Correspondence

Long-QT Syndrome

N Engl J Med 2008; 358:1967-1968May 1, 2008

Article

To the Editor:

In his discussion of the Clinical Practice vignette involving a fatal arrhythmic event in a child with the long-QT syndrome, Roden (Jan. 10 issue)1 emphasizes the importance of rigorously screening family members in order to provide an optimal prevention strategy. The author recommends that affected persons not participate in competitive sports; he also mentions the benefit of beta-blocker therapy in these patients and the use of implantable cardiac defibrillators in the highest-risk cases. However, the current recommendations consider “lifestyle modifications,” defined by the contraindication of competitive sports activity and of all drugs known to prolong the QT interval, as a class I recommendation (level of evidence B).2 According to such recommendations, education of these patients about the risk associated with certain drugs must be clearly integrated into the strategy of fatal-arrhythmia prevention. All these drugs are listed and regularly updated in the International Registry for Drug-Induced Arrhythmias (www.qtdrugs.org).

Eloi Marijon, M.D.
European Georges Pompidou Hospital, 75908 Paris, France
eloi_marijon@yahoo.fr

Nicolas Combes, M.D.
Jean Paul Albenque, M.D.
Clinique Pasteur, 31076 Toulouse, France

2 References

1. 1

   Roden DM. Long-QT syndrome. N Engl J Med 2008;358:169-176
   Full Text | Web of Science | Medline

2. 2

   Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006 Guidelines for Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Circulation 2006;114:e385-e484
   CrossRef | Medline

To the Editor:

Effective therapies exist for the long-QT syndrome. The use of beta-blockers should be viewed as the mainstay of therapy, as noted in the Clinical Practice article. Although there is a reduction in cardiovascular events among patients with the long-QT syndrome who receive beta-blocker therapy, a substantial proportion of patients will have devastating events while receiving beta-blocker therapy.1 Thus, left cardiac sympathetic denervation (LCSD) should be highlighted as well, not only as a viable therapeutic option but also as one that is distinct from and can be used as an alternative to mere beta-blockade in high-risk patients, including those who do not have a response to traditional beta-blockade. In a study of high-risk patients with the long-QT syndrome, 75% of whom had cardiovascular events despite treatment with beta-blockers, LCSD was associated with a significant reduction in the incidence of aborted cardiac arrest and syncope.2 The mean yearly number of cardiovascular events in patients treated with LCSD decreased significantly, by 91%, in this study. The authors concluded that “LCSD should be considered in patients with recurrent syncope despite beta-blockade.”

John R. Kapoor, M.D., Ph.D.
Stanford University, Stanford, CA 94305
jkapoor@stanford.edu

2 References

1. 1

   Moss AJ, Zareba W, Hall WJ, et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 2000;101:616-623
   Web of Science | Medline

2. 2

   Schwartz PJ, Priori SG, Cerrone M, et al. Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation 2004;109:1826-1833
   CrossRef | Web of Science | Medline

Author/Editor Response

I agree with the emphasis placed by Marijon et al. on an integrated approach — involving many aspects of lifestyle — to the care of patients and families with the long-QT syndrome. This includes minimizing exposure to potential triggers such as activity and drugs.

Kapoor notes the potential role of left stellate ganglionectomy in the management of the long-QT syndrome. In their 2004 report, Schwartz et al.1 described excellent results in high-risk patients, whereas earlier reports had suggested less success.2,3 This difference could reflect different patient populations or differences in specific surgical approaches. In my view, ganglionectomy should be considered in patients who have recurrent arrhythmias during conventional therapies; the procedure should be performed in centers that are experienced with the technique in these patients.

Both Marijon et al. and Kapoor address management issues in the long-QT syndrome. It is important to bear in mind that the past decade has seen tremendous advances in our understanding of the fundamental basis of this disease and its subtypes. This information is only now being used in patient care, and the hope is that management guidelines will continue to evolve and improve.

Dan M. Roden, M.D.
Vanderbilt University School of Medicine, Nashville, TN 37232-0575
dan.roden@vanderbilt.edu

3 References

1. 1

   Schwartz PJ, Priori SG, Cerrone M, et al. Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation 2004;109:1826-1833
   CrossRef | Web of Science | Medline

2. 2

   Bhandari AK, Scheinman MM, Morady F, Svinarich J, Mason J, Winkle R. Efficacy of left cardiac sympathectomy in the treatment of patients with the long QT syndrome. Circulation 1984;70:1018-1023
   CrossRef | Web of Science | Medline

3. 3

   Moss AJ, Schwartz PJ, Crampton RS, et al. The long QT syndrome: prospective longitudinal study of 328 families. Circulation 1991;84:1136-1144
   Web of Science | Medline