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Circulation, Vol 65, 384-398, Copyright © 1982 by American Heart Association
FI Marcus, GH Fontaine, G Guiraudon, R Frank, JL Laurenceau, C Malergue and Y Grosgogeat
Right ventricular dysplasia is characterized by an abnormality in the
development of part of the right ventricular musculature. Patients with
right ventricular dysplasia may present with ventricular tachycardia,
supraventricular arrhythmias, right-heart failure or asymptomatic
cardiomegaly. Twenty-two adult patients with right ventricular dysplasia
who had recurrent ventricular tachycardia were seen during a 7-year period.
The male/female ratio was 2.7:1. The mean age at the time of
hospitalization was 39 years. All but one of the patients had ventricular
tachycardia of a left bundle branch block configuration. With few
exceptions, the T waves were inverted over the right precordial leads. The
heart was usually enlarged and the pulmonary vasculature was usually
normal. In six patients who had two-dimensional echocardiograms, all showed
increased right ventricular diastolic dimensions. All patients had right
ventricular angiography; the diagnosis of right ventricular dysplasia was
substantiated during surgery in 12 patients and at autopsy in another. Two
other patients who did not have arrhythmias had right ventricular dysplasia
diagnosed by right- and left-heart angiography. Our unique experience, when
combined with a literature review of 34 adult cases, permits a composite
clinical profile of this condition in the adult.
ARTICLES
Right ventricular dysplasia: a report of 24 adult cases
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S. Sano, K. Ishino, M. Kawada, S. Kasahara, T. Kohmoto, M. Takeuchi, and S.-i. Ohtsuki Total right ventricular exclusion procedure: An operation for isolated congestive right ventricular failure J. Thorac. Cardiovasc. Surg., April 1, 2002; 123(4): 640 - 647. [Abstract] [Full Text] [PDF]
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F Niroomand, C Carbucicchio, C Tondo, S Riva, G Fassini, A Apostolo, N Trevisi, and P Della Bella Electrophysiological characteristics and outcome in patients with idiopathic right ventricular arrhythmia compared with arrhythmogenic right ventricular dysplasia Heart, January 1, 2002; 87(1): 41 - 47. [Abstract] [Full Text] [PDF]
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C. Gemayel, A. Pelliccia, and P. D. Thompson Arrhythmogenic right ventricular cardiomyopathy J. Am. Coll. Cardiol., December 1, 2001; 38(7): 1773 - 1781. [Abstract] [Full Text] [PDF]
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M. Boulos, I. Lashevsky, S. Reisner, and L. Gepstein Electroanatomic mapping of arrhythmogenic right ventricular dysplasia J. Am. Coll. Cardiol., December 1, 2001; 38(7): 2020 - 2027. [Abstract] [Full Text] [PDF]
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N. Protonotarios, A. Tsatsopoulou, A. Anastasakis, E. Sevdalis, G. McKoy, K. Stratos, K. Gatzoulis, K. Tentolouris, C. Spiliopoulou, D. Panagiotakos, et al. Genotype-phenotype assessment in autosomal recessive arrhythmogenic right ventricular cardiomyopathy (Naxos disease) caused by a deletion in plakoglobin J. Am. Coll. Cardiol., November 1, 2001; 38(5): 1477 - 1484. [Abstract] [Full Text] [PDF]
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S.G. Priori, E. Aliot, C. Blomstrom-Lundqvist, L. Bossaert, G. Breithardt, P. Brugada, A.J. Camm, R. Cappato, S.M. Cobbe, C. Di Mario, et al. Task Force on Sudden Cardiac Death of the European Society of Cardiology Eur. Heart J., August 2, 2001; 22(16): 1374 - 1450. [PDF]
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F. Gaita, C. Giustetto, P. Di Donna, E. Richiardi, L. Libero, M. C. R. Brusin, G. Molinari, and G. Trevi Long-term follow-up of right ventricular monomorphic extrasystoles J. Am. Coll. Cardiol., August 1, 2001; 38(2): 364 - 370. [Abstract] [Full Text] [PDF]
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L Lindstrom, U M Wilkenshoff, H Larsson, and B Wranne Echocardiographic assessment of arrhythmogenic right ventricular cardiomyopathy Heart, July 1, 2001; 86(1): 31 - 38. [Abstract] [Full Text] [PDF]
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P. Turrini, D. Corrado, C. Basso, A. Nava, B. Bauce, and G. Thiene Dispersion of Ventricular Depolarization-Repolarization : A Noninvasive Marker for Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy Circulation, June 26, 2001; 103(25): 3075 - 3080. [Abstract] [Full Text] [PDF]
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D. Corrado, C. Basso, and G. Thiene Sudden cardiac death in young people with apparently normal heart Cardiovasc Res, May 1, 2001; 50(2): 399 - 408. [Abstract] [Full Text] [PDF]
