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Congenital Short QT Syndrome: Landmarks of the Newest Arrhythmogenic Cardiac Channelopathy.
MedLine Citation:
PMID:  23677719     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Congenital short QT syndrome (SQTS) is a rare inherited channelopathy without structural heart disease. It is an autosomal dominant primary electrical disorder with a low degree of penetrance. It has a characteristic clinical-electrocardiographic-electrophysiological phenotype consisting of irregular palpitations due to the frequent episodes of paroxysmal atrial fibrillation, dizziness and/or sudden cardiac death (SCD). Affected individuals usually have a positive family history of syncope or SCD in first-or second degree young relatives (younger than 40 years of age) with autopsy negative SCD. Resting electrocardiograms (ECGs) have very short and uniform QT/QTc intervals (QTc interval ≤330 ms with the exception of the calcium-dependent variants 4 and 5), absent or minimal ST segments, interval from J point to T wave peak (Jp-Tp) measured in the precordial lead with the T wave of greatest amplitude.
Authors:
Andrés Ricardo Pérez Riera; Adail Paixão-Almeida; Raimundo Barbosa-Barros; Frank G Yanowitz; Adrian Baranchuk; Sergio Dubner; Antônio Carlos Palandri Chagas
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-5-15
Journal Detail:
Title:  Cardiology journal     Volume:  -     ISSN:  1897-5593     ISO Abbreviation:  Cardiol J     Publication Date:  2013 May 
Date Detail:
Created Date:  2013-5-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101392712     Medline TA:  Cardiol J     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
ABC's Faculty of Medicine - ABC Foundation - Cardiology Discipline- Electrovectorcardiogram Sector -Santo André - São Paulo - Brazil.. riera@uol.com.br.
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