Yonsei Med J.  2009 Jun;50(3):448-451. 10.3349/ymj.2009.50.3.448.

A Case of Catecholaminergic Polymorphic Ventricular Tachycardia

• Lee SY
• Kim JB
• Im E
• Yang WI
• Joung B
• Lee MH
• Kim SS

• KMID: 1758577
• DOI: http://doi.org/10.3349/ymj.2009.50.3.448

Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a familial cardiac arrhythmia that is related to RYR2 or CASQ2 gene mutation. It occurs in patients with structurally normal heart and causes exercise-emotion-triggered syncope and sudden cardiac death. We experienced a case of CPVT in an 11 year-old female patient who was admitted for sudden cardiovascular collapse. The initial electrocardiogram (ECG) on emergency department revealed ventricular fibrillation. After multiple defibrillations, sinus rhythm was restored. However, recurrent ventricular fibrillation occurred during insertion of nasogastric tube without sedation in coronary care unit. On ECG monitoring, bidirectional ventricular tachycardia occurred with sinus tachycardia and then degenerated into ventricular fibrillation. To our knowledge, there has been no previous case report of CPVT triggered by sinus tachycardia in Korea. Therefore, we report the case as well as a review of the literature.

Keyword

Catecholamine; ventricular tachycardia

MeSH Terms

Catecholamines/*metabolism
Child
Electrocardiography
Female
Humans
Tachycardia, Ventricular/*diagnosis/genetics/pathology

Figure

• 

  Fig. 1 Six years ago, the resting electrocardiogram showed normal sinus rhythm without delta wave nor QT interval prolongation (QTc 399 ms).

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  Fig. 2 The initial rhythm strip in emergency department showed ventricular defibrillation. After 200 J defibrillation (arrow), sinus rhythm was restored.

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  Fig. 3 The rhythm strip in coronary care unit. The sinus tachycardia (arrow) triggered bidirectional ventricular tachycardia (arrow-heads). Then, the bidirectional ventricular tachycardia degenerated into ventricular fibrillation.

Reference

1. Reid DS, Tynan M, Braidwood L, Fitzgerald GR. Bidirectional tachycardia in a child. A study using His bundle electrography. Br Heart J. 1975. 37:339–344.

2. Coumel P, Fidelle J, Lucet V, Attuel P, Bouvrain Y. Catecholaminergic-induced severe ventricular arrhythmias with Adams-Stokes syndrome in children: report of four cases. Br Heart J. 1978. 40:28–37.

3. Laitinen PJ, Brown KM, Piippo K, Swan H, Devaney JM, Brahmbhatt B, et al. Mutations of the cardiac ryanodine receptor (RyR2) gene in familial polymorphic ventricular tachycardia. Circulation. 2001. 103:485–490.

4. George CH, Higgs GV, Lai FA. Ryanodine receptor mutations associated with stress-induced ventricular tachycardia mediate increased calcium release in stimulated cardiomyocytes. Circ Res. 2003. 93:531–540.

5. Lahat H, Pras E, Olender T, Avidan N, Ben-Asher E, Man O, et al. A missense mutation in a highly conserved region of CASQ2 is associated with autosomal recessive catecholamine-induced polymorphic ventricular tachycardia in Bedouin families from Israel. Am J Hum Genet. 2001. 69:1378–1384.

6. Leenhardt A, Lucet V, Denjoy I, Grau F, Ngoc DD, Coumel P. Catecholaminergic polymorphic ventricular tachycardia in children. A 7-year follow-up of 21 patients. Circulation. 1995. 91:1512–1519.

7. Tester DJ, Spoon DB, Valdivia HH, Makielski JC, Ackerman MJ. Targeted mutational analysis of the RyR2-encoded cardiac ryanodine receptor in sudden unexplained death: a molecular autopsy of 49 medical examiner/coroner's cases. Mayo Clin Proc. 2004. 79:1380–1384.

8. Priori SG, Napolitano C, Memmi M, Colombi B, Drago F, Gasparini M, et al. Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia. Circulation. 2002. 106:69–74.

9. George CH, Higgs GV, Lai FA. Ryanodine receptor mutations associated with stress-induced ventricular tachycardia mediate increased calcium release in stimulated cardiomyocytes. Circ Res. 2003. 93:531–540.

10. Yano K, Zarain-Herzberg A. Sarcoplasmic reticulum calsequestrins: structural and functional properties. Mol Cell Biochem. 1994. 135:61–70.

11. Postma AV, Denjoy I, Kamblock J, Alders M, Lupoglazoff JM, Vaksmann G, et al. Catecholaminergic polymorphic ventricular tachycardia: RYR2 mutations, bradycardia, and follow up of the patients. J Med Genet. 2005. 42:863–870.

12. Gupta A, Lawrence AT, Krishnan K, Kavinsky CJ, Trohman RG. Current concepts in the mechanisms and management of drug-induced QT prolongation and torsade de pointes. Am Heart J. 2007. 153:891–899.

13. Roden DM. Taking the "idio" out of "idiosyncratic": predicting torsades de pointes. Pacing Clin Electrophysiol. 1998. 21:1029–1034.