G M Vincent

Summary

Affiliation: LDS Hospital
Country: USA

Publications

  1. ncbi request reprint The molecular genetics of the long QT syndrome: genes causing fainting and sudden death
    G M Vincent
    Department of Internal Medicine, LDS Hospital, Salt Lake City, Utah 84143, USA
    Annu Rev Med 49:263-74. 1998
  2. ncbi request reprint The role of genotyping in diagnosing cardiac channelopathies : progress to date
    G Michael Vincent
    LDS Hospital and University of Utah School of Medicine, Salt Lake City, USA
    Mol Diagn 9:105-18. 2005
  3. ncbi request reprint The Long QT and Brugada syndromes: causes of unexpected syncope and sudden cardiac death in children and young adults
    G Michael Vincent
    University of Utah School of Medicine, Department of Medicine, LDS Hospital, Salt Lake City, UT 84103, USA
    Semin Pediatr Neurol 12:15-24. 2005
  4. ncbi request reprint Congenital Long QT syndrome
    G Michael Vincent
    Department of Medicine, LDS Hospital and University of Utah School of Medicine, 324 10th Avenue, Suite 130, Salt Lake City, UT 84103, USA
    Card Electrophysiol Rev 6:57-60. 2002
  5. ncbi request reprint Commotio cordis: a deadly consequence of chest trauma
    G M Vincent
    Department of Medicine, LDS Hospital, Salt Lake City, UT, 84143, USA
    Phys Sportsmed 28:31-9. 2000
  6. ncbi request reprint Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry
    E H Locati
    Department of Medicine, University of Rochester, NY, USA
    Circulation 97:2237-44. 1998
  7. ncbi request reprint Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2
    I Splawski
    Department of Human Genetics, Howard Hughes Medical Institute, Division of Cardiology, Salt Lake City, Utah, USA
    Circulation 102:1178-85. 2000
  8. ncbi request reprint Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome
    A J Moss
    Department of Medicine, University of Rochester School of Medicine and Dentistry, NY 14642, USA
    Circulation 101:616-23. 2000
  9. ncbi request reprint Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome
    A J Moss
    Department of Medicine, University of Rochester School of Medicine and Dentistry, New York, USA
    Am J Cardiol 84:876-9. 1999
  10. ncbi request reprint Genomic structure of three long QT syndrome genes: KVLQT1, HERG, and KCNE1
    I Splawski
    Cardiology Division, University of Utah, Salt Lake City, Utah, 84112, USA
    Genomics 51:86-97. 1998

