Document Detail

Ventricular dysfunction in a family with long QT syndrome type 3.
MedLine Citation:
PMID:  23612729     Owner:  NLM     Status:  Publisher    
AIMS: Long QT syndrome (LQTS) type 3 is characterized by prolonged ventricular repolarization due to persistent sodium inward current secondary to a mutation in SCN5a, the gene encoding for the α-subunit of the sodium channel. We speculated that by disrupting calcium homeostasis the persistent inward sodium current in patients with LQTS type 3 might cause derangement of diastolic function. We aimed to identify functional myocardial alterations in a family with a sodium channelopathy with a phenotype of both LQTS type 3 and Brugada syndrome.METHODS AND RESULTS: The study group comprised 12 SCN5a mutation carriers (SCN5a-1795insD), 9 females and 3 males, mean age 35.7 ± 7.3 years, and 12 healthy controls. In addition to conventional echocardiographic measurements, two-dimensional speckle tracking was performed to assess tissue properties. Mean e' was lower in the patients compared with the controls (5.6 ± 0.75 vs. 6.7 ± 0.98 cm/s, P = 0.006). Onset QRS to maximum s' was longer in the patients than in the controls (0.20 ± 0.04 vs. 0.15 ± 0.05 s, P = 0.007), and the number of segments with post-systolic shortening was higher (6.58 ± 2.54 vs. 1.83 ± 1.64, P < 0.001).CONCLUSION: Patients in this family with LQTS type 3 showed post-systolic shortening, as well as both left and right ventricular diastolic dysfunction. The underlying mechanism remains to be elucidated but the persistent sodium inward current leading to calcium overload might play a role, in particular regarding diastolic dysfunction.
Yoran M Hummel; Arthur A M Wilde; Adriaan A Voors; Silvia Bugatti; Hans L Hillege; Maarten P van den Berg
Related Documents :
23851059 - Clinical outcomes with synchronized left ventricular pacing: analysis of the adaptive c...
20716839 - Effect of tai chi training on baroreflex sensitivity and heart rate variability in pati...
19291559 - Can paramedics read st-segment elevation myocardial infarction on prehospital 12-lead e...
22649679 - Modeling myocardial infarction in mice: methodology, monitoring, pathomorphology.
24803489 - Nanoparticle oxygen delivery to the ischemic heart.
23370639 - Right- and left-ventricular strain evaluation in repaired pediatric tetralogy of fallot...
3518369 - Quantitative analysis of intracranial circulation using rapid-sequence dsa.
24224999 - 18f-fluoride positron emission tomography for identification of ruptured and high-risk ...
8085549 - Comparable safety of blood collection in "high-risk" autologous donors versus non-high-...
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-4-23
Journal Detail:
Title:  Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology     Volume:  -     ISSN:  1532-2092     ISO Abbreviation:  Europace     Publication Date:  2013 Apr 
Date Detail:
Created Date:  2013-4-24     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883649     Medline TA:  Europace     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Department of Cardiology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, PO Box 30001, 9700 RB, Groningen, The Netherlands.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Classification of electrophysiological types of atrioventricular nodal re-entrant tachycardia: a rea...
Next Document:  MediGuide in supraventricular tachycardia: initial experience from a multicentre registry.