Permanent cardiac pacing in patients with the long QT syndrome

A permanent pacemaker was inserted in eight patients with the long QT syndrome. All had recurrent syncope or seizures, six had documented torsade de pointes and four had aborted sudden death. Among the eight patients, permanent pacing was instituted in three who were unsuccessfully treated with both a beta-adrenergic blocking agent and left cardiothoracic sympathectomy, and in two who proved refractory or intolerant to beta-blockers. Another three patients had pacemaker implantation and long-term beta-blocker therapy because of spontaneous atrioventricular (AV) block in one, aborted sudden death in one and patient preference in one. After pacing (70 to 85 beats/min), there was no significant change in the mean corrected QT interval, but the mean QT interval decreased significantly (534.4 +/- 51.4 to 425.6 +/- 18.9 ms, p less than 0.0001). Over a mean follow-up period of 35.1 +/- 18.9 months, all patients are alive and currently free of syncope. One patient without a history of stress-induced syncope had two syncopal episodes (believed to be due to hyperventilation) while under severe emotional stress, but has been symptom free for the past 5 years. One patient with an atrial demand (AAI) pacemaker developed dizziness due to documented episodes of AV block, but remains asymptomatic after conversion to atrial rate-responsive dual chamber (DDD) pacing. Either atrial or ventricular pacing combined with beta-blocker therapy appears to be effective treatment for a subset of patients with the long QT syndrome, by either preventing episodes of torsade de pointes or alleviating symptoms due to bradycardia from beta-blocker therapy.

This article has been cited by other articles:

				A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N. A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons J. Am. Coll. Cardiol., May 27, 2008; 51(21): e1 - e62. [Full Text] [PDF]

				A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N. A. Mark Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons J. Am. Coll. Cardiol., May 27, 2008; 51(21): 2085 - 2105. [Full Text] [PDF]

				Writing Committee Members, A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons Circulation, May 27, 2008; 117(21): 2820 - 2840. [Full Text] [PDF]

				Writing Committee Members, A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons Circulation, May 27, 2008; 117(21): e350 - e408. [Full Text] [PDF]

				R. Passman and A. Kadish Sudden Death Prevention With Implantable Devices Circulation, July 31, 2007; 116(5): 561 - 571. [Full Text] [PDF]

				V. Khalameizer, N. Pancheva, L. Reizin, and I. E. Ovsyshcher "Benign" course and malignant clinical presentations of congenital long QT syndrome Europace, January 1, 2005; 7(1): 50 - 53. [Abstract] [Full Text] [PDF]

				X. H.T. Wehrens, M. A. Vos, P. A. Doevendans, and H. J.J. Wellens Novel Insights in the Congenital Long QT Syndrome Ann Intern Med, December 17, 2002; 137(12): 981 - 992. [Abstract] [Full Text] [PDF]

				Committee Members, G. Gregoratos, J. Abrams, A. E. Epstein, R. A. Freedman, D. L. Hayes, M. A. Hlatky, R. E. Kerber, G. V. Naccarelli, M. H. Schoenfeld, et al. ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices--Summary Article: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines) J. Am. Coll. Cardiol., November 6, 2002; 40(9): 1703 - 1719. [Full Text] [PDF]

				G. Gregoratos, J. Abrams, A. E. Epstein, R. A. Freedman, D. L. Hayes, M. A. Hlatky, R. E. Kerber, G. V. Naccarelli, M. H. Schoenfeld, M. J. Silka, et al. ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices: Summary Article: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines) Circulation, October 15, 2002; 106(16): 2145 - 2161. [Full Text] [PDF]

				S Viskin, R Fish, D Zeltser, B Belhassen, K Heller, D Brosh, S Laniado, and H V Barron Arrhythmias in the congenital long QT syndrome: how often is torsade de pointes pause dependent? Heart, June 1, 2000; 83(6): 661 - 666. [Abstract] [Full Text]

				P. C. Dorostkar, M. Eldar, B. Belhassen, and M. M. Scheinman Long-Term Follow-Up of Patients With Long-QT Syndrome Treated With {beta}-Blockers and Continuous Pacing Circulation, December 14, 1999; 100(24): 2431 - 2436. [Abstract] [Full Text] [PDF]

				S Viskin, R Fish, A Roth, and Y Copperman Prevention of torsade de pointes in the congenital long QT syndrome: use of a pause prevention pacing algorithm Heart, April 1, 1998; 79(4): 417 - 419. [Abstract] [Full Text]

				D. M. Roden, R. Lazzara, M. Rosen, P. J. Schwartz, J. Towbin, and G. M. Vincent Multiple Mechanisms in the Long-QT Syndrome: Current Knowledge, Gaps, and Future Directions Circulation, October 15, 1996; 94(8): 1996 - 2012. [Abstract] [Full Text]

				M. R. Rosen Long QT Syndrome Patients With Gene Mutations Circulation, December 15, 1995; 92(12): 3373 - 3375. [Full Text]

				R. Brooks, B. A. McGovern, H. Garan, and J. N. Ruskin Current Treatment of Patients Surviving Out-of-Hospital Cardiac Arrest JAMA, February 13, 1991; 265(6): 762 - 768. [Abstract] [PDF]

				A. S. Manolis, M. Linzer, D. Salem, and N. A. M. Estes III Syncope: Current Diagnostic Evaluation and Management Ann Intern Med, June 1, 1990; 112(11): 850 - 863. [Abstract] [PDF]