Collaborators

Detail Information

Publications35

  1. ncbi request reprint The molecular genetics of the long QT syndrome: genes causing fainting and sudden death
    G M Vincent
    Department of Internal Medicine, LDS Hospital, Salt Lake City, Utah 84143, USA
    Annu Rev Med 49:263-74. 1998
    ..The manifestations vary, depending on the genotype present. The phenotype also probably varies, depending on the specific mutation involved. Phenotypic heterogeneity is also caused by variable penetrance and expressivity...
  2. ncbi request reprint The role of genotyping in diagnosing cardiac channelopathies : progress to date
    G Michael Vincent
    LDS Hospital and University of Utah School of Medicine, Salt Lake City, USA
    Mol Diagn 9:105-18. 2005
    ..The recommendations will progressively change as new research findings and new genotyping technologies appear...
  3. ncbi request reprint The Long QT and Brugada syndromes: causes of unexpected syncope and sudden cardiac death in children and young adults
    G Michael Vincent
    University of Utah School of Medicine, Department of Medicine, LDS Hospital, Salt Lake City, UT 84103, USA
    Semin Pediatr Neurol 12:15-24. 2005
    ..Very effective treatments are available and prompt, correct diagnosis and appropriate treatment are life-saving...
  4. ncbi request reprint Congenital Long QT syndrome
    G Michael Vincent
    Department of Medicine, LDS Hospital and University of Utah School of Medicine, 324 10th Avenue, Suite 130, Salt Lake City, UT 84103, USA
    Card Electrophysiol Rev 6:57-60. 2002
  5. ncbi request reprint Commotio cordis: a deadly consequence of chest trauma
    G M Vincent
    Department of Medicine, LDS Hospital, Salt Lake City, UT, 84143, USA
    Phys Sportsmed 28:31-9. 2000
    ..Preventive measures include education of participants and coaches, chest protection, and softer baseballs. Other considerations include having external automatic defibrillators and trained personnel at youth sporting events...
  6. ncbi request reprint Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry
    E H Locati
    Department of Medicine, University of Rochester, NY, USA
    Circulation 97:2237-44. 1998
    ..Our objectives were to evaluate age- and sex-related differences in events among LQTS patients referred to the LQTS International Registry...
  7. ncbi request reprint Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2
    I Splawski
    Department of Human Genetics, Howard Hughes Medical Institute, Division of Cardiology, Salt Lake City, Utah, USA
    Circulation 102:1178-85. 2000
    ..Mutations in KVLQT1 and KCNE1 also cause the Jervell and Lange-Nielsen syndrome, a form of LQTS associated with deafness, a phenotypic abnormality inherited in an autosomal recessive fashion...
  8. ncbi request reprint Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome
    A J Moss
    Department of Medicine, University of Rochester School of Medicine and Dentistry, NY 14642, USA
    Circulation 101:616-23. 2000
    ..beta-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness and limitations of beta-blockers in this disorder have not been evaluated...
  9. ncbi request reprint Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome
    A J Moss
    Department of Medicine, University of Rochester School of Medicine and Dentistry, New York, USA
    Am J Cardiol 84:876-9. 1999
    ..The clinical profile and genotype findings of patients with LQTS who experience cardiac events related to acute auditory stimuli are quite different from those who experience events accompanying swimming activities...
  10. ncbi request reprint Genomic structure of three long QT syndrome genes: KVLQT1, HERG, and KCNE1
    I Splawski
    Cardiology Division, University of Utah, Salt Lake City, Utah, 84112, USA
    Genomics 51:86-97. 1998
    ..This work has clinical implications for presymptomatic diagnosis and therapy...
  11. ncbi request reprint Clinical implications for affected parents and siblings of probands with long-QT syndrome
    J Kimbrough
    Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA
    Circulation 104:557-62. 2001
    ..CONCLUSIONS: The severity profile of LQTS in a proband was not found to be useful in identifying the clinical severity of LQTS in affected first-degree relatives of the proband...
  12. pmc Clinical aspects of type-1 long-QT syndrome by location, coding type, and biophysical function of mutations involving the KCNQ1 gene
    Arthur J Moss
    Cardiology Division, Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA
    Circulation 115:2481-9. 2007
