Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study
D Corrado,
C Basso,
G Thiene,
WJ McKenna,
MJ Davies,
F Fontaliran,
A Nava,
F Silvestri,
C Blomstrom-Lundqvist,
EK Wlodarska,
G Fontaine,
and
F Camerini
University of Padua Medical Center, Italy. cardpath@uxl.unipd.it
OBJECTIVES: The aim of the present investigation was to redefine the clinicopathologic profile of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC), with special reference to disease progression and left ventricular (LV) involvement. BACKGROUND: Long-term follow-up data from clinical studies indicate that ARVC is a progressive heart muscle disease that with time may lead to more diffuse right ventricular (RV) involvement and LV abnormalities and culminate in heart failure. METHODS: Forty-two patients (27 male, 15 female; 9 to 65 years old, mean [+/-SD] age 29.6 +/- 18) from six collaborative medical centers, with a pathologic diagnosis of ARVC at autopsy or heart transplantation, and with the whole heart available, were studied according to a specific clinicomorphologic protocol. RESULTS: Thirty-four patients died suddenly (16 during effort); 4 underwent heart transplantation; 2 died as a result of advanced heart failure; and 2 died of other causes. Sudden death was the first sign of disease in 12 patients; the other 30 had palpitations, with syncope in 11, heart failure in 8 and stroke in 3. Twenty-seven patients experienced ventricular arrhythmias (ventricular tachycardia in 17), and 5 received a pacemaker. Ten patients had isolated RV involvement (group A); the remaining 32 (76%) also had fibrofatty LV involvement that was observed histologically only in 15 (group B) and histologically and macroscopically in 17 (group C). Patients in group C were significantly older than those in groups A and B (39 +/- 15 years vs. 20 +/- 8.8 and 25 +/- 9.7 years, respectively), had significantly longer clinical follow-up (9.3 +/- 7.3 years vs. 1.2 +/- 2.1 and 3.4 +/- 2.2 years, respectively) and developed heart failure significantly more often (47% vs. 0 and 0, respectively). Patients in groups B and C had warning symptoms (80% and 87%, respectively, vs. 30%) and clinical ventricular arrhythmias (73% and 82%, respectively, vs. 20%) significantly more often than patients in group A. Hearts from patients in group C weighed significantly more than those from patients in groups A and B (500 +/- 150 g vs. 328 +/- 40 and 380 +/- 95 g, respectively), whereas hearts from both group B and C patients had severe RV thinning (87% and 71%, respectively, vs. 20%) and inflammatory infiltrates (73% and 88%, respectively, vs. 30%) significantly more often than those from group A patients. CONCLUSIONS: LV involvement was found in 76% of hearts with ARVC, was age dependent and was associated with clinical arrhythmic events, more severe cardiomegaly, inflammatory infiltrates and heart failure. ARVC can no longer be regarded as an isolated disease of the right ventricle.
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Circumstances of Death and Gross and Microscopic Observations in a Series of 200 Cases of Sudden Death Associated With Arrhythmogenic Right Ventricular Cardiomyopathy and/or Dysplasia
Circulation,
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[Abstract]
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D. Corrado, C. Basso, G. Rizzoli, M. Schiavon, and G. Thiene
Does sports activity enhance the risk of sudden death in adolescents and young adults?
