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. 2019 Oct;27(10):480-486.
doi: 10.1007/s12471-019-1270-1.

The Netherlands Arrhythmogenic Cardiomyopathy Registry: design and status update

L P Bosman  1 , T E Verstraelen  2 , F H M van Lint  3 , M G P J Cox  4 , J A Groeneweg  4 , T P Mast  4 , P A van der Zwaag  5 , P G A Volders  6 , R Evertz  7 , L Wong  8 , N M S de Groot  9 , K Zeppenfeld  10 , J F van der Heijden  4 , M P van den Berg  11 , A A M Wilde  2 , F W Asselbergs  1 , R N W Hauer  1 , A S J M Te Riele  12 , J P van Tintelen  12 ; Netherlands ACM Registry
Collaborators, Affiliations

The Netherlands Arrhythmogenic Cardiomyopathy Registry: design and status update

L P Bosman et al. Neth Heart J. 2019 Oct.

Abstract

Background: Clinical research on arrhythmogenic cardiomyopathy (ACM) is typically limited by small patient numbers, retrospective study designs, and inconsistent definitions.

Aim: To create a large national ACM patient cohort with a vast amount of uniformly collected high-quality data that is readily available for future research.

Methods: This is a multicentre, longitudinal, observational cohort study that includes (1) patients with a definite ACM diagnosis, (2) at-risk relatives of ACM patients, and (3) ACM-associated mutation carriers. At baseline and every follow-up visit, a medical history as well information regarding (non-)invasive tests is collected (e. g. electrocardiograms, Holter recordings, imaging and electrophysiological studies, pathology reports, etc.). Outcome data include (non-)sustained ventricular and atrial arrhythmias, heart failure, and (cardiac) death. Data are collected on a research electronic data capture (REDCap) platform in which every participating centre has its own restricted data access group, thus empowering local studies while facilitating data sharing.

Discussion: The Netherlands ACM Registry is a national observational cohort study of ACM patients and relatives. Prospective and retrospective data are obtained at multiple time points, enabling both cross-sectional and longitudinal research in a hypothesis-generating approach that extends beyond one specific research question. In so doing, this registry aims to (1) increase the scientific knowledge base on disease mechanisms, genetics, and novel diagnostic and treatment strategies of ACM; and (2) provide education for physicians and patients concerning ACM, e. g. through our website ( www.acmregistry.nl ) and patient conferences.

Keywords: Arrhythmogenic right ventricular dysplasia; Cardiomyopathies; Registries; Research design.

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Conflict of interest statement

L.P. Bosman, T.E. Verstraelen, F.H.M. van Lint, M.G.P.J. Cox, J.A. Groeneweg, T.P. Mast, P.A. van der Zwaag, P.G.A. Volders, R. Evertz, L. Wong, N.M.S. de Groot, K. Zeppenfeld, J.F. van der Heijden, M.P. van den Berg, A.A.M. Wilde, F.W. Asselbergs, R.N.W. Hauer, A.S.J.M. te Riele and J.P. van Tintelen declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Graphic representation of the Netherlands ACM Registry: data access, logistics and sharing. The ACM Registry is hosted on a central server at the Netherlands Heart Institute. The database is divided in 8 data-access groups, managed by local coordinators of each participating centre. The central coordinators have access to the complete database for quality control and coordination of collaboration. The central coordinators together with the local coordinators form the ACM Registry working group
See this image and copyright information in PMC

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