    ..We evaluated the effect of location, coding type, and biophysical function of KCNQ1 mutations on the clinical phenotype of this disorder...
  13. ncbi request reprint Improved long-term survival associated with stent deployment during percutaneous coronary interventions: results from a registry of 3399 patients
    Joseph B Muhlestein
    Division of Cardiology, Department of Cardiovascular Medicine, LDS Hospital, Salt Lake City, Utah 84143, USA
    Am Heart J 150:182-7. 2005
    ..However, few studies have had a sufficient sample size or adequate follow-up to determine whether this advantage results in a positive effect on mortality...
  14. ncbi request reprint An intronic mutation causes long QT syndrome
    Li Zhang
    LDS Hospital, Salt Lake City, Utah 84103, USA
    J Am Coll Cardiol 44:1283-91. 2004
    ..The purpose of this research was to determine whether an intronic variant (T1945+6C) in KCNH2 is a disease-causing mutation, and if expanded phenotyping criteria produce improved identification of long QT syndrome (LQTS) patients...
  15. ncbi request reprint Electrocardiographic features in Andersen-Tawil syndrome patients with KCNJ2 mutations: characteristic T-U-wave patterns predict the KCNJ2 genotype
    Li Zhang
    LDS Hospital, 324 10th Ave, Suite 130, Salt Lake City, Utah 84103, USA
    Circulation 111:2720-6. 2005
    ..This study aimed to define ECG features of KCNJ2 mutation carriers, to determine whether characteristic T-U-wave patterns exist, and to establish whether T-U patterns predict the ATS1 genotype...
  16. pmc Long QT syndrome in patients over 40 years of age: increased risk for LQTS-related cardiac events in patients with coronary disease
    Edward Sze
    Cardiology Division, Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642 8653, USA
    Ann Noninvasive Electrocardiol 13:327-31. 2008
    ....
  17. ncbi request reprint Corrected QT variability in serial electrocardiograms in long QT syndrome: the importance of the maximum corrected QT for risk stratification
    Ilan Goldenberg
    Cardiology Division, Department of Medicine, University of Rochester Medical Center, Rochester, New York 14642, USA
    J Am Coll Cardiol 48:1047-52. 2006
    ..We evaluated the incremental prognostic information provided by multiple corrected QT (QTc) measurements on serial electrocardiograms (ECGs) in patients with the inherited long QT syndrome (LQTS)...
  18. ncbi request reprint Modulating effects of age and gender on the clinical course of long QT syndrome by genotype
    Wojciech Zareba
    Department of Medicine, Heart Research Follow Up Program, Medical Center, University of Rochester School of Medicine, Rochester, NY 14642 8653, USA
    J Am Coll Cardiol 42:103-9. 2003
    ..We aimed to determine whether long QT syndrome (LQTS) genotype has a differential effect on clinical course of disease in male and female children and adults after adjustment for QTc duration...
  19. ncbi request reprint The long-QT syndrome--bedside to bench to bedside
    G Michael Vincent
    Department of Internal Medicine, LDS Hospital and the University of Utah, Salt Lake City, USA
    N Engl J Med 348:1837-8. 2003
  20. doi request reprint High efficacy of beta-blockers in long-QT syndrome type 1: contribution of noncompliance and QT-prolonging drugs to the occurrence of beta-blocker treatment "failures"
    G Michael Vincent
    Department of Medicine, LDS Hospital and University of Utah, Salt Lake City, UT, USA
    Circulation 119:215-21. 2009
    ..Beta-blocker efficacy in long-QT syndrome type 1 is good but variably reported, and the causes of cardiac events despite beta-blocker therapy have not been ascertained...
  21. doi request reprint Risk of cardiac events in patients with asthma and long-QT syndrome treated with beta(2) agonists
    Princy Thottathil
    Cardiology Division of the Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York, USA
    Am J Cardiol 102:871-4. 2008
    ..14, p = 0.05). In conclusion, beta(2)-agonist therapy was associated with an increased risk for cardiac events in patients with asthma with LQTS, and this risk was diminished in patients receiving beta blockers...
  22. pmc Risk of death in the long QT syndrome when a sibling has died
    Elizabeth S Kaufman
    Heart and Vascular Research Center, MetroHealth Campus, Case Western Reserve University, Cleveland, Ohio, USA
    Heart Rhythm 5:831-6. 2008
    ..Sudden death of a sibling is thought to be associated with greater risk of death in long QT syndrome (LQTS). However, there is no evidence of such an association...
  23. doi request reprint Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome
    Ilan Goldenberg
