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

Christoph Sinning, Elvin Zengin, Gerhard-Paul Diller, Francesco Onorati, María-Angeles Castel, Thibault Petit, Yih-Sharng Chen, Mauro Lo Rito, Carmelina Chiarello, Romain Guillemain, Karine Nubret-Le Coniat, Christina Magnussen, Dorit Knappe, Peter Moritz Becher, Benedikt Schrage, Jacqueline M Smits, Andreas Metzner, Christoph Knosalla, Felix Schoenrath, Oliver Miera, Mi-Young Cho, Alexander Bernhardt, Jessica Weimann, Alina Goßling, Amedeo Terzi, Antonio Amodeo, Sara Alfieri, Emanuela Angeli, Luca Ragni, Carlo Pace Napoleone, Gino Gerosa, Nicola Pradegan, Inez Rodrigus, Julia Dumfarth, Michel de Pauw, Katrien François, Olivier Van Caenegem, Arnaut Ancion, Johan Van Cleemput, Davor Miličić, Ajay Moza, Peter Schenker, Josef Thul, Michael Steinmetz, Gregor Warnecke, Fabio Ius, Susanne Freyt, Murat Avsar, Tim Sandhaus, Assad Haneya, Sandra Eifert, Diyar Saeed, Michael Borger, Henryk Welp, László Ablonczy, Bastian Schmack, Arjang Ruhparwar, Shiho Naito, Xiaoqin Hua, Nina Fluschnik, Moritz Nies, Laura Keil, Juliana Senftinger, Djemail Ismaili, Shinwan Kany, Dora Csengeri, Massimo Cardillo, Alessandra Oliveti, Giuseppe Faggian, Richard Dorent, Carine Jasseron, Alicia Pérez Blanco, José Manuel Sobrino Márquez, Raquel López-Vilella, Ana García-Álvarez, María Luz Polo López, Alvaro Gonzalez Rocafort, Óscar González Fernández, Raquel Prieto-Arevalo, Eduardo Zatarain-Nicolás, Katrien Blanchart, Aude Boