In Utero Diagnosis of Long QT Syndrome by Magnetocardiography

Cuneo, Bettina F.; Strasburger, Janette F.; Yu, Shasha; Horigome, Hitoshi; Hosono, Takayoshi; Kandori, Akihiko; Wakai, Ronald T.

doi:10.1161/circulationaha.113.004840

Cited by 91 publications

(88 citation statements)

References 19 publications

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“…435 Management of the fetus with suspected LQTS includes close observation, postnatal evaluation, and measurement of the QTc by fMCG or fetal electrocardiography if available. 436 Fetal treatment is not recommended for bradycardia; however, torsades de pointes and ventricular tachycardia (VT) require treatment if they occur (Tables 13 and14). 435 Maternal electrolyte abnormalities, especially hypomagnesemia and hypocalcemia, should be avoided, as well as drugs and anesthetic agents that lengthen the QT interval.…”

Section: Lqts and Other Ion Channelopathiesmentioning

confidence: 99%

“…353 Unfortunately, although terbutaline may increase fetal rates and prolong pregnancy, no studies have shown survival benefit. Although there is merit to the notion, because of significant technical limitations, fetal pacing has not been shown to be successful in improving survival or prolonging gestation 436 and therefore at present is experimental and not recommended as part of usual care.…”

Section: Av Blockmentioning

confidence: 99%

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Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

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907

465

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Background— The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. Methods and Results— A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin–twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. Conclusions— Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.

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Section: Lqts and Other Ion Channelopathiesmentioning

confidence: 99%

Section: Av Blockmentioning

confidence: 99%

Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

Self Cite

907

465

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“…Multichannel MCG has been used clinically for the detection and diagnosis of fetal arrhythmias, 20 and for analysis in healthy adults as well as detection of abnormalities in various heart diseases. 7,21-23 Korhonen et al have reported the usefulness of QRS fragmentation detected quantitatively using the time-waveforms of MCG for predicting MACE in patients with previous myocardial infarction.…”

Section: Conflicts Of Interestmentioning

confidence: 99%

Utility of High-Resolution Magnetocardiography to Predict Later Cardiac Events in Nonischemic Cardiomyopathy Patients With Normal QRS Duration

Kawakami

Takaki

Hashimoto

et al. 2017

Circ J

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found as a normal variant. 4,5 Late potential in the early or middle portion of the QRS complex may be buried in the QRS and undetectable.Multichannel magnetocardiography (MCG) has been reported to have superior spatial and temporal resolution power, and allow detailed evaluation of the propagation of LV activation. 7, 8 In the present study, we hypothesized that MCG would detect left intraventricular disorganized conduction (LiDC) caused by localized LV conduction block, and thus be able to predict future cardiac events. Methods Study Population and Data AcquisitionThis study was approved by the Institutional Review Board of the National Cerebral and Cardiovascular Center, with a waiver of individual consent (M23-050, M24-050-2). We retrospectively reviewed the medical records of patients I n nonischemic dilated cardiomyopathy (NIDCM) patients, fibrosis and cellular degeneration in the left ventricular (LV) myocardium result in localized conduction block. 1 As the LV conduction delay becomes pronounced, QRS duration increases and prolonged QRS is known to be associated with poor prognosis. 2 However, even in NIDCM patients with apparently normal QRS duration, a cardiac event rate of 33% in approximately 1.5 years has been reported. 3 The prognosis of these particular patients is indeed not favorable. Therefore, novel indicators capable of predicting cardiac events sensitively even in NIDCM patients with normal QRS duration are required.Various methods that reflect left intraventricular conduction abnormality in NIDCM have been proposed. Although the usefulness of fragmented QRS (fQRS) observed on 12-lead ECG 4,5 and late potential detected on signalaveraged ECG (SAECG) 6 have been reported, their uses are limited. fQRS is defined qualitatively and can also be Utility of High-Resolution Magnetocardiography to Predict Later Cardiac Events in Nonischemic Cardiomyopathy Patients With Normal QRS DurationShoji Kawakami, MD; Hiroshi Takaki, MD, PhD; Shuji Hashimoto; Yoshitaka Kimura, MD; Takahiro Nakashima, MD; Takeshi Aiba, MD, PhD; Kengo F. Kusano, MD, PhD; Shiro Kamakura, MD, PhD; Satoshi Yasuda, MD, PhD; Masaru Sugimachi, MD, PhD Background: Nonischemic dilated cardiomyopathy (NIDCM) patients, even those with a narrow QRS, are at increased risk for major adverse cardiac events (MACE). We hypothesized that 64-channel magnetocardiography (MCG) would be useful to detect prognostic left intraventricular disorganized conduction (LiDC) by overcoming the limitations of fragmented QRS (fQRS, qualitative definitions, low specificity) and late potential (abnormality undetectable in earlier QRS).

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“…11 In addition to a low baseline heart rate, nonreactive heart rate patterns are also suggestive in LQTS fetuses and may be explained by lower-than-normal right sympathetic cardiac activity or a blunted response to a sympathetic drive, as seen postnatally. 8 LQTS with 2:1 AVB is commonly observed during the fetal and neonatal periods, 9,10 but rarely observed in childhood and adulthood. In neonates with LQT2 and LQT3, 2:1 AVB was observed in 55% and 83% of the patients, respectively (Figure 1).…”

mentioning

confidence: 99%

“…10 AVB may be caused by functional block of the ventricle, because of prolongation of the ventricular refractory period, and in the majority of patients the atrioventricular conduction returns with a significant decrease in the QTc interval during the follow-up period. 8 That may explain the rare occurrence of AVB in older children and adults with LQTS.…”

mentioning

confidence: 99%

Clinical Features of Long QT Syndrome in Children

Sumitomo

2016

Circ J

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Circulation Journal Official Journal of the Japanese Circulation Society http://www. j-circ.or.jp gested if the fetal heart rate is less than the 3rd percentile. 11 In addition to a low baseline heart rate, nonreactive heart rate patterns are also suggestive in LQTS fetuses and may be explained by lower-than-normal right sympathetic cardiac activity or a blunted response to a sympathetic drive, as seen postnatally. 8 LQTS with 2:1 AVB is commonly observed during the fetal and neonatal periods, 9,10 but rarely observed in childhood and adulthood. In neonates with LQT2 and LQT3, 2:1 AVB was observed in 55% and 83% of the patients, respectively (Figure 1). 10 AVB may be caused by functional block of the ventricle, because of prolongation of the ventricular refractory period, and in the majority of patients the atrioventricular conduction returns with a significant decrease in the QTc interval during the follow-up period. 8 That may explain the rare occurrence of AVB in older children and adults with LQTS. Prognosis of LQTS in ChildrenPrevious reports showed that males with LQTS before the age of 15 years have a significant increased risk of syncope, aborted cardiac arrest, and sudden cardiac death. 12 However, the risk of these cardiac events is inverted after the age of 14 years. 12 Comparing the genotype of LQTS carriers, LQT1 females have a significantly lower risk of cardiac events than LQT1 males ≤15 years old (hazard ratio (HR) 0.58; P=0.005), but a significantly higher risk of cardiac events than males between the ages of 16 and 40 years (HR 3.35; P=0.007) (Figure 2). 13 LQT2 and LQT3 children show no significant differences between male and female carriers. Females with LQT2 have a significantly higher risk of a first cardiac event than males between the ages of 16 and 40 years (HR 3.71; P=0.010). 13 During 0-12 years old, males with LQT1 have the highest rate of a first syncope episode (P<0.001), but within the age range of 13-20 years, LQT2 females experience the highest rate of both first and subsequent syncope events (P<0.001 and P=0.01). 14 In this issue of the Journal, Ozawa et al 15 report that the LQT2 phenotype presents with more frequent cardiac arrests or repetitive torsade de points (TdP) episodes than the LQT1 phenotype. They also demonstrate that LQT2 females have a repeat TdP episode within a short time period after a prior TdP episode, especially after puberty. As they note in their literature, the effect of estrogen, which prolongs the action potential duration (APD) through the inhibition of IKr, may contribute to the high occurrence of TdP in LQT2 females after adolescence.A high occurrence of cardiac events in LQT1 males during childhood is also reported. 13-15 Male children may be more vigorous than females, which may result in them having more ong QT syndrome (LQTS) is a genetic channelopathy with prolonged ventricular repolarization of the myocardial cells, associated with severe cardiac events such as syncope, aborted cardiac arrest, and sudden cardiac death. 1 Children with LQTS hav...

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In Utero Diagnosis of Long QT Syndrome by Magnetocardiography

Cited by 91 publications

References 19 publications

Diagnosis and Treatment of Fetal Cardiac Disease

Diagnosis and Treatment of Fetal Cardiac Disease

Utility of High-Resolution Magnetocardiography to Predict Later Cardiac Events in Nonischemic Cardiomyopathy Patients With Normal QRS Duration

Clinical Features of Long QT Syndrome in Children

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