    Cardiology Division of the Department of Medicine, University of Rochester Medical Center, Rochester, NY 14642, USA
    Circulation 117:2184-91. 2008
    ..However, specific risk factors for life-threatening cardiac events in children with this genetic disorder have not been identified...
  24. doi request reprint Long-QT syndrome after age 40
    Ilan Goldenberg
    Cardiology Division of the Department of Medicine, University of Rochester Medical Center, Rochester, NY 14642, USA
    Circulation 117:2192-201. 2008
    ....
  25. pmc Nonsense mutations in hERG cause a decrease in mutant mRNA transcripts by nonsense-mediated mRNA decay in human long-QT syndrome
    Qiuming Gong
    Division of Cardiovascular Medicine, Oregon Health and Science University, 3181 SW Sam Jackson Park Rd, Portland, OR 97239, USA
    Circulation 116:17-24. 2007
    ..However, the role of nonsense-mediated mRNA decay in LQT2 mutations has not been explored...
  26. ncbi request reprint Long QT syndrome and pregnancy
    Rahul Seth
    Cardiology Division of the Department of Medicine, University of Rochester Medical Center, Rochester, New York 14642, USA
    J Am Coll Cardiol 49:1092-8. 2007
    ..This study was designed to investigate the clinical course of women with long QT syndrome (LQTS) throughout their potential childbearing years...
  27. ncbi request reprint Location of mutation in the KCNQ1 and phenotypic presentation of long QT syndrome
    Wojciech Zareba
    Cardiology Unit of the Department of Medicine, Heart Research Follow Up Program, Box 653, University of Rochester Medical Center, Rochester, NY 14642, USA
    J Cardiovasc Electrophysiol 14:1149-53. 2003
    ..The aim of this study was to determine whether there is an association between the location of mutations in the KCNQ1 gene and cardiac events in LQT1 patients...
  28. ncbi request reprint Risk assessment in long QT syndrome: the Achilles heel of appropriate treatment
    G Michael Vincent
    Heart Rhythm 2:505-6. 2005
  29. ncbi request reprint Atrial arrhythmias in the inherited long QT syndrome: laboratory quirk or clinical arrhythmia?
    G Michael Vincent
    J Cardiovasc Electrophysiol 14:1034-5. 2003
  30. ncbi request reprint 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
    Arthur J Moss
    Cardiology Unit of the Department of Medicine, University of Rochester Medical Center, Rochester, NY 14642, USA
    Circulation 105:794-9. 2002
    ..We investigated the clinical features and prognostic implications of mutations involving pore and nonpore regions of the HERG channel in the LQT2 form of this disorder...
  31. ncbi request reprint Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome
    Jenny B Hobbs
    Cardiology Unit of the Department of Medicine, University of Rochester Medical Center, Rochester, NY, USA
    JAMA 296:1249-54. 2006
    ..Risk factors specific for aborted cardiac arrest and sudden cardiac death have not been investigated...
  32. ncbi request reprint Long QT syndrome in adults
    Andrew J Sauer
    Cardiology Unit of the Department of Medicine, University of Rochester Medical Center, Rochester, New York 14642 8653, USA
    J Am Coll Cardiol 49:329-37. 2007
    ....
  33. pmc Protective effect of KCNH2 single nucleotide polymorphism K897T in LQTS families and identification of novel KCNQ1 and KCNH2 mutations
    Xianqin Zhang
    Key Laboratory of Molecular Biophysics of the Ministry of Education, College of Life Science and Technology and Center for Human Genome Research, Huazhong University of Science and Technology, Wuhan, Hubei 430074, PR China
    BMC Med Genet 9:87. 2008
    ..To investigate the disease expressivity, this study aimed to identify mutations and common variants that can modify LQTS phenotype...
  34. pmc A splice site mutation in hERG leads to cryptic splicing in human long QT syndrome
    Qiuming Gong
    Division of Cardiovascular Medicine, Oregon Health and Science University, Portland, OR, USA
    J Mol Cell Cardiol 44:502-9. 2008
    ..Our results demonstrate that 2398+1G>C activates a cryptic site and generates a full-length hERG protein with an insertion of 18 amino acids, which leads to a trafficking defect of the mutant channel...
  35. ncbi request reprint Inaccurate electrocardiographic interpretation of long QT: the majority of physicians cannot recognize a long QT when they see one
    Sami Viskin
    Department of Cardiology, Tel Aviv Sourasky Medical Center, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
    Heart Rhythm 2:569-74. 2005
    ..It is important to define the percentage of physicians capable of distinguishing QT intervals that are long from those that are normal because LQTS can be lethal when left untreated...