Copyright © 2004, European Society of Cardiology
Arrhythmogenic right ventricular dysplasia type 1 and mutations in transforming growth factor β3 gene regulatory regions: a breakthrough?
aResearch Center and Department of Medicine, Montreal Heart Institute and University of Montreal, 5000 Belanger St. E., Montreal, Quebec, Canada H1T 1C8
bINSERM U-533 and l'Institut du Thorax, University of Nantes, Nantes, France
* Corresponding author. Tel.: +1 514 376 3330; fax: +1 514 376 1355. Email address: stanley.nattel@icm-mhi.org
Received 16 November 2004; accepted 18 November 2004
| The first 10% of the full text of this article appears below. |
See article by Beffagna et al. (pages 366–373) in this issue.
Arrhythmogenic right ventricular dysplasia (ARVD) is the second most common cause of sudden unexpected death in young adults [1], responsible for
20% of sudden deaths in this population [2]. Subsequent to the description of the first case in 1961 [3], great progress has been made in the diagnosis, evaluation and management of the condition. The hallmark of ARVD is right ventricular atrophy with fibro-fatty infiltration. There is approximately threefold male predominance, and typical abnormalities include signs of right ventricular thinning and dysfunction and fragmented, low-amplitude
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