An update on arrythmogenic right ventricular cardiomyopathy. Part 2 Review article
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Published:
Jun 30, 2015
Keywords:
arrhythmogenic right ventricular cardiomyopathy, ventricular arrhythmias, heart failure
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Abstract
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a quite common heart disease, characterized by ventricular arrhythmias and a specific ventricular pathology. It is an important cause of sudden cardiac death in young adults. The second part of the article summarizes treatment options and prognosis.
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Możeńska, O., Różańska , M., Bil , J., Furmanek , M., Jaworski , K., & Kosior , D. A. (2015). An update on arrythmogenic right ventricular cardiomyopathy. Part 2. Cardiology in Practice, 9(2), 17-25. Retrieved from https://journalsmededu.pl/index.php/kwp/article/view/1343
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References
1. Wichter T., Breithardt G.: Implantable cardioverter-defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy: a role for genotyping in decision-making? Journal of the American College of Cardiology 2005; 45(3): 409-411.
2. Zipes D.P., Camm A.J., Borggrefe M. et al.: European Heart Rhythm Association, Heart Rhythm Society: ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). Journal of the American College of Cardiology 2006; 48(5): e247-346.
3. Gemayel C., Pelliccia A., Thompson P.D.: Arrhythmogenic right ventricular cardiomyopathy. Journal of the American College of Cardiology 2001; 38(7): 1773-1781.
4. Nava A., Bauce B., Basso C. et al.: Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. Journal of the American College of Cardiology 2000; 36(7): 2226-2233.
5. Maron B.J., Ackerman M.J., Nishimura R.A. et al.: Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. Journal of the American College of Cardiology 2005; 45(8): 1340-1345.
6. Dalal D., Nasir K., Bomma C. et al.: Arrhythmogenic right ventricular dysplasia: a United States experience. Circulation 2005; 112(25): 3823-3832.
7. Hodgkinson K.A., Parfrey P.S., Bassett A.S. et al.: The impact of implantable cardioverter-defibrillator therapy on survival in autosomal-dominant arrhythmogenic right ventricular cardiomyopathy (ARVD5). Journal of the American College of Cardiology 2005; 45(3): 400-408.
8. Corrado D., Leoni L., Link M.S. et al.: Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 2003; 108(25): 3084-3091.
9. Wichter T., Paul M., Wollmann C. et al.: Implantable cardioverter/defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy: single-center experience of long-term follow-up and complications in 60 patients. Circulation 2004; 109(12): 1503-1508.
10. Corrado D., Calkins H., Link M.S. et al.: Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia. Circulation 2010; 122(12): 1144-1152.
11. Tavernier R., Gevaert S., De Sutter J. et al.: Long term results of cardioverter-defibrillator implantation in patients with right ventricular dysplasia and malignant ventricular tachyarrhythmias. Heart 2001; 85(1): 53-56.
12. Link M.S., Wang P.J., Haugh C.J.et al.: Arrhythmogenic right ventricular dysplasia: clinical results with implantable cardioverter defibrillators. Journal of interventional cardiac electrophysiology: an international journal of arrhythmias and pacing 1997; 1(1): 41-48.
13. Corrado D., Fontaine G., Marcus F.I. et al.: Arrhythmogenic right ventricular dysplasia/cardiomyopathy: need for an international registry. Study Group on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation. Circulation 2000; 101(11): E101-106.
14. Rampazzo A., Nava A., Malacrida S. et al.: Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. American Journal of Human Genetics 2002; 71(5): 1200-1206.
15. Basso C., Ronco F., Marcus F. et al.: Quantitative assessment of endomyocardial biopsy in arrhythmogenic right ventricular cardiomyopathy/dysplasia: an in vitro validation of diagnostic criteria. European Heart Journal 2008; 29(22): 2760-2771.
16. Hulot J.S., Jouven X., Empana J.P. et al.: Natural history and risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation 2004; 110(14): 1879-1884.
