Journal ArticleDOI
The long Q-T syndrome.
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TLDR
The possiblity for the correctly diagnosed and treated patients to escape an otherwise impending death calls urgently for diffusion of the knowledge about the long Q-T syndrome.About:
This article is published in American Heart Journal.The article was published on 1975-03-01. It has received 830 citations till now. The article focuses on the topics: Stellate ganglion & Romano–Ward syndrome.read more
Citations
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Journal ArticleDOI
A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome
Mark E. Curran,Igor Splawski,Katherine W. Timothy,Vincent Gm,Eric D. Green,Keating Mt,Keating Mt +6 more
TL;DR: In this article, the authors investigated patients with long QT syndrome (LQT), an inherited disorder causing sudden death from a ventricular tachyarrythmia, torsade de pointes.
Journal ArticleDOI
Positional cloning of a novel potassium channel gene: KVLQT1 mutations cause cardiac arrhythmias.
Qing Wang,Mark E. Curran,Igor Splawski,Timothy C. Burn,J. M. Millholland,T. J. VanRaay,Jiaxiang Shen,Katherine W. Timothy,Vincent Gm,Vincent Gm,T. De Jager,Peter J. Schwartz,J.A. Towbin,Arthur J. Moss,Donald L. Atkinson,Gregory M. Landes,Timothy D. Connors,M T Keating +17 more
TL;DR: In this article, positional cloning was used to establish KVLQT1 as the chromosome 11-linked LQT 1 gene responsible for the most common inherited cardiac arrhythmia.
Journal ArticleDOI
Genotype-Phenotype Correlation in the Long-QT Syndrome Gene-Specific Triggers for Life-Threatening Arrhythmias
Peter J. Schwartz,Silvia G. Priori,Carla Spazzolini,Arthur J. Moss,G. Michael Vincent,Carlo Napolitano,Isabelle Denjoy,Pascale Guicheney,Günter Breithardt,Mark T. Keating,Jeffrey A. Towbin,Alan H. Beggs,Paul A. Brink,Arthur A.M. Wilde,Lauri Toivonen,Wojciech Zareba,Jennifer L. Robinson,Katherine W. Timothy,Valerie A. Corfield,Duangrurdee Wattanasirichaigoon,Clive Corbett,Wilhelm Haverkamp,Eric Schulze-Bahr,Michael H. Lehmann,Ketty Schwartz,Philippe Coumel,Raffaella Bloise +26 more
TL;DR: Life-threatening arrhythmias in LQTS patients tend to occur under specific circumstances in a gene-specific manner, allowing new insights into the mechanisms that relate the electrophysiological consequences of mutations on specific genes to clinical manifestations and offer the possibility of complementing traditional therapy with gene- specific approaches.
Journal ArticleDOI
SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome
Qing Kenneth Wang,Jiaxiang Shen,Jiaxiang Shen,Igor Splawski,Donald L. Atkinson,Donald L. Atkinson,Zhizhong Li,Jennifer L. Robinson,Arthur J. Moss,Jeffrey A. Towbin,Mark T. Keating +10 more
TL;DR: Genetic linkage between LQT3 and polymorphisms within SCN5A, the cardiac sodium channel gene, and single strand conformation polymorphism and DNA sequence analyses suggest that mutations in SCN 5A cause chromosome 3-linked LQt and indicate a likely cellular mechanism for this disorder.
Journal ArticleDOI
Spectrum of Mutations in Long-QT Syndrome Genes KVLQT1, HERG, SCN5A, KCNE1, and KCNE2
Igor Splawski,Jiaxiang Shen,Katherine W. Timothy,Michael H. Lehmann,Silvia G. Priori,Jennifer L. Robinson,Arthur J. Moss,Peter J. Schwartz,Jeffrey A. Towbin,G. Michael Vincent,Mark T. Keating +10 more
TL;DR: KVLQT1 and HERG accounted for 87% of identified mutations, and SCN5A, KCNE1, and KCNE2 accounted for the other 13%, and Missense mutations were most common, followed by frameshift mutations, in-frame deletions, and nonsense and splice-site mutations.
References
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Journal ArticleDOI
Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and sudden death
Anton Jervell,Fred Lange-Nielsen +1 more
TL;DR: Three deaf-mute children all suffered attacks of fainting, probably Adams-Stokes seizures caused by standstill of the heart, and 3 of the children died in such attacks at the ages of 4, 5, and 9 years, respectively.
Journal ArticleDOI
Functional distribution of right and left stellate innervation to the ventricles
TL;DR: The electrocardiographic form changes observed following unilateral alteration of sympathetic tone paralleled those electrocardsiographic abnormalities seen in patients with lesions of the central nervous system, suggesting a possible functional explanation for these clinical findings.
Journal ArticleDOI
Electrical alternation of the T-wave: Clinical and experimental evidence of its relationship with the sympathetic nervous system and with the long Q-T syndrome
TL;DR: These experiments provide further support on the relationships between the "long Q-T" syndrome and the sympathetic nervous system and indicate that alternation of the T-wave may depend on abrupt increases in the sympathetic discharge.
Journal ArticleDOI
Unilateral Cervicothoracic Sympathetic Ganglionectomy for the Treatment of Long QT Interval Syndrome
TL;DR: The syndrome of congenital deafness, syncopal episodes, long QT interval on the electrocardiogram and sudden death, and ventricular dysrhythmias have been well described.
Related Papers (5)
Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and sudden death
Anton Jervell,Fred Lange-Nielsen +1 more
Genotype-Phenotype Correlation in the Long-QT Syndrome Gene-Specific Triggers for Life-Threatening Arrhythmias
Peter J. Schwartz,Silvia G. Priori,Carla Spazzolini,Arthur J. Moss,G. Michael Vincent,Carlo Napolitano,Isabelle Denjoy,Pascale Guicheney,Günter Breithardt,Mark T. Keating,Jeffrey A. Towbin,Alan H. Beggs,Paul A. Brink,Arthur A.M. Wilde,Lauri Toivonen,Wojciech Zareba,Jennifer L. Robinson,Katherine W. Timothy,Valerie A. Corfield,Duangrurdee Wattanasirichaigoon,Clive Corbett,Wilhelm Haverkamp,Eric Schulze-Bahr,Michael H. Lehmann,Ketty Schwartz,Philippe Coumel,Raffaella Bloise +26 more