Journal article
Severe sinus bradycardia in a patient with Rett syndrome: a new cause for a pause?
Pediatric cardiology, v 25(1)
Jan 2004
PMID: 14534758
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
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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Details
- Title
- Severe sinus bradycardia in a patient with Rett syndrome: a new cause for a pause?
- Creators
- N Madan - St. Christopher's Hospital for ChildrenM Levine - St. Christopher's Hospital for ChildrenK Pourmoghadam - St. Christopher's Hospital for ChildrenM Sokoloski - St. Christopher's Hospital for Children
- Publication Details
- Pediatric cardiology, v 25(1)
- Publisher
- Springer Nature
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pediatrics
- Web of Science ID
- WOS:000188455300011
- Scopus ID
- 2-s2.0-0942265407
- Other Identifier
- 991019167986904721
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- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Cardiac & Cardiovascular Systems
- Pediatrics