17. Elvas L.: ICD: effective but not without complications. Revista Portuguesa de Cardiologia 2001; 20(11): 1087-1089.
18. Gatzoulis K., Protonotarios N., Anastasakis A. et al.: Implantable defibrillator therapy in Naxos disease. Pacing and clinical electrophysiology: PACE 2000; 23(7): 1176-1178.
19. Roguin A., Bomma C.S., Nasir K. et al.: Implantable cardioverter-defibrillators in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Journal of the American College of Cardiology 2004; 43(10): 1843-1852.
20. Wichter T., Borggrefe M., Haverkamp W. et al.: Efficacy of antiarrhythmic drugs in patients with arrhythmogenic right ventricular disease. Results in patients with inducible and noninducible ventricular tachycardia. Circulation 1992; 86(1): 29-37.
21. Verma A., Kilicaslan F., Schweikert R.A. et al.: Short- and long-term success of substrate-based mapping and ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia. Circulation 2005; 111(24): 3209-3216.
22. Marchlinski F.E., 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(16): 2293-2298.
23. Reithmann C., Hahnefeld A., Remp T. et al.: Electroanatomic mapping of endocardial right ventricular activation as a guide for catheter ablation in patients with arrhythmogenic right ventricular dysplasia. Pacing and clinical electrophysiology: PACE 2003; 26(6): 1308-1316.
24. Dalal D., Jain R., Tandri H. et al.: Long-term efficacy of catheter ablation of ventricular tachycardia in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Journal of the American College of Cardiology 2007; 50(5): 432-440.
25. Philips B., Madhavan S., James C. et al.: Outcomes of catheter ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia/ cardiomyopathy. Circulation Arrhythmia and Electrophysiology 2012; 5(3): 499-505.
26. Berruezo A., Fernandez-Armenta J., Mont L. et al.: Combined endocardial and epicardial catheter ablation in arrhythmogenic right ventricular dysplasia incorporating scar dechanneling technique. Circulation Arrhythmia and Electrophysiology 2012; 5(1): 111-121.
27. Guiraudon G.M., Klein G.J., Sharma A.D. et al.: Surgical therapy for arrhythmogenic right ventricular adiposis. European Heart Journal 1989; 10(supl. D): 82-83.
28. Damiano R.J. Jr, Asano T., Smith P.K. et al.: Effect of the right ventricular isolation procedure on ventricular vulnerability to fibrillation. Journal of the American College of Cardiology 1990; 15(3): 730-736.
29. Tedford R.J., James C., Judge D.P. et al.: Cardiac transplantation in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Journal of the American College of Cardiology 2012; 59(3): 289-290.
30. Marcus F.I., Fontaine G.H., Frank R. et al.: Long-term follow-up in patients with arrhythmogenic right ventricular disease. European Heart Journal 1989; 10(supl. D): 68-73.
31. Lemery R., Brugada P., Janssen J. et al.: Nonischemic sustained ventricular tachycardia: clinical outcome in 12 patients with arrhythmogenic right ventricular dysplasia. Journal of the American College of Cardiology 1989; 14(1): 96-105.
32. Quarta G., Muir A., Pantazis A. et al.: Familial evaluation in arrhythmogenic right ventricular cardiomyopathy: impact of genetics and revised task force criteria. Circulation 2011; 123(23): 2701-2709.
33. Le Guludec D., Gauthier H., Porcher R. et al.: Prognostic value of radionuclide angiography in patients with right ventricular arrhythmias. Circulation 2001; 103(15): 1972-1976.
34. Merner N.D., Hodgkinson K.A., Haywood A.F. et al.: Arrhythmogenic right ventricular cardiomyopathy type 5 is a fully penetrant, lethal arrhythmic disorder caused by a missense mutation in the TMEM43 gene. American Journal of Human Genetics 2008; 82(4): 809-821.
