Developmental Aspects of Long QT Syndrome Type 3 and Brugada Syndrome on the Basis of a Single SCN5A Mutation in Childhood
Gertie C.M. Beaufort-Krol, MD, PhD*,
Maarten P. van den Berg, MD, PhD ,
Arthur A.M. Wilde, MD, PhD ,
J. Peter van Tintelen, MD ,
Jan Willem Viersma, MD, PhD,
Connie R. Bezzina, PhD,|| and
Margreet Th.E. Bink-Boelkens, MD, PhD*,*
* Beatrix Children's Hospital, Department of Pediatric Cardiology
University Hospital, Department of Cardiology
Clinical Genetics, Groningen, the Netherlands
Experimental and Molecular Cardiology Group
|| Department of Clinical Genetics, Academic Medical Center, Amsterdam, the Netherlands
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Figure 1  QT = QT interval after the longest pause in a Holter recording minus QT of the preceding QRS interval (B – A).
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Figure 2 (A) QT interval for carriers (solid bars) and non-carriers (open bars) in different age groups. (B) ST-segment elevation. Open bars for 0 to 1 year and solid bars for 1 to 3 years are not visible, because the values are 0.0 ± 0.0 mm. Mean ± SD. *p < 0.05.
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Figure 3 Electrocardiogram of a 12-year-old carrier showing ST-segment elevation in the right precordial leads.
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Figure 4 Relation between heart rate and QT interval for carriers (solid circles) and non-carriers (open circles) in different age groups. Mean ± SD. *p < 0.05. bpm = beats per minute.
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