Electrophysiological findings in Fabry cardiomyopathy: mapping the maze of risk stratification

We report a case of Anderson-Fabry disease in a young man presenting with cardiac hypertrophy and asymptomatic non-sustained ventricular tachycardia. The patient was referred for evaluation of implantable cardioverter/defibrillator therapy. Assessment of left ventricular ejection fraction is conside...

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Main Authors: Li, Jin (Author) , Warth, Arne (Author) , Schnabel, Philipp Albert (Author) , Schweizer, Patrick Alexander (Author) , Buß, Sebastian Johannes (Author) , Steen, Henning (Author) , Becker, Rüdiger (Author) , Katus, Hugo (Author) , Thomas, Dierk (Author)
Format: Article (Journal)
Language:English
Published: 2012
In: Acta cardiologica
Year: 2012, Volume: 67, Issue: 4, Pages: 481-485
ISSN:1784-973X
DOI:10.1080/AC.67.4.2170694
Online Access:Verlag, Volltext: http://dx.doi.org/10.1080/AC.67.4.2170694
Verlag, Volltext: http://poj.peeters-leuven.be/content.php?url=article&id=2170694&journal_code=AC
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Author Notes:Jin Li , Arne Warth , Philipp Schnabel , Patrick A. Schweizer , Sebastian J. Buss , Henning Steen , Rüdiger Becker , Hugo A. Katus , Dierk Thomas
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Summary:We report a case of Anderson-Fabry disease in a young man presenting with cardiac hypertrophy and asymptomatic non-sustained ventricular tachycardia. The patient was referred for evaluation of implantable cardioverter/defibrillator therapy. Assessment of left ventricular ejection fraction is considered the gold standard for identifying patients at risk of sudden cardiac death. However, this patient’s left ventricular function was preserved. Electrophysiological study did not reveal inducible arrhythmia or cardiac conduction abnormalities. Review of the literature indicates limited knowledge on the electrophysiology of Fabry cardiomyopathy and highlights the need for optimized risk stratification strategies.
Item Description:Published online: 23 May 2017
Gesehen am 29.10.2018
Physical Description:Online Resource
ISSN:1784-973X
DOI:10.1080/AC.67.4.2170694