35. Turrini P., Corrado D., Basso C. et al.: Dispersion of ventricular depolarization-repolarization: a noninvasive marker for risk stratification in arrhythmogenic right ventricular cardiomyopathy. Circulation 2001; 103(25): 3075-3080.
36. Protonotarios N., Tsatsopoulou A., Anastasakis A. et al.: Genotype-phenotype assessment in autosomal recessive arrhythmogenic right ventricular cardiomyopathy (Naxos disease) caused by a deletion in plakoglobin. Journal of the American College of Cardiology 2001; 38(5): 1477-1484.
2. Zipes D.P., Camm A.J., Borggrefe M. et al.: European Heart Rhythm Association, Heart Rhythm Society: ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). Journal of the American College of Cardiology 2006; 48(5): e247-346.
3. Gemayel C., Pelliccia A., Thompson P.D.: Arrhythmogenic right ventricular cardiomyopathy. Journal of the American College of Cardiology 2001; 38(7): 1773-1781.
4. Nava A., Bauce B., Basso C. et al.: Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. Journal of the American College of Cardiology 2000; 36(7): 2226-2233.
5. Maron B.J., Ackerman M.J., Nishimura R.A. et al.: Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. Journal of the American College of Cardiology 2005; 45(8): 1340-1345.
6. Dalal D., Nasir K., Bomma C. et al.: Arrhythmogenic right ventricular dysplasia: a United States experience. Circulation 2005; 112(25): 3823-3832.
7. Hodgkinson K.A., Parfrey P.S., Bassett A.S. et al.: The impact of implantable cardioverter-defibrillator therapy on survival in autosomal-dominant arrhythmogenic right ventricular cardiomyopathy (ARVD5). Journal of the American College of Cardiology 2005; 45(3): 400-408.
8. Corrado D., Leoni L., Link M.S. et al.: Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 2003; 108(25): 3084-3091.
9. Wichter T., Paul M., Wollmann C. et al.: Implantable cardioverter/defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy: single-center experience of long-term follow-up and complications in 60 patients. Circulation 2004; 109(12): 1503-1508.
10. Corrado D., Calkins H., Link M.S. et al.: Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia. Circulation 2010; 122(12): 1144-1152.
11. Tavernier R., Gevaert S., De Sutter J. et al.: Long term results of cardioverter-defibrillator implantation in patients with right ventricular dysplasia and malignant ventricular tachyarrhythmias. Heart 2001; 85(1): 53-56.
12. Link M.S., Wang P.J., Haugh C.J.et al.: Arrhythmogenic right ventricular dysplasia: clinical results with implantable cardioverter defibrillators. Journal of interventional cardiac electrophysiology: an international journal of arrhythmias and pacing 1997; 1(1): 41-48.
13. Corrado D., Fontaine G., Marcus F.I. et al.: Arrhythmogenic right ventricular dysplasia/cardiomyopathy: need for an international registry. Study Group on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation. Circulation 2000; 101(11): E101-106.
14. Rampazzo A., Nava A., Malacrida S. et al.: Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. American Journal of Human Genetics 2002; 71(5): 1200-1206.
15. Basso C., Ronco F., Marcus F. et al.: Quantitative assessment of endomyocardial biopsy in arrhythmogenic right ventricular cardiomyopathy/dysplasia: an in vitro validation of diagnostic criteria. European Heart Journal 2008; 29(22): 2760-2771.
16. Hulot J.S., Jouven X., Empana J.P. et al.: Natural history and risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation 2004; 110(14): 1879-1884.
17. Elvas L.: ICD: effective but not without complications. Revista Portuguesa de Cardiologia 2001; 20(11): 1087-1089.
18. Gatzoulis K., Protonotarios N., Anastasakis A. et al.: Implantable defibrillator therapy in Naxos disease. Pacing and clinical electrophysiology: PACE 2000; 23(7): 1176-1178.
19. Roguin A., Bomma C.S., Nasir K. et al.: Implantable cardioverter-defibrillators in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Journal of the American College of Cardiology 2004; 43(10): 1843-1852.
