Catheter ablation of ventricular fibrillation storm in a long QT syndrome genotype carrier with normal QT interval

Yap J, Tan VH, Hsu LF, Liew R
Correspondence: Dr Reginald Liew, reginald.liew@duke-nus.edu.sg

ABSTRACT
Patients with long QT syndrome can sometimes present with a ventricular fibrillation (VF) storm. Catheter ablation of culprit premature ventricular complexes responsible for the triggering of the VF episodes may be required in rare cases of electrical storm that do not respond to conventional measures, and this can be life-saving. We describe a case of emergency catheter ablation in a young woman with a normal corrected QT interval, who presented with malignant VF storm for the first time. We also discuss the diagnostic and management challenges involved, as well as the value of genetic testing in refining the diagnosis.

Keywords: catheter ablation, long QT syndrome, ventricular fibrillation
Singapore Med J 2013; 54:e1-e4; http://dx.doi.org/10.11622/smedj.2013015

http://smj.org.sg/sites/default/files/5401/5401cr1.pdf

REFERENCES

1. Goldenberg I, Horr S, Moss AJ, et al. Risk for life-threatening cardiac events in patients with genotype-confirmed long-QT syndrome and normal-range corrected QT intervals. J Am Coll Cardiol 2011; 57:51-9. http://dx.doi.org/10.1016/j.jacc.2010.07.038

2. Haissaguerre M, Shoda M, Jais P, et al. Mapping and ablation of idiopathic ventricular fibrillation. Circulation 2002; 106:962-7. http://dx.doi.org/10.1161/?01.CIR.0000027564.55739.B1

3. Peichl P, Cihak R, Kozeluhova M, et al. Catheter ablation of arrhythmic storm triggered by monomorphic ectopic beats in patients with coronary artery disease. J Interv Card Electrophysiol 2010; 27:51-9. http://dx.doi.org/10.1007/s10840-009-9443-2

4. Vincent GM, Timothy KW, Leppert M, Keating M. The spectrum of symptoms and QT intervals in carriers of the gene for the long-QT syndrome. N Engl J Med 1992; 327:846-52. http://dx.doi.org/10.1056/NEJM199209173271204

5. Kapplinger JD, Tester DJ, Salisbury BA, et al. Spectrum and prevalence of mutations from the first 2,500 consecutive unrelated patients referred for the FAMILION long QT syndrome genetic test. Heart Rhythm 2009; 6:1297-303. http://dx.doi.org/10.1016/j.hrthm.2009.05.021

6. Moss AJ, Zareba W, Kaufman ES, et al. 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. Circulation 2002; 105:794-9. http://dx.doi.org/10.1161/hc0702.105124

7. Migdalovich D, Moss AJ, Lopes CM, et al. Mutation and gender-specific risk in type 2 long QT syndrome: implications for risk stratification for life-threatening cardiac events in patients with long QT syndrome. Heart Rhythm 2011; 8:1537-43. http://dx.doi.org/10.1016/j.hrthm.2011.03.049

8. Kim JA, Lopes CM, Moss AJ, et al. Trigger-specific risk factors and response to therapy in long QT syndrome type 2. Heart Rhythm 2010; 7:1797-805. http://dx.doi.org/10.1016/j.hrthm.2010.09.011

9. Dursun R, Alpaslan M, Caliskan M, et al. Isotretinoin does not prolong QT intervals and QT dispersion in patients with severe acne: a surprising finding for a drug with numerous side effects. J Drugs Dermatol 2011; 10:710-4.

10. Srivatsa UN, Ebrahimi R, El-Bialy A, Wachsner RY. Electrical storm: case series and review of management. J Cardiovasc Pharmacol Ther 2003; 8:237-46. http://dx.doi.org/10.1177/107424840300800309

11. Srivathsan K, Gami AS, Ackerman MJ, Asirvatham SJ. Treatment of ventricular fibrillation in a patient with prior diagnosis of long QT syndrome: importance of precise electrophysiologic diagnosis to successfully ablate the trigger. Heart Rhythm 2007; 4:1090-3. http://dx.doi.org/10.1016/j.hrthm.2007.03.006

12.Haissaguerre M, Extramiana F, Hocini M, et al. Mapping and ablation of ventricular fibrillation associated with long-QT and Brugada syndromes. Circulation 2003; 108:925-8. http://dx.doi.org/10.1161/01.CIR.0000088781.99943.95

13.Haissaguerre M, Shah DC, Jais P, et al. Role of Purkinje conducting system in triggering of idiopathic ventricular fibrillation. Lancet 2002; 359:677-8. http://dx.doi.org/10.1016/S0140-6736(02)07807-8

14.Knecht S, Sacher F, Wright M, et al. Long-term follow-up of idiopathic ventricular fibrillation ablation: a multicenter study. J Am Coll Cardiol 2009; 54:522-8. http://dx.doi.org/10.1016/j.jacc.2009.03.065

15. Reithmann C, Fiek M, Beckmann BM, Kaab S. Recurrent torsades de pointes after catheter ablation of incessant ventricular bigeminy in combination with QT prolongation. Europace 2012; 14:299-300. http://dx.doi.org/10.1093/europace/eur278