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, Groningen, the Netherlands
University Hospital, Department of Cardiology, Groningen, the Netherlands
Clinical Genetics, Groningen, the Netherlands
Experimental and Molecular Cardiology Group, Academic Medical Center, Amsterdam, the Netherlands
|| 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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