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Arrhythmogenic right ventricular cardiomyopathy

Debate session

Non-Invasive Imaging

EuroCMR 2014 was rewarded to a particularly informational perspective on the role of CMR in diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC). The unusual Pro and Con debate had both sides of the debate presented by Professor William J. McKenna, MD from the Heart Hospital and Great Ormond Street Hospital, University College London.  Having been a key expert in working groups that developed the diagnostic criteria for ARVC in 1994 and revised the criteria in 2010 (McKenna WJ et al Br Heart J. 1994 Mar;71(3):215-8, Marcus FI et al European Heart Journal (2010) 31, 806–814), Professor McKenna was a perfect selection for the session.


    The Con side of the debate started with a perspective on the clinical and pathophysiological features of ARVC. Professor McKenna highlighted some of the differences between the 1994 and 2010 diagnostic criteria. In particular, he noted that the role of imaging is now focused primarily on right ventricular structure and function. The new guidelines specifically avoid including tissue characterization by CMR leaving this territory entirely to endomyocardial biopsy and autopsy. These changes were based primarily on experience from the US where CMR showed poor ability to diagnostically differentiate ARVC from normal and other cardiomyopathies.


    The Pro side of the debate started with an assertion that the current diagnostic criteria greatly underestimate the clinical value of ate gadolinium enhancement (LGE) imaging. The complexity of interpreting genetics and phenotype in young patients was contrasted with published experience highlighting the predominance of sudden cardiac deaths in probands between 11 and 40 years of age.  The transient nature of electrical features of ARVC can complicate early risk stratification of family members. In discussion of the genetics, he pointed out that ARVC is a disease of the desmosome. Professor McKenna concluded that the guidelines are already out of date and that ARVC is incorrectly named and should be diagnosed as Arrhythmogenic Cardiomyopathy since ventricular involvement is common on both sides of the heart.


Arrhythmogenic right ventricular cardiomyopathy
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.