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2007, Number S4

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Arch Cardiol Mex 2007; 77 (S4)

Ventricular arrhythmias of catecholaminergic origin and sudden death

Jalife J
Full text How to cite this article

Language: Spanish
References: 11
Page: 175-177
PDF size: 87.39 Kb.


Key words:

Catecholaminergic ventricular tachycardia, Ryanodine receptors mutation, Purkinje fibers.

ABSTRACT

The hereditary disease known as polymorphic catecholaminergic ventricular tachycardia (PCVT) is highly lethal. Almost 30% of the affected patients die before 40 years old, mainly due to sudden cardiac death. We have used isolated hearts from mutant mice (type 2 ryanodine receptors, RyR2/RyR2R4496C) to investigate arrhythmia mechanisms that are adrenergic- and intracellular calcium ([Ca2+]o) levels-dependent. Our results corroborate that polymorphic and bidirectional ventricular arrhythmias, as well as ventricular fibrillation, occurs in 50% of RyR2/RyR2R4496C mice, and in less than 12% of the non-affected mice. Our hypothesis suggests that the origin of catecholaminergic arrhythmias in animals, and possibly in humans, is conditioned by the focal activity that begins by late post-potentials in the Purkinje fibers.


REFERENCES

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  3. Priori SG, Napolitano C, Tiso N, Memmi M, Vignati G, Bloise R, Sorrentino V, Danieli GA: Mutations in the cardiac ryanodine receptor gene (hRyR2) underlie catecholaminergic polymorphic ventricular tachycardia. Circulation 2001; 103: 196-200.

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  5. Laitinen PJ, Brown KM, Piippo K, Swan H, Devaney JM, Brahmbhatt B, Donarum EA, Marino M, Tiso N, Viitasalo M, Toivonen L, Stephan DA, Kontula K: Mutations of the cardiac ryanodine receptor (RyR2) gene in familial polymorphic ventricular tachycardia. Circulation 2001; 103: 485-490.

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  7. Jiang D, Wang R, Xiao B, Kong H, Hunt DJ, Choi P, Zhang L, Chen SR: Enhanced store overload-induced Ca2+ release and channel sensitivity to luminal Ca2+ activation are common defects of RyR2 mutations linked to ventricular tachycardia and sudden death. Circ Res 2005; 97: 1173-1181.

  8. Wehrens XH, Lehnart SE, Marks AR: Intracellular calcium release and cardiac disease. Annu Rev Physiol 2005; 67: 69-98.

  9. Cerrone M, Colombi B, Santoro M, di Barletta MR, Scelsi M, Villani L, Napolitano C, Priori SG: Bidirectional ventricular tachycardia and fibrillation elicited in a knock-in mouse model carrier of a mutation in the cardiac ryanodine receptor. Circ Res 2005; 96: e77-82.

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  11. Tamaddon HS, Vaidya D, Simon AM, Paul DL, Jalife J, Morley GE: High-resolution optical mapping of the right bundle branch in connexin40 knockout mice reveals slow conduction in the specialized conduction system. Circ Res 2000; 87: 929-936.




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Arch Cardiol Mex. 2007;77