Lack of uniform progression of endocardial scar in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy and ventricular tachycardia

Michael P. Riley, Erica Zado, Rupa Bala, David J. Callans, Joshua Cooper, Sanjay Dixit, Fermin Garcia, Edward P. Gerstenfeld, Mathew D. Hutchinson, David Lin, Vickas Patel, Ralph Verdino, Francis E. Marchlinski

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

Background-The endocardial substrate for ventricular arrhythmias in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is thought to be caused by a progressive degenerative process. Many clinical decisions and treatment plans are guided by this pathophysiologic assumption, but the extent of progression of macroscopic endocardial scar and right ventricular (RV) dilatation have not been assessed. Methods and Results-Eleven patients with ARVD/C and ventricular tachycardia had 2 detailed sinus rhythm electroanatomic endocardial voltage maps (average, 291±122 points per map; range, 114 to 558 points) performed a mean of 57 months apart (minimum, 9 months) as part of ventricular tachycardia ablation procedures. Voltage-defined scar (2; 32 cm2) confirmed by visual inspection. The remaining 9 (81%; 95% CI, 48% to 98%) patients had no increase (2 difference) in scar area between studies. In contrast, 10 of the 11 patients had a significant increase in RV volume, with an average increase of 24% (212±67 mL to 263 ±52 mL; P≤0.01). Conclusions-In patients with ARVD/C and ventricular tachycardia, progressive RV dilatation is the rule, and rapid progression of significant macroscopic endocardial scar occurs in only a subset of patients. These results have important management implications, suggesting that efforts to prevent RV dilatation in this population are needed and that an aggressive substrate-based ablation strategy offers the potential to provide long-term ventricular tachycardia control.

Original languageEnglish (US)
Pages (from-to)332-338
Number of pages7
JournalCirculation: Arrhythmia and Electrophysiology
Volume3
Issue number4
DOIs
StatePublished - Aug 2010
Externally publishedYes

Fingerprint

Arrhythmogenic Right Ventricular Dysplasia
Ventricular Tachycardia
Cicatrix
Dilatation
Cardiac Arrhythmias
Population

Keywords

  • Cardiomyopathies
  • Catheter ablation
  • Endocardial mapping
  • Ventricular remodeling
  • Ventricular tachycardia

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)
  • Medicine(all)

Cite this

Lack of uniform progression of endocardial scar in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy and ventricular tachycardia. / Riley, Michael P.; Zado, Erica; Bala, Rupa; Callans, David J.; Cooper, Joshua; Dixit, Sanjay; Garcia, Fermin; Gerstenfeld, Edward P.; Hutchinson, Mathew D.; Lin, David; Patel, Vickas; Verdino, Ralph; Marchlinski, Francis E.

In: Circulation: Arrhythmia and Electrophysiology, Vol. 3, No. 4, 08.2010, p. 332-338.

Research output: Contribution to journalArticle

Riley, MP, Zado, E, Bala, R, Callans, DJ, Cooper, J, Dixit, S, Garcia, F, Gerstenfeld, EP, Hutchinson, MD, Lin, D, Patel, V, Verdino, R & Marchlinski, FE 2010, 'Lack of uniform progression of endocardial scar in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy and ventricular tachycardia', Circulation: Arrhythmia and Electrophysiology, vol. 3, no. 4, pp. 332-338. https://doi.org/10.1161/CIRCEP.109.919530
Riley, Michael P. ; Zado, Erica ; Bala, Rupa ; Callans, David J. ; Cooper, Joshua ; Dixit, Sanjay ; Garcia, Fermin ; Gerstenfeld, Edward P. ; Hutchinson, Mathew D. ; Lin, David ; Patel, Vickas ; Verdino, Ralph ; Marchlinski, Francis E. / Lack of uniform progression of endocardial scar in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy and ventricular tachycardia. In: Circulation: Arrhythmia and Electrophysiology. 2010 ; Vol. 3, No. 4. pp. 332-338.
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abstract = "Background-The endocardial substrate for ventricular arrhythmias in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is thought to be caused by a progressive degenerative process. Many clinical decisions and treatment plans are guided by this pathophysiologic assumption, but the extent of progression of macroscopic endocardial scar and right ventricular (RV) dilatation have not been assessed. Methods and Results-Eleven patients with ARVD/C and ventricular tachycardia had 2 detailed sinus rhythm electroanatomic endocardial voltage maps (average, 291±122 points per map; range, 114 to 558 points) performed a mean of 57 months apart (minimum, 9 months) as part of ventricular tachycardia ablation procedures. Voltage-defined scar (2; 32 cm2) confirmed by visual inspection. The remaining 9 (81{\%}; 95{\%} CI, 48{\%} to 98{\%}) patients had no increase (2 difference) in scar area between studies. In contrast, 10 of the 11 patients had a significant increase in RV volume, with an average increase of 24{\%} (212±67 mL to 263 ±52 mL; P≤0.01). Conclusions-In patients with ARVD/C and ventricular tachycardia, progressive RV dilatation is the rule, and rapid progression of significant macroscopic endocardial scar occurs in only a subset of patients. These results have important management implications, suggesting that efforts to prevent RV dilatation in this population are needed and that an aggressive substrate-based ablation strategy offers the potential to provide long-term ventricular tachycardia control.",
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AU - Zado, Erica

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AU - Callans, David J.

AU - Cooper, Joshua

AU - Dixit, Sanjay

AU - Garcia, Fermin

AU - Gerstenfeld, Edward P.

AU - Hutchinson, Mathew D.

AU - Lin, David

AU - Patel, Vickas

AU - Verdino, Ralph

AU - Marchlinski, Francis E.

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AB - Background-The endocardial substrate for ventricular arrhythmias in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is thought to be caused by a progressive degenerative process. Many clinical decisions and treatment plans are guided by this pathophysiologic assumption, but the extent of progression of macroscopic endocardial scar and right ventricular (RV) dilatation have not been assessed. Methods and Results-Eleven patients with ARVD/C and ventricular tachycardia had 2 detailed sinus rhythm electroanatomic endocardial voltage maps (average, 291±122 points per map; range, 114 to 558 points) performed a mean of 57 months apart (minimum, 9 months) as part of ventricular tachycardia ablation procedures. Voltage-defined scar (2; 32 cm2) confirmed by visual inspection. The remaining 9 (81%; 95% CI, 48% to 98%) patients had no increase (2 difference) in scar area between studies. In contrast, 10 of the 11 patients had a significant increase in RV volume, with an average increase of 24% (212±67 mL to 263 ±52 mL; P≤0.01). Conclusions-In patients with ARVD/C and ventricular tachycardia, progressive RV dilatation is the rule, and rapid progression of significant macroscopic endocardial scar occurs in only a subset of patients. These results have important management implications, suggesting that efforts to prevent RV dilatation in this population are needed and that an aggressive substrate-based ablation strategy offers the potential to provide long-term ventricular tachycardia control.

KW - Cardiomyopathies

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KW - Ventricular remodeling

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