Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study

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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				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]

				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]

				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]

				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]

				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]

				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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				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]

				M J Davies CARDIOMYOPATHY: The cardiomyopathies: an overview Heart, April 1, 2000; 83(4): 469 - 474. [Full Text]

				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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				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 J. Am. Coll. Cardiol., December 1, 1999; 34(7): 2035 - 2042. [Abstract] [Full Text] [PDF]

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