Published online before print September 27, 2004, doi: 10.1161/01.CIR.0000143375.93288.82
| |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
(Circulation. 2004;110:1879-1884.)
© 2004 American Heart Association, Inc.
Arrhythmia/Electrophysiology |
Natural History and Risk Stratification of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
From INSERM Avenir & U252 (J.-S.H., X.J., J.-P.E., G.F.), Department of Pharmacology (J.-S.H.) and Department of Cardiology, Cardiac Arrhythmias Department (R.F., G.F.), Pitié-Salpêtrière University Hospital, Assistance-Publique Hôpitaux de Paris; and Department of Cardiology, Hôpital Européen Georges Pompidou, University Paris 5 (X.J., J.-P.E.), Paris, France.
Correspondence to Dr Xavier Jouven, Department of Cardiology, Hôpital Européen Georges Pompidou, 20 Rue Leblanc, 75015 Paris, France. E-mail xavier.jouven{at}hop.egp.ap-hop-paris.fr
Received March 8, 2004; revision received June 1, 2004; accepted June 3, 2004.
Background— Management of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is complicated by the incomplete information on the natural history of the disease and by the lack of risk stratification for cardiovascular death. The aim of the study was the identification of risk factors related to long-term prognosis.
Methods and Results— Data were collected from 130 patients (100 men; age at onset of symptoms, 31.8±14.4 years) from a tertiary center between 1977 and 2000 who fulfilled the international standardized diagnostic criteria for ARVD/C. Risk factors for cardiovascular death were determined by a logistic regression model. After a mean follow-up of 8.1±7.8 years, 24 deaths were recorded, with a mean age at death of 54±19 years (annual mortality rate, 2.3%). There were 21 deaths with a cardiovascular origin (progressive heart failure for 14 patients and sudden death for the remaining 7 patients). All patients who died had a history of ventricular tachycardia. Multivariate analysis showed that after adjustment for sex, history of syncope, chest pain, inaugural ventricular tachycardia, recurrence of ventricular tachycardia, and QRS dispersion, clinical signs of right ventricular failure and left ventricular dysfunction both remained independently associated with cardiovascular mortality. The combined presence of one of these risk factors and ventricular tachycardia identifies high-risk subjects for cardiovascular mortality, whereas patients without ventricular tachycardia displayed the best prognosis.
Conclusions— The information on the natural history of patients with ARVD allowed us to identify risks factors for cardiovascular mortality. An analysis of a large international registry is needed to refine these results.
Key Words: cardiomyopathy • death, sudden • heart failure • risk factors
This article has been cited by other articles:
 |
|
 |
|
  A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons J. Am. Coll. Cardiol., May 27, 2008; 51(21): e1 - e62. [Full Text] [PDF]
|
|
|
|
 |
|
 A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons Circulation, May 27, 2008; 117(21): e350 - e408. [Full Text] [PDF]
|
|
|
|
|
|
F. Haddad, R. Doyle, D. J. Murphy, and S. A. Hunt Right Ventricular Function in Cardiovascular Disease, Part II: Pathophysiology, Clinical Importance, and Management of Right Ventricular Failure Circulation, April 1, 2008; 117(13): 1717 - 1731. [Full Text] [PDF]
|
|
|
|
 |
|
 L. T. Cooper, K. L. Baughman, A. M. Feldman, A. Frustaci, M. Jessup, U. Kuhl, G. N. Levine, J. Narula, R. C. Starling, J. Towbin, et al. The role of endomyocardial biopsy in the management of cardiovascular disease: A Scientific Statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology Eur. Heart J., December 2, 2007; 28(24): 3076 - 3093. [Full Text] [PDF]
|
|
|
|
|
|