20. Wichter T., Borggrefe M., Haverkamp W. et al.: Efficacy of antiarrhythmic drugs in patients with arrhythmogenic right ventricular disease. Results in patients with inducible and noninducible ventricular tachycardia. Circulation 1992; 86(1): 29-37.
21. Verma A., Kilicaslan F., Schweikert R.A. et al.: Short- and long-term success of substrate-based mapping and ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia. Circulation 2005; 111(24): 3209-3216.
22. Marchlinski F.E., 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(16): 2293-2298.
23. Reithmann C., Hahnefeld A., Remp T. et al.: Electroanatomic mapping of endocardial right ventricular activation as a guide for catheter ablation in patients with arrhythmogenic right ventricular dysplasia. Pacing and clinical electrophysiology: PACE 2003; 26(6): 1308-1316.
24. Dalal D., Jain R., Tandri H. et al.: Long-term efficacy of catheter ablation of ventricular tachycardia in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Journal of the American College of Cardiology 2007; 50(5): 432-440.
25. Philips B., Madhavan S., James C. et al.: Outcomes of catheter ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia/ cardiomyopathy. Circulation Arrhythmia and Electrophysiology 2012; 5(3): 499-505.
26. Berruezo A., Fernandez-Armenta J., Mont L. et al.: Combined endocardial and epicardial catheter ablation in arrhythmogenic right ventricular dysplasia incorporating scar dechanneling technique. Circulation Arrhythmia and Electrophysiology 2012; 5(1): 111-121.
27. Guiraudon G.M., Klein G.J., Sharma A.D. et al.: Surgical therapy for arrhythmogenic right ventricular adiposis. European Heart Journal 1989; 10(supl. D): 82-83.
28. Damiano R.J. Jr, Asano T., Smith P.K. et al.: Effect of the right ventricular isolation procedure on ventricular vulnerability to fibrillation. Journal of the American College of Cardiology 1990; 15(3): 730-736.
29. Tedford R.J., James C., Judge D.P. et al.: Cardiac transplantation in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Journal of the American College of Cardiology 2012; 59(3): 289-290.
30. Marcus F.I., Fontaine G.H., Frank R. et al.: Long-term follow-up in patients with arrhythmogenic right ventricular disease. European Heart Journal 1989; 10(supl. D): 68-73.
31. Lemery R., Brugada P., Janssen J. et al.: Nonischemic sustained ventricular tachycardia: clinical outcome in 12 patients with arrhythmogenic right ventricular dysplasia. Journal of the American College of Cardiology 1989; 14(1): 96-105.
32. Quarta G., Muir A., Pantazis A. et al.: Familial evaluation in arrhythmogenic right ventricular cardiomyopathy: impact of genetics and revised task force criteria. Circulation 2011; 123(23): 2701-2709.
33. Le Guludec D., Gauthier H., Porcher R. et al.: Prognostic value of radionuclide angiography in patients with right ventricular arrhythmias. Circulation 2001; 103(15): 1972-1976.
34. Merner N.D., Hodgkinson K.A., Haywood A.F. et al.: Arrhythmogenic right ventricular cardiomyopathy type 5 is a fully penetrant, lethal arrhythmic disorder caused by a missense mutation in the TMEM43 gene. American Journal of Human Genetics 2008; 82(4): 809-821.
35. Turrini P., Corrado D., Basso C. et al.: Dispersion of ventricular depolarization-repolarization: a noninvasive marker for risk stratification in arrhythmogenic right ventricular cardiomyopathy. Circulation 2001; 103(25): 3075-3080.
36. Protonotarios N., Tsatsopoulou A., Anastasakis A. et al.: Genotype-phenotype assessment in autosomal recessive arrhythmogenic right ventricular cardiomyopathy (Naxos disease) caused by a deletion in plakoglobin. Journal of the American College of Cardiology 2001; 38(5): 1477-1484.