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In utero diagnosis of long QT syndrome by magnetocardiography.

Circulation (2013-11-13)
Bettina F Cuneo, Janette F Strasburger, Suhong Yu, Hitoshi Horigome, Takayoshi Hosono, Akihiko Kandori, Ronald T Wakai
ABSTRACT

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. 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. 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.

MATERIALS
Product Number
Brand
Product Description

Supelco
Lidocaine, Pharmaceutical Secondary Standard; Certified Reference Material
Sigma-Aldrich
Lidocaine hydrochloride monohydrate, solid
Sigma-Aldrich
Lidocaine, analytical standard
Sigma-Aldrich
Lidocaine, powder
Supelco
Lidocaine solution, 1.0 mg/mL in methanol, ampule of 1 mL, certified reference material, Cerilliant®
Supelco
Lidocaine hydrochloride, Pharmaceutical Secondary Standard; Certified Reference Material
Lidocaine, European Pharmacopoeia (EP) Reference Standard
Lidocaine hydrochloride, European Pharmacopoeia (EP) Reference Standard