L. T. Cooper, K. L. Baughman, A. M. Feldman, A. Frustaci, M. Jessup, U. Kuhl, G. N. Levine, J. Narula, R. C. Starling, J. Towbin, et al. The Role of Endomyocardial Biopsy in the Management of Cardiovascular Disease: A Scientific Statement From the American Heart Association, the American College of Cardiology, and the European Society of Cardiology Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology J. Am. Coll. Cardiol., November 6, 2007; 50(19): 1914 - 1931. [Full Text] [PDF]
|
|
|
|
|
|
L. T. Cooper, K. L. Baughman, A. M. Feldman, A. Frustaci, M. Jessup, U. Kuhl, G. N. Levine, J. Narula, R. C. Starling, J. Towbin, et al. The Role of Endomyocardial Biopsy in the Management of Cardiovascular Disease: A Scientific Statement From the American Heart Association, the American College of Cardiology, and the European Society of Cardiology Circulation, November 6, 2007; 116(19): 2216 - 2233. [Full Text] [PDF]
|
|
|
|
 |
|
 D. Mariano-Goulart, L. Dechaux, F. Rouzet, E. Barbotte, C. Caderas de Kerleau, M. Rossi, and D. Le Guludec Diagnosis of Diffuse and Localized Arrhythmogenic Right Ventricular Dysplasia by Gated Blood-Pool SPECT J. Nucl. Med., September 1, 2007; 48(9): 1416 - 1423. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. A. Stephenson and C. I. Berul Electrophysiological Interventions for Inherited Arrhythmia Syndromes Circulation, August 28, 2007; 116(9): 1062 - 1080. [Full Text] [PDF]
|
|
|
|
|
|
D. Dalal, R. Jain, H. Tandri, J. Dong, S. M. Eid, K. Prakasa, C. Tichnell, C. James, T. Abraham, S. D. Russell, et al. Long-Term Efficacy of Catheter Ablation of Ventricular Tachycardia in Patients With Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy J. Am. Coll. Cardiol., July 31, 2007; 50(5): 432 - 440. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Antoniades, A. Tsatsopoulou, A. Anastasakis, P. Syrris, A. Asimaki, D. Panagiotakos, C. Zambartas, C. Stefanadis, W. J. McKenna, and N. Protonotarios Arrhythmogenic right ventricular cardiomyopathy caused by deletions in plakophilin-2 and plakoglobin (Naxos disease) in families from Greece and Cyprus: genotype-phenotype relations, diagnostic features and prognosis Eur. Heart J., September 2, 2006; 27(18): 2208 - 2216. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Stern Electrocardiogram: Still the Cardiologist's Best Friend Circulation, May 16, 2006; 113(19): e753 - e756. [Full Text] [PDF]
|
|
|
|
|
|
D. Dalal, L. H. Molin, J. Piccini, C. Tichnell, C. James, C. Bomma, K. Prakasa, J. A. Towbin, F. I. Marcus, P. J. Spevak, et al. Clinical Features of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Associated With Mutations in Plakophilin-2 Circulation, April 4, 2006; 113(13): 1641 - 1649. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J.P. van Tintelen, M. M. Entius, Z. A. Bhuiyan, R. Jongbloed, A. C.P. Wiesfeld, A. A.M. Wilde, J. van der Smagt, L. G. Boven, M. M.A.M. Mannens, I. M. van Langen, et al. Plakophilin-2 Mutations Are the Major Determinant of Familial Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Circulation, April 4, 2006; 113(13): 1650 - 1658. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Dalal, K. Nasir, C. Bomma, K. Prakasa, H. Tandri, J. Piccini, A. Roguin, C. Tichnell, C. James, S. D. Russell, et al. Arrhythmogenic Right Ventricular Dysplasia: A United States Experience Circulation, December 20, 2005; 112(25): 3823 - 3832. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Verma, F. Kilicaslan, R. A. Schweikert, G. Tomassoni, A. Rossillo, N. F. Marrouche, V. Ozduran, O. M. Wazni, S. C. Elayi, L. C. Saenz, et al. Short- and Long-Term Success of Substrate-Based Mapping and Ablation of Ventricular Tachycardia in Arrhythmogenic Right Ventricular Dysplasia Circulation, June 21, 2005; 111(24): 3209 - 3216. [Abstract] [Full Text] [PDF]
|
|