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[Abstract]
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E. S. Antezano and M. Hong
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[Abstract]
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P. Motta, E. Mossad, and R. Savage
Right Ventricular Exclusion Surgery for Arrhythmogenic Right Ventricular Dysplasia with Cardiomyopathy
Anesth. Analg.,
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[Abstract]
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D. O'Donnell, D. Cox, J. Bourke, L. Mitchell, and S. Furniss
Clinical and electrophysiological differences between patients with arrhythmogenic right ventricular dysplasia and right ventricular outflow tract tachycardia
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J. C. Chachques, P. G. Argyriadis, G. Fontaine, J.-L. Hebert, R. A. Frank, N. D'Attellis, J.-N.o. Fabiani, and A. F. Carpentier
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Ann. Thorac. Surg.,
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[Abstract]
[Full Text]
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G. Aviram, J. E. Fishman, M.-L. Young, E. Redha, G. Biliciler-Denktas, and M. M. Rodriguez
MR Evaluation of Arrhythmogenic Right Ventricular Cardiomyopathy in Pediatric Patients
Am. J. Roentgenol.,
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[Abstract]
[Full Text]
[PDF]
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D. Fatkin and R. M. Graham
Molecular Mechanisms of Inherited Cardiomyopathies
Physiol Rev,
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[Abstract]
[Full Text]
[PDF]
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F. Kimura, F. Sakai, Y. Sakomura, M. Fujimura, E. Ueno, N. Matsuda, H. Kasanuki, and N. Mitsuhashi
Helical CT Features of Arrhythmogenic Right Ventricular Cardiomyopathy
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[Abstract]
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[PDF]
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S. Sano, K. Ishino, M. Kawada, S. Kasahara, T. Kohmoto, M. Takeuchi, and S.-i. Ohtsuki
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N. E. Bowles, J. Ni, F. Marcus, and J. A. Towbin
The detection of cardiotropic viruses in the myocardium of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy
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[Abstract]
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X.H.T. WEHRENS and A.R. MARKS
Myocardial Disease in Failing Hearts: Defective Excitation-Contraction Coupling
Cold Spring Harb Symp Quant Biol,
January 1, 2002;
67(0):
533 - 542.
[Abstract]
[PDF]
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S. G. Priori, E. Aliot, C. Blomstrom-Lundqvist, L. Bossaert, G. Breithardt, P. Brugada, J. A. Camm, R. Cappato, S. M. Cobbe, C. Di Mario, et al.
TASK FORCE ON SUDDEN CARDIAC DEATH, EUROPEAN SOCIETY OF CARDIOLOGY: Summary of Recommendations
Europace,
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[Abstract]
[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,
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[Abstract]
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C. Gemayel, A. Pelliccia, and P. D. Thompson
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M. Boulos, I. Lashevsky, S. Reisner, and L. Gepstein
Electroanatomic mapping of arrhythmogenic right ventricular dysplasia
J. Am. Coll. Cardiol.,
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[Abstract]
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[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;
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[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
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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
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August 1, 2001;
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[Abstract]
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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):
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[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):
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[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
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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
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[Abstract]
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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;
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[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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D. Corrado, C. Basso, and G. Thiene
CARDIOMYOPATHY: Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment
Heart,
May 1, 2000;
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[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):
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[Abstract]
[Full Text]
[PDF]
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M J Davies
CARDIOMYOPATHY: The cardiomyopathies: an overview
Heart,
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[Full Text]
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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;
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[Abstract]
[Full Text]
[PDF]
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J. D. Fisher, D. Krikler, and K. A. Hallidie-Smith
Familial polymorphic ventricular arrhythmias: A quarter century of successful medical treatment based on serial exercise-pharmacologic testing
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December 1, 1999;
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[Abstract]
[Full Text]
[PDF]
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H. Swan, K. Piippo, M. Viitasalo, P.a. Heikkila, T. Paavonen, K. Kainulainen, J. Kere, P. Keto, K. Kontula, and L. Toivonen
Arrhythmic disorder mapped to chromosome 1q42-q43 causes malignant polymorphic ventricular tachycardia in structurally normal hearts
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[Abstract]
[Full Text]
[PDF]
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G. Junga, S. Kneifel, A.V. Smekal, H. Steinert, and U. Bauersfeld
Myocardial ischaemia in children with isolated ventricular non-compaction
Eur. Heart J.,
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[Abstract]
[PDF]
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D. P. Zipes and H. J. J. Wellens
Sudden Cardiac Death
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November 24, 1998;
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[Full Text]
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