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D. Le Guludec, H. Gauthier, R. Porcher, R. Frank, D. Daou, S. Benelhadj, A. Leenhardt, T. Lavergne, M. Faraggi, and M. S. Slama Prognostic Value of Radionuclide Angiography in Patients With Right Ventricular Arrhythmias Circulation, April 17, 2001; 103(15): 1972 - 1976. [Abstract] [Full Text] [PDF]
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R. J. Verdino, F. E. Marchlinski, F. J. Fernandez-Fernandez, P. Sesma, and W. N. Kapoor Syncope N. Engl. J. Med., April 5, 2001; 344(14): 1098 - 1099. [Full Text] [PDF]
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A. Nava, B. Bauce, C. Basso, M. Muriago, A. Rampazzo, C. Villanova, L. Daliento, G. Buja, D. Corrado, G. A. Danieli, et al. Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy J. Am. Coll. Cardiol., December 1, 2000; 36(7): 2226 - 2233. [Abstract] [Full Text] [PDF]
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M. Eguchi, K. Tsuchihashi, T. Nakata, A. Hashimoto, and K. Shimamoto Right ventricular abnormalities assessed by myocardial single-photon emission computed tomography using technetium-99m sestamibi/tetrofosmin in right ventricle-originated ventricular tachyarrhythmias J. Am. Coll. Cardiol., November 15, 2000; 36(6): 1767 - 1773. [Abstract] [Full Text] [PDF]
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P. R. Fox, B. J. Maron, C. Basso, S.-K. Liu, and G. Thiene Spontaneously Occurring Arrhythmogenic Right Ventricular Cardiomyopathy in the Domestic Cat : A New Animal Model Similar to the Human Disease Circulation, October 10, 2000; 102(15): 1863 - 1870. [Abstract] [Full Text] [PDF]
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X. Jouven, M. Zureik, M. Desnos, D. Courbon, and P. Ducimetiere Long-Term Outcome in Asymptomatic Men with Exercise-Induced Premature Ventricular Depolarizations N. Engl. J. Med., September 21, 2000; 343(12): 826 - 833. [Abstract] [Full Text] [PDF]
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H. Calkins Premature Ventricular Depolarizations during Exercise N. Engl. J. Med., September 21, 2000; 343(12): 879 - 880. [Full Text]
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W. G. Stevenson and H. T. Aretz Case 20-2000- A 61-Year-Old Man with a Wide-Complex Tachycardia N. Engl. J. Med., June 29, 2000; 342(26): 1979 - 1987. [Full Text] [PDF]
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D. Corrado, C. Basso, and G. Thiene CARDIOMYOPATHY: Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment Heart, May 1, 2000; 83(5): 588 - 595. [Full Text]
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T. Wichter, M. Schafers, C. G. Rhodes, M. Borggrefe, H. Lerch, A. A. Lammertsma, F. Hermansen, O. Schober, G. Breithardt, and P. G. Camici Abnormalities of Cardiac Sympathetic Innervation in Arrhythmogenic Right Ventricular Cardiomyopathy : Quantitative Assessment of Presynaptic Norepinephrine Reuptake and Postsynaptic {beta}-Adrenergic Receptor Density With Positron Emission Tomography Circulation, April 4, 2000; 101(13): 1552 - 1558. [Abstract] [Full Text] [PDF]
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D. Corrado, G. Fontaine, F. I. Marcus, W. J. McKenna, A. Nava, G. Thiene, and T. Wichter Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy : Need for an International Registry Circulation, March 21, 2000; 101 (11): e101 - e106. [Abstract] [Full Text] [PDF]
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A Nava, A.F Folino, B Bauce, P Turrini, G.F Buja, L Daliento, and G Thiene Signal-averaged electrocardiogram in patients with arrhythmogenic right ventricular cardiomyopathy and ventricular arrhythmias Eur. Heart J., January 1, 2000; 21(1): 58 - 65. [Abstract] [PDF]
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K. Matsuo, T. Nishikimi, C. Yutani, T. Kurita, W. Shimizu, A. Taguchi, K. Suyama, N. Aihara, S. Kamakura, K. Kangawa, et al. Diagnostic Value of Plasma Levels of Brain Natriuretic Peptide in Arrhythmogenic Right Ventricular Dysplasia Circulation, December 1, 1998; 98(22): 2433 - 2440. [Abstract] [Full Text] [PDF]
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B. Pinamonti, L. Pagnan, R. Bussani, C. Ricci, F. Silvestri, and F. Camerini Right Ventricular Dysplasia With Biventricular Involvement Circulation, November 3, 1998; 98(18): 1943 - 1945. [Full Text] [PDF]
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A. S. Coonar, N. Protonotarios, A. Tsatsopoulou, E. W.A. Needham, R. S. Houlston, S. Cliff, M. I. Otter, V. A. Murday, R. K. Mattu, and W. J. McKenna Gene for Arrhythmogenic Right Ventricular Cardiomyopathy With Diffuse Nonepidermolytic Palmoplantar Keratoderma and Woolly Hair (Naxos Disease) Maps to 17q21 Circulation, May 26, 1998; 97(20): 2049 - 2058. [Abstract] [Full Text] [PDF]
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G. Fontaine, F. Fontaliran, and R. Frank Arrhythmogenic Right Ventricular Cardiomyopathies : Clinical Forms and Main Differential Diagnoses Circulation, April 28, 1998; 97(16): 1532 - 1535. [Full Text] [PDF]
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