dilatation and dysfunction, aortic atresia, and atrial flutter. ().

Repolarization Predictors of Fetal Long QT Syndrome.

Background: Diagnosis of fetal long QT syndrome (LQTS) using fetal magnetocardiography (fMCG) is straightforward in cases of overt QTc prolongation accompanied by LQTS rhythms; however, cases of isolated QTc prolongation can be challenging.

Objective: To characterize repolarization in normal and phenotype-positive LQTS fetuses with the goal of utilizing additional parameters of repolarization to improve the accuracy of fMCG diagnosis of LQTS.

Methods: FMCG recordings were taken from 37 phenotype-positive fetuses with confirmed LQTS and 132 normal controls.

Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome.

Objectives: This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs).

Background: LQTS presenting with 2:1 atrioventricular block or torsades de pointes in the fetus and/or neonate has been associated with risk for major CEs, but overall outcomes and predictors remain unknown.

Methods: A retrospective study involving 25 international centers evaluated the course of fetuses/newborns diagnosed with congenital LQTS and either 2:1 atrioventricular block or torsades de pointes.

Dynamics of the use of magnetocardiography in the study of the cardiac conduction system of the chick embryo.

Introduction: Human fetal magnetocardiography (fMCG) has been done for several decades to evaluate fetal arrhythmias using a superconducting quantum interference device (SQUID) magnetometer, but there is little work in embryonic/fetal animal models. This study uses an optically-pumped magnetometer (OPM) to obtain an fMCG in the chick embryo.

Methods: White Leghorn chick embryos were examined from incubation Day #10-19.

Noninvasive Fetal Electrocardiography in the Diagnosis of Long QT Syndrome: A Case Series.

Introduction: Early detection and monitoring for malignant arrhythmias is fundamental to prenatal care in long QT syndrome (LQTS). Recently, we studied the feasibility of isolating the fetal electrocardiogram (fECG) and measuring electrocardiographic intervals with a noninvasive fECG device using blind source separation with reference signal. Our aim was to evaluate the ability of fECG to diagnose LQTS.

Complex and Novel Arrhythmias Precede Stillbirth in Fetuses With De Novo Long QT Syndrome.

Background: Long QT syndrome (LQTS) is a leading cause of sudden cardiac death in early life and has been implicated in ≈10% of sudden infant deaths and unexplained stillbirths. The purpose of our study was to use fetal magnetocardiography to characterize the electrophysiology and rhythm phenotypes of fetuses with de novo and inherited LQTS variants and identify risk factors for sudden death before birth.

Methods: We reviewed the fetal magnetocardiography database from the University of Wisconsin Biomagnetism Laboratory for fetuses with confirmed LQTS.

Fetal diagnosis of KCNQ1-variant long QT syndrome using fetal echocardiography and magnetocardiography.

A pregnant woman with KCNQ1 variant long QT syndrome (LQTS) underwent fetal magnetocardiography (fMCG) after atrioventricular (AV) block was noted during fetal echocardiogram-atypical for LQTS type 1. Concern for fetal LQTS on fMCG prompted monitoring of maternal labs, change of maternal beta blocker therapy, and frequent fetal echocardiograms. Collaboration between obstetricians, neonatologists, and pediatric cardiologists ensured safe delivery.