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Long QT syndrome in neonates

Conduction disorders associated with HERG mutations and sinus bradycardia with KCNQ1 mutations

Jean-Marc Lupoglazoff, MD, PhD^*,*, Isabelle Denjoy, MD^*, Elisabeth Villain, MD, Véronique Fressart, MD^||, Françoise Simon^||, André Bozio, MD^¶, Myriam Berthet, Nawal Benammar, Bernard Hainque, PhD^|| and Pascale Guicheney, PhD

^* Pediatric Cardiology, Robert Debré Hospital (AP/HP), Paris, France
INSERM U582, Institut de Myologie Pitié-Salpêtrière Hospital (AP/HP), Paris, France
Cardiology, Lariboisière Hospital (AP/HP), Paris, France
Pediatric Cardiology, Necker-Enfants-Malades (AP/HP), Paris, France
^|| Biochemistry and Molecular Biology, Pitié-Salpêtrière Hospital (AP/HP), Paris, France
^¶ Pediatric Cardiology, Lyon, France

View larger version (103K): [in a new window]   Figure 1 Electrocardiographic (A) and Holter (B) recordings of a one-day-old neonate with bradycardia attributable to 2:1 atrioventricular block (AVB). The Holter recording showed 1:1 sinus rhythm periods, thus demonstrating that the AVB was intermittent. Furthermore, the notched T-wave morphology pointed to a mutation in HERG, which was identified.