The diagnosis and management of long QT syndrome based on fetal echocardiography

Blais, Benjamin A.; Satou, Gary; Sklansky, Mark; Madnawat, Himani; Moore, Jeremy P.

doi:10.1016/j.hrcr.2017.04.007

Cited by 5 publications

(5 citation statements)

References 10 publications

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“…LQTS type 1 fetuses typically do not manifest the severe QTc lengthening (>600 msec) associated with high‐risk rhythm abnormalities such as second‐degree AV block, T‐wave alternans, or torsade de pointes 5 . Prior literature shows these findings are typically seen in patients with SCN5A and KCNH2 mutations 1,5,8 . Fetuses with a KCNQ1 mutation like our patient most commonly present with sinus bradycardia as the only rhythm abnormality, so his findings of 2:1 AV block and T‐wave morphology variability were unexpected 5,8 .…”

Section: Discussionmentioning

confidence: 56%

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

Desai

Wakai

Tsao

et al. 2020

Pacing Clinical Electrophis

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A pregnant woman with KCNQ1 variant long QT syndrome (LQTS) underwent fetal magnetocardiography (fMCG) after atrioventricular (AV) block was noted during fetal echocardiogramatypical 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. Beta blocker therapy was initiated after birth, and postnatal evaluation confirmed genotype and phenotype positive LQTS in the infant. Our experience suggests diagnosis and evaluation of fetal LQTS can alter antenatal management to reduce risk of poor fetal and postnatal outcomes. K E Y W O R D S fetal bradycardia, fetal echocardiography, fetal magnetocardiography, long QT syndrome 1

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Section: Discussionmentioning

confidence: 56%

“…Long QT syndrome (LQTS) affects up to 1 in 2500 individuals and can result in sudden cardiac death 1 . Timely recognition of the disease is crucial.…”

Section: Introductionmentioning

confidence: 99%

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

Desai

Wakai

Tsao

et al. 2020

Pacing Clinical Electrophis

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“…In the majority of cases, fetal sinus bradycardia, defined as fetal heart rate <110 bpm (or <3 rd percentile for gestational age), is often the only fetal arrhythmia detected in utero on routine prenatal ultrasound and should prompt referral to pediatric cardiology for close monitoring with fetal echocardiography. [ 6 7 ] Specific markers noted on fetal echocardiogram evaluation can point clinicians toward diagnosing LQTS. In otherwise healthy fetuses with suspected LQTS, the cardiac anatomy is often normal; however, mechanical conduction can become aberrant, and this can be noted on echocardiography.…”

Section: Discussionmentioning

confidence: 99%

“…M-mode detects transient episodes of ventricular tachycardia with variable cycle lengths, normal atrial rate and intermittent ventriculoatrial dissociation. [ 6 ] A rapid early contraction phase with slow contraction during the late thickening phase has been previously described in patients with LQTS. [ 8 ] In addition, tissue Doppler is a well-established and reliable method for cardiac function evaluation and can contribute key evidence to diagnosing congenital LQTS as it allows analyzing cardiac wall motion and myocardial velocities.…”

Section: Discussionmentioning

confidence: 99%

Congenital long QT syndrome

Santi

Restrepo

2022

Annals of Pediatric Cardiology

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The diagnosis of long QT syndrome (LQTS) in utero presents many challenges for clinicians, and there is high risk for intrauterine fetal demise as life-threatening arrhythmias develop secondary to QT prolongation. We describe a challenging case of a fetus presenting with sinus bradycardia and second-degree atrioventricular block with episodes of ventricular tachycardia. A prenatal diagnosis of LQTS was suspected given the fetal echocardiographic findings of a short ventricular relaxation time, due to extremely prolonged refractory period. The patient was delivered emergently due to Torsade's with hydrops, with ongoing arrhythmia despite medical management requiring implantation of pacemaker and sympathectomy. Early recognition of LQTS is important to optimize fetal survival with prompt medical management.

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“…This way rhythm abnormalities can be detected and a structurally healthy heart can be assured. Real-time fetal heart rhythm monitoring is possible, while cardiac repolarization and measurement of the QT interval is not performable [13].…”

Section: Diagnosismentioning

confidence: 99%

Fetal Long QT Syndrome – Challenges in Perinatal Management: A Review and Case Report. Induction of Labor and Vaginal Birth Under Continuous Magnesium Therapy

Wegner,

Steinhard,

Frank

et al. 2024

Z Geburtshilfe Neonatol

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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.

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The diagnosis and management of long QT syndrome based on fetal echocardiography

Cited by 5 publications

References 10 publications

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

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

Congenital long QT syndrome

Fetal Long QT Syndrome – Challenges in Perinatal Management: A Review and Case Report. Induction of Labor and Vaginal Birth Under Continuous Magnesium Therapy

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