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. 2021 Dec;8(6):5542-5550.
doi: 10.1002/ehf2.13574. Epub 2021 Sep 12.

Study design and rationale of the pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R)

Christoph Sinning  1   2 , Elvin Zengin  1 , Gerhard-Paul Diller  3 , Francesco Onorati  4 , María-Angeles Castel  5 , Thibault Petit  6 , Yih-Sharng Chen  7 , Mauro Lo Rito  8 , Carmelina Chiarello  8 , Romain Guillemain  9 , Karine Nubret-Le Coniat  10 , Christina Magnussen  1   2 , Dorit Knappe  1 , Peter Moritz Becher  1   2 , Benedikt Schrage  1   2 , Jacqueline M Smits  11 , Andreas Metzner  1 , Christoph Knosalla  12   13   14 , Felix Schoenrath  12   13 , Oliver Miera  15 , Mi-Young Cho  16 , Alexander Bernhardt  2   17 , Jessica Weimann  1 , Alina Goßling  1 , Amedeo Terzi  18 , Antonio Amodeo  19 , Sara Alfieri  19 , Emanuela Angeli  20 , Luca Ragni  21 , Carlo Pace Napoleone  22 , Gino Gerosa  23 , Nicola Pradegan  23 , Inez Rodrigus  24 , Julia Dumfarth  25 , Michel de Pauw  26 , Katrien François  27 , Olivier Van Caenegem  28 , Arnaut Ancion  29 , Johan Van Cleemput  30 , Davor Miličić  31 , Ajay Moza  32 , Peter Schenker  33 , Josef Thul  34 , Michael Steinmetz  35   36 , Gregor Warnecke  37 , Fabio Ius  38 , Susanne Freyt  38 , Murat Avsar  38 , Tim Sandhaus  39 , Assad Haneya  40 , Sandra Eifert  41 , Diyar Saeed  41 , Michael Borger  41 , Henryk Welp  42 , László Ablonczy  43 , Bastian Schmack  44 , Arjang Ruhparwar  44 , Shiho Naito  17 , Xiaoqin Hua  17 , Nina Fluschnik  1 , Moritz Nies  1 , Laura Keil  1 , Juliana Senftinger  1 , Djemail Ismaili  1 , Shinwan Kany  1 , Dora Csengeri  1 , Massimo Cardillo  45 , Alessandra Oliveti  45 , Giuseppe Faggian  4 , Richard Dorent  46 , Carine Jasseron  46 , Alicia Pérez Blanco  47 , José Manuel Sobrino Márquez  48 , Raquel López-Vilella  49 , Ana García-Álvarez  5 , María Luz Polo López  50 , Alvaro Gonzalez Rocafort  50 , Óscar González Fernández  51 , Raquel Prieto-Arevalo  52 , Eduardo Zatarain-Nicolás  52 , Katrien Blanchart  53 , Aude Boignard  54 , Pascal Battistella  55 , Soulef Guendouz  56 , Lucile Houyel  57 , Marylou Para  58 , Erwan Flecher  59 , Arnaud Gay  60 , Éric Épailly  61 , Camille Dambrin  62 , Kaitlyn Lam  63 , Cally Ho Ka-Lai  64 , Yang Hyun Cho  65 , Jin-Oh Choi  66 , Jae-Joong Kim  67 , Louise Coats  6   68 , David Steven Crossland  6   68 , Lisa Mumford  69 , Samer Hakmi  70 , Cumaraswamy Sivathasan  71 , Larissa Fabritz  1   72   73 , Stephan Schubert  74 , Jan Gummert  75 , Michael Hübler  2   76 , Peter Jacksch  77 , Andreas Zuckermann  78 , Günther Laufer  78 , Helmut Baumgartner  3 , Alessandro Giamberti  8 , Hermann Reichenspurner  2   17 , Paulus Kirchhof  1   2   72
Affiliations

Study design and rationale of the pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R)

Christoph Sinning et al. ESC Heart Fail. 2021 Dec.

Abstract

Aim: Due to improved therapy in childhood, many patients with congenital heart disease reach adulthood and are termed adults with congenital heart disease (ACHD). ACHD often develop heart failure (HF) as a consequence of initial palliative surgery or complex anatomy and subsequently require advanced HF therapy. ACHD are usually excluded from trials evaluating heart failure therapies, and in this context, more data about heart failure trajectories in ACHD are needed to guide the management of ACHD suffering from HF.

Methods and results: The pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R) will collect data from ACHD evaluated or listed for heart or heart-combined organ transplantation from 16 countries in Europe and the Asia/Pacific region. We plan retrospective collection of data from 1989-2020 and will include patients prospectively. Additional organizations and hospitals in charge of transplantation of ACHD will be asked in the future to contribute data to the register. The primary outcome is the combined endpoint of delisting due to clinical worsening or death on the waiting list. The secondary outcome is delisting due to clinical improvement while on the waiting list. All-cause mortality following transplantation will also be assessed. The data will be entered into an electronic database with access to the investigators participating in the register. All variables of the register reflect key components important for listing of the patients or assessing current HF treatment.

Conclusion: The ARTORIA-R will provide robust information on current management and outcomes of adults with congenital heart disease suffering from advanced heart failure.

Keywords: Adults with congenital heart disease; Arrhythmia; Heart failure; Heart transplantation; Lung transplantation; Ventricular assist device.

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Conflict of interest statement

Dr. Sinning (CS) is deputy editor of the European Heart Journal – Case Reports. Dr. Zengin (EZ) reports speaker fees from AstraZeneca unrelated to the submitted work. Dr. Magnussen (CM) reports speaker fees from AstraZeneca, Novartis, and Heinen & Loewenstein unrelated to the submitted work. Dr. Bernhardt (AB) reports speaker and consulting fees from Abbott, Abiomed, AstraZeneca, Berlin Heart, Medtronic, and Novartis unrelated to the submitted work. Dr. Schoenrath (FS) reports non‐financial support from Medtronic, grants from Novartis and institutional fees from Cardiorentis AG, Abbott, AstraZeneca, and Orion Pharma unrelated to the submitted work. Dr. Ius (FI) has received speaker fees and congress fees from Biotest AG unrelated to the submitted work. Dr. Fabritz (LF) has received institutional research grants and non‐financial support from European Union, British Heart Foundation, Medical Research Council (UK), several biomedical companies and DFG. The Institute of Cardiovascular Research, University of Birmingham, has received an Accelerator Award by the British Heart Foundation AA/18/2/34218. LF is listed as inventor of two patents held by University of Birmingham (Atrial Fibrillation Therapy WO 2015140571, Markers for Atrial Fibrillation WO 2016012783). Dr. Kirchhof (PK) receives research support for basic, translational, and clinical research projects from European Union, British Heart Foundation, Leducq Foundation, Medical Research Council (UK), and German Centre for Cardiovascular Research, from several drug and device companies active in atrial fibrillation, and has received honoraria from several such companies in the past, but not in the last 3 years. PK is listed as inventor on two patents held by University of Birmingham (Atrial Fibrillation Therapy WO 2015140571, Markers for Atrial Fibrillation WO 2016012783). All other authors have no conflicts of interest in relation to the content of this manuscript.

Figures

Figure 1
Figure 1
Countries currently participating in the ARTORIA‐R.
Figure 2
Figure 2
Institutions and organizations contributing data to the register and the inclusion and exclusion criteria.
Figure 3
Figure 3
Currently planned study projects with the outlook of additional projects in the future.
See this image and copyright information in PMC

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