Literature DB >> 22263042

Congenital long QT syndrome: A case report.

Fahad Aziz1, Sudheer Penupolu, Sujatha Doddi, Babajide Togonu-Bickersteth, Abdul Ameen.   

Abstract

The congenital long QT syndrome (LQTS) is characterized by abnormally prolonged ventricular repolarization due to inherited defects in cardiac sodium and potassium channels, which predispose the patients to syncope, seizure like activity, ventricular arrhythmias, and sudden cardiac death. Early diagnosis and preventive treatment are instrumental in preventing sudden cardiac deaths in patients with the congenital LQTS. The diagnostic criteria for congenital LQTS are based on certain electrocardiographic findings, clinical findings and findings of epinephrine stress test. Recently genotype specific electrocardiographic pattern in the congenital LQTS has also been described. Recent studies suggest feasibility of genotype specific treatment of LQTS and, in near future, mutation specific treatment will probably become a novel approach to this potentially fatal syndrome. We describe one case that fulfilled the electrocardiographic, historical diagnostic criteria and epinephrine stress test suggestive of LQT syndrome.

Entities:  

Keywords:  cardiac sodium; congenital long QT syndrome; potassium channels

Year:  2010        PMID: 22263042      PMCID: PMC3256463          DOI: 10.3978/j.issn.2072-1439.2010.02.03.12

Source DB:  PubMed          Journal:  J Thorac Dis        ISSN: 2072-1439            Impact factor:   2.895


  13 in total

1.  Epinephrine-induced QT interval prolongation: a gene-specific paradoxical response in congenital long QT syndrome.

Authors:  Michael J Ackerman; Anant Khositseth; David J Tester; Joseph B Hejlik; Win-Kuang Shen; Co-burn J Porter
Journal:  Mayo Clin Proc       Date:  2002-05       Impact factor: 7.616

2.  Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias.

Authors:  P J Schwartz; S G Priori; C Spazzolini; A J Moss; G M Vincent; C Napolitano; I Denjoy; P Guicheney; G Breithardt; M T Keating; J A Towbin; A H Beggs; P Brink; A A Wilde; L Toivonen; W Zareba; J L Robinson; K W Timothy; V Corfield; D Wattanasirichaigoon; C Corbett; W Haverkamp; E Schulze-Bahr; M H Lehmann; K Schwartz; P Coumel; R Bloise
Journal:  Circulation       Date:  2001-01-02       Impact factor: 29.690

3.  Differential effects of beta-adrenergic agonists and antagonists in LQT1, LQT2 and LQT3 models of the long QT syndrome.

Authors:  W Shimizu; C Antzelevitch
Journal:  J Am Coll Cardiol       Date:  2000-03-01       Impact factor: 24.094

4.  KVLQT1 C-terminal missense mutation causes a forme fruste long-QT syndrome.

Authors:  C Donger; I Denjoy; M Berthet; N Neyroud; C Cruaud; M Bennaceur; G Chivoret; K Schwartz; P Coumel; P Guicheney
Journal:  Circulation       Date:  1997-11-04       Impact factor: 29.690

Review 5.  Long QT syndromes and torsade de pointes.

Authors:  S Viskin
Journal:  Lancet       Date:  1999-11-06       Impact factor: 79.321

Review 6.  The long QT syndromes: genetic basis and clinical implications.

Authors:  C E Chiang; D M Roden
Journal:  J Am Coll Cardiol       Date:  2000-07       Impact factor: 24.094

7.  Increased risk of arrhythmic events in long-QT syndrome with mutations in the pore region of the human ether-a-go-go-related gene potassium channel.

Authors:  Arthur J Moss; Wojciech Zareba; Elizabeth S Kaufman; Eric Gartman; Derick R Peterson; Jesaia Benhorin; Jeffrey A Towbin; Mark T Keating; Silvia G Priori; Peter J Schwartz; G Michael Vincent; Jennifer L Robinson; Mark L Andrews; Changyong Feng; W Jackson Hall; Aharon Medina; Li Zhang; Zhiqing Wang
Journal:  Circulation       Date:  2002-02-19       Impact factor: 29.690

8.  Afterdepolarizations as a mechanism for the long QT syndrome: electrophysiologic studies of a case.

Authors:  E Schechter; C C Freeman; R Lazzara
Journal:  J Am Coll Cardiol       Date:  1984-06       Impact factor: 24.094

9.  Low penetrance in the long-QT syndrome: clinical impact.

Authors:  S G Priori; C Napolitano; P J Schwartz
Journal:  Circulation       Date:  1999-02-02       Impact factor: 29.690

10.  Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate. Implications for gene-specific therapy.

Authors:  P J Schwartz; S G Priori; E H Locati; C Napolitano; F Cantù; J A Towbin; M T Keating; H Hammoude; A M Brown; L S Chen; T J Colatsky
Journal:  Circulation       Date:  1995-12-15       Impact factor: 29.690

View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.