Apr 12, 2018

CHFR workshop on Arrhythmogenic Cardiomyopathy


CHFR workshop on Arrhythmogenic Cardiomyopathy

Arrhythmogenic cardiomyopathy (AC) is an inheritable heart disease associated with high risk of life-threatening ventricular arrhythmias and sudden cardiac death. The AC-causing mutations can be identified in family members of AC-patients, and risk stratification and recognition of early disease in these at-risk individuals are major research tasks.

The Department of Cardiology, Oslo University Hospital, Rikshospitalet, handles one of the largest AC-cohorts in the world, and the research conducted at the Center for Cardiological Innovation (CCI) has contributed to improved patient care worldwide.

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The research group at Utrecht University Hospital, The Netherlands, is another major contributor to the scientific field. On the 4th of April 2018, key researchers from the Dutch group joined key researchers from Oslo to an open AC workshop with the support of the Center for Heart Failure Research and Norheart. Dr. Arco Teske of Utrecht University Hospital has a strong track record of AC-research. He has contributed considerably to the current knowledge in the value of modern cardiac imaging in characterization of AC-patients.

Dr. Teske and Dr. Joost Lumens of the University of Maastricht, The Netherlands, were pivotal in the development and validation of a novel echocardiographic strain pattern algorithm. This method was published in JACC in 2016 (1), and has the potential to recognize AC mutation carriers with very early signs of disease (2). Dr. Lumens is a world-renowned cardiac researcher and specialist of computational modeling, whose expertise spans from cardiac dyssynchrony via athletic adaptation to AC. These two invited speakers held inspiring talks of the development and research process, as well highlighting the possibilities for future scientific progress.

Dr. Christine Rootwelt-Norberg of the CCI gave a strong presentation on the clinical presentation of Norwegian AC-patients before and after the implementation of genetic screening, highlighting the major clinical progress made during the last decade. Still, life-threatening ventricular arrhythmias are highly prevalent, but most patients experience possible warning symptoms before the events.

Dr. Mathis K. Stokke of the CCI and Department of Cardiology presented an important study from the Nordic AC-registry challenging the common assumption that electrical signs of disease precede structural or functional manifestation. This is an important message, as many centers in the world handle AC-patients strictly by electrophysiological methods, instead of by an integrated approach with cardiac imaging.

Feddo Kirkels is a talented young medical student and researcher from Utrecht who spent 2 months as a guest researcher at the CCI this winter. He presented his research that has been dedicated to comparing the Dutch strain pattern algorithm with the pioneering Norwegian method of assessing right ventricular mechanical dispersion by calculation of the standard deviation of the time intervals from electrical activation to peak myocardial shortening in 6 individual right ventricular segments (3, 4).

Associate Professor Kristina H. Haugaa at the CCI and Department of Cardiology recently published an editorial together with Dr. Øyvind H. Lie in JACC Cardiovascular Imaging, in which they highlighted the need for such head-to-head comparisons and international collaboration (5). Øyvind H. Lie also provided an overlook of the ongoing AC-research in the Oslo group, and discussion of further collaboration and innovation ensued. The workshop was a scientific success that hopefully will yield advances in research and improved patient care in years to come.



1. Mast TP, Teske AJ, Walmsley J, van der Heijden JF, van Es R, Prinzen FW, Delhaas T, van Veen TA, Loh P, Doevendans PA, Cramer MJ, Lumens J
Right Ventricular Imaging and Computer Simulation for Electromechanical Substrate Characterization in Arrhythmogenic Right Ventricular Cardiomyopathy.
Journal of the American College of Cardiology. 2016;68:2185-2197

2. Mast TP, Taha K, Cramer MJ, Lumens J, van der Heijden JF, Bouma BJ, van den Berg MP, Asselbergs FW, Doevendans PA, Teske AJ
The prognostic value of right ventricular deformation imaging in early arrhythmogenic right ventricular cardiomyopathy.
JACC. Cardiovascular imaging. 2018

3. Leren IS, Saberniak J, Haland TF, Edvardsen T, Haugaa KH
Combination of ecg and echocardiography for identification of arrhythmic events in early arvc.
J Am Coll Cardiol Img. 2017;10:503-513

4. Sarvari SI, Haugaa KH, Anfinsen OG, Leren TP, Smiseth OA, Kongsgaard E, Amlie JP, Edvardsen T
Right ventricular mechanical dispersion is related to malignant arrhythmias: A study of patients with arrhythmogenic right ventricular cardiomyopathy and subclinical right ventricular dysfunction.
Eur Heart J. 2011;32:1089-1096

5. Haugaa KH, Lie OH.
Reveal the concealed: The quest for early disease detection in family members at risk of developing arrhythmogenic cardiomyopathy.
JACC. Cardiovascular imaging. 2018 Mar 12

Center for Cardiological Innovation