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Severe sinus bradycardia in a patient with Rett syndrome: a new cause for a pause?
Journal article   Peer reviewed

Severe sinus bradycardia in a patient with Rett syndrome: a new cause for a pause?

N Madan, M Levine, K Pourmoghadam and M Sokoloski
Pediatric cardiology, v 25(1)
Jan 2004
PMID: 14534758

Abstract

Arrhythmia, Sinus - complications Bradycardia - etiology Bradycardia - physiopathology Bradycardia - therapy Child, Preschool Electrocardiography, Ambulatory Female Humans Pacemaker, Artificial Rett Syndrome - complications
Rett syndrome is a progressive disorder seen primarily in young females. It is characterized by autonomic dysfunction affecting many organ systems. Although sudden death is common in these patients, little is known about the cardiovascular manifestations of this dysautonomia. Earlier reports focused on the role of sympathetic overactivity manifest as reduced heart rate variability and prolonged QT intervals indicating a propensity for lethal ventricular arrhythmias. Bradyarrhthmias and sinus node dysfunction, however, have not previously been reported. We describe a 2-year-old with Rett syndrome and severe sinus bradycardia requiring a pacemaker implant. This case represents the first description of a new cardiovascular manifestation of Rett syndrome and may provide an explanation for sudden and unexpected death in some of these patients.

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Collaboration types
Domestic collaboration
Web of Science research areas
Cardiac & Cardiovascular Systems
Pediatrics
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