Document Detail


In utero diagnosis of long QT syndrome by magnetocardiography.
MedLine Citation:
PMID:  24218437     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: The electrophysiology of long QT syndrome (LQTS) in utero is virtually unstudied. Our goal here was to evaluate the efficacy of fetal magnetocardiography (fMCG) for diagnosis and prognosis of fetuses at risk of LQTS.
METHODS AND RESULTS: We reviewed the pre/postnatal medical records of 30 fetuses referred for fMCG because of a family history of LQTS (n=17); neonatal/childhood sudden cardiac death (n=3), or presentation of prenatal LQTS rhythms (n=12): 2° atrioventricular block, ventricular tachycardia, heart rate < 3(rd) percentile. We evaluated heart rate and reactivity, cardiac time intervals, T-wave characteristics, and initiation/termination of Torsade de Pointes, and compared these with neonatal ECG findings. After birth, subjects were tested for LQTS mutations. Based on accepted clinical criteria, 21 subjects (70%; 9 KCNQ1, 5 KCNH2, 2 SCN5A, 2 other, 3 untested) had LQTS. Using a threshold of corrected QT= 490 ms, fMCG accurately identified LQTS fetuses with 89% (24/27) sensitivity and 89% (8/9) specificity in 36 sessions. Four fetuses (2 KCNH2 and 2 SCN5A), all with corrected QT ≥ 620 ms, had frequent episodes of Torsade de Pointes, which were present 22-79% of the time. Although some episodes initiated with a long-short sequence, most initiations showed QRS aberrancy and a notable lack of pause dependency. T-wave alternans was strongly associated with severe LQTS phenotype.
CONCLUSIONS: Corrected QT prolongation (≥490 ms) assessed by fMCG accurately identified LQTS in utero; extreme corrected QT prolongation (≥620 ms) predicted Torsade de Pointes. FMCG can play a critical role in the diagnosis and management of fetuses at risk of LQTS.
Authors:
Bettina F Cuneo; Janette F Strasburger; Suhong Yu; Hitoshi Horigome; Takayoshi Hosono; Akihiko Kandori; Ronald T Wakai
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural    
Journal Detail:
Title:  Circulation     Volume:  128     ISSN:  1524-4539     ISO Abbreviation:  Circulation     Publication Date:  2013 Nov 
Date Detail:
Created Date:  2013-11-12     Completed Date:  2014-01-07     Revised Date:  2014-11-14    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2183-91     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Anti-Arrhythmia Agents / therapeutic use
Cohort Studies
Electrocardiography
Female
Fetal Diseases / diagnosis*,  drug therapy,  genetics
Genetic Association Studies
Heart Rate, Fetal
Humans
Infant, Newborn
Lidocaine / therapeutic use
Long QT Syndrome / diagnosis*,  drug therapy,  genetics
Magnetocardiography / methods*
Male
Pregnancy
Prenatal Diagnosis / methods*
Refractory Period, Electrophysiological
Retrospective Studies
Sensitivity and Specificity
Torsades de Pointes / diagnosis*,  drug therapy,  genetics
Grant Support
ID/Acronym/Agency:
R01 HL063174/HL/NHLBI NIH HHS; R01HL63174/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Anti-Arrhythmia Agents; 98PI200987/Lidocaine
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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