Document Detail

Inducibility of life-threatening ventricular arrhythmias is related to maximum left ventricular thickness and clinical markers of sudden cardiac death in patients with hypertrophic cardiomyopathy attributable to the Asp175Asn mutation in the alpha-tropomyosin gene.
MedLine Citation:
PMID:  14734051     Owner:  NLM     Status:  MEDLINE    
We investigated inducibility of life-threatening arrhythmias with programmed ventricular stimulation (PVS) in relation to clinical markers of sudden cardiac death (SCD) in subjects with hypertrophic cardiomyopathy (HCM) attributable to the Asp175Asn mutation in the alpha-tropomyosin gene (TPM1-Asp175Asn). PVS was performed with up to three extrastimuli and distribution of markers of SCD was evaluated in 21 adult subjects with the TPM1-Asp175Asn. Sustained polymorphic ventricular tachycardia (VT) or ventricular fibrillation (VF) was induced in seven of 21 subjects (33%). Inducible subjects had more severe left ventricular hypertrophy (LVH) and an increased number of markers of SCD (family history of SCD, syncope or presyncope, fall in systolic blood pressure (BP) during exercise, documented non-sustained VT (NSVT), and marked LVH) compared to non-inducible subjects (IVS 2.4 +/- 0.3 cm vs. 1.6 +/- 0.5 cm, P < 0.001; and two to three vs. one to two markers of SCD, P = 0.007, respectively). In conclusion, in HCM attributable to the Asp175Asn mutation in the alpha-tropomyosin gene, life-threatening arrhythmias were induced in one third of the patients. Inducibility was associated with the maximum left ventricular (LV) thickness and the number of markers of SCD, suggesting that in HCM patients with an identical causative mutation, susceptibility to ventricular arrhythmias is related to the cardiomyopathic phenotype.
A Hedman; J Hartikainen; E Vanninen; T Laitinen; P Jääskeläinen; M Laakso; K Peuhkurinen; J Kuusisto
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of molecular and cellular cardiology     Volume:  36     ISSN:  0022-2828     ISO Abbreviation:  J. Mol. Cell. Cardiol.     Publication Date:  2004 Jan 
Date Detail:
Created Date:  2004-01-21     Completed Date:  2004-11-16     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0262322     Medline TA:  J Mol Cell Cardiol     Country:  England    
Other Details:
Languages:  eng     Pagination:  91-9     Citation Subset:  IM    
Department of Medicine, Kuopio University Hospital, 70211 Kuopio, Finland.
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MeSH Terms
Arrhythmias, Cardiac / complications*,  physiopathology*
Asparagine / genetics,  metabolism
Aspartic Acid / genetics,  metabolism
Biological Markers / analysis
Cardiomyopathy, Hypertrophic / complications*,  genetics*,  physiopathology
Death, Sudden, Cardiac / pathology*
Exercise Test
Genetic Predisposition to Disease / genetics
Heart Ventricles / pathology*,  physiopathology
Mutation, Missense / genetics
Tropomyosin / genetics*
Reg. No./Substance:
0/Biological Markers; 0/Tropomyosin; 56-84-8/Aspartic Acid; 7006-34-0/Asparagine
Erratum In:
J Mol Cell Cardiol. 2004 Apr;36(4):607-8

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