Heart Rhythm
Volume 6, Issue 6 , Pages 769-775 , June 2009

Prolonged RV endocardial activation duration: A novel marker of arrhythmogenic right ventricular dysplasia/cardiomyopathy

  • Harikrishna Tandri, MD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    • Corresponding Author InformationAddress reprint requests and correspondence: Dr. Harikrishna Tandri, Carnegie 568, 600 North Wolfe Street, Baltimore, Maryland 21287
    • ,
  • Angeliki Asimaki, PhD

      Affiliations

    • Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts
    • ,
  • Theodore Abraham, MD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    • ,
  • Darshan Dalal, MD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    • ,
  • Laurens Tops, MD

      Affiliations

    • Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands
    • ,
  • Rahul Jain, MD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    • ,
  • Jeffrey E. Saffitz, MD, PhD

      Affiliations

    • Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts
    • ,
  • Daniel P. Judge, MD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    • ,
  • Stuart D. Russell, MD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    • ,
  • Marc Halushka, MD, PhD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    • ,
  • David A. Bluemke, MD, PhD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    • ,
  • David A. Kass, MD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    • ,
  • Hugh Calkins, MD

      Affiliations

    • Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland

Received 3 December 2008 ,Accepted 14 February 2009.

References 

  1. Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia—a report of 24 adult cases. Circulation. 1982;65:384–398
  2. Corrado D, Basso C, Thiene G, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. 1997;30:1512–1520
  3. Dalal D, Nasir K, Bomma C, et al. Arrhythmogenic right ventricular dysplasia—a United States experience. Circulation. 2005;112:3823–3832
  4. McKenna WJ, Thiene G, Nava A, et al. diagnosis of arrhythmogenic right-ventricular dysplasia/cardiomyopathy. Br Heart J. 1994;71:215–218
  5. Cox MG, Nelen MR, Wilde AA, et al. Activation delay and VT parameters in arrhythmogenic right ventricular dysplasia/cardiomyopathy: toward improvement of diagnostic ECG criteria. J Cardiovasc Electrophysiol. 2008;19:775–781
  6. Nasir K, Bomma C, Tandri H, et al. Electrocardiographic features of arrhythmogenic right ventricular dysplasia/cardiomyopathy according to disease severity—a need to broaden diagnostic criteria. Circulation. 2004;110:1527–1534
  7. Peters S, McKenna WJ, Davies MJ, et al. arrhythmogenic right-ventricular cardiomyopathy/dysplasia—ECG and echocardiography as screening methods in right-ventricular arrhythmias. Verh Dtsch Ges Herz Kreislaufforsch. 1995;27:185–187
  8. Peters S, Weber B, Peters H, et al. Localized right precordial QRS prolongation as the basic ECG finding in arrhythmogenic right ventricular dysplasia-cardiomyopathy. Circulation. 1997;96:2566
  9. Kaplan SR, Gard JJ, Protonotarios N, et al. Remodeling of myocyte gap junctions in arrhythmogenic right ventricular cardiomyopathy due to a deletion in plakoglobin (Naxos disease). Heart Rhythm. 2004;1:3–11
  10. Saffitz JE, Schuessler RB, Yamada KA. Mechanisms of remodeling of gap junction distributions and the development of anatomic substrates of arrhythmias. Cardiovasc Res. 1999;42:309–317
  11. Tsatsopoulou AA, Protonotarios NI, McKenna WJ. Arrhythmogenic right ventricular dysplasia, a cell adhesion cardiomyopathy: insights into disease pathogenesis from preliminary genotype-phenotype assessment. Heart. 2006;92:1720–1723
  12. Kaplan SR, Gard JJ, Carvajal-Huerta L, et al. Structural and molecular pathology of the heart in Carvajal syndrome. Cardiovasc Pathol. 2004;13:26–32
  13. Tandri H, Asimaki A, Dalal D, et al. Gap junction remodeling in a case of arrhythmogenic right ventricular dysplasia due to plakophilin-2 mutation. J Cardiovasc Electrophysiol. 2008;19:1212–1214
  14. Tsatsopoulou AA, Kaplan SR, Gard JJ, et al. Remodeling of gap junctions in Naxos disease: a potential mechanism of lethal arrhythmias in arrhythmogenic right ventricular cardiomyopathy. Eur Heart J. 2004;25:114
  15. Kirchhof P, Fabritz L, Zwiener M, et al. Age- and training-dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin-deficient mice. Circulation. 2006;114:1799–1806
  16. Tandri H, Castillo E, Ferrari VA, et al. Magnetic resonance imaging of arrhythmogenic right ventricular dysplasia—sensitivity, specificity, and observer variability of fat detection versus functional analysis of the right ventricle. J Am Coll Cardiol. 2006;48:2277–2284
  17. Yoerger DM, Marcus F, Sherrill D, et al. Echocardiographic findings in patients meeting task force criteria for arrhythmogenic right ventricular dysplasia—new insights from the multidisciplinary study of right ventricular dysplasia. J Am Coll Cardiol. 2005;45:860–865
  18. Corrado D, Leoni L, Bauce B, et al. Electroanatomic right ventricular voltage mapping combined with endomyocardial biopsy increases accuracy in diagnosing arrhythmogenic right ventricular cardiomyopathy/dysplasia. Eur Heart J. 2004;25:113
  19. Marchlinski FE, Zado E, Dixit S, et al. Electroanatomic substrate and outcome of catheter ablative therapy for ventricular tachycardia in setting of right ventricular cardiomyopathy. Circulation. 2004;110:2293–2298
  20. Corrado D, Basso C, Leoni L, et al. Three-dimensional electroanatomical voltage mapping and histologic evaluation of myocardial substrate in right ventricular outflow tract tachycardia. J Am Coll Cardiol. 2008;51:731–739
  21. Durrer D, van Dam RT, Freud GE, et al. Total excitation of isolated human heart. Circulation. 1970;41:899–912

 Supported by the National Heart, Lung, and Blood Institute (K23HL093350 to Dr. Tandri). The Johns Hopkins ARVD Program is supported by the Bogle Foundation, St. Jude Medical Foundation, the Healing Hearts Foundation, the Campanella family, and the Wilmerding Endowments.

 The authors thank the ARVD patients and families who have made this work possible.

PII: S1547-5271(09)00213-6

doi: 10.1016/j.hrthm.2009.02.031

Heart Rhythm
Volume 6, Issue 6 , Pages 769-775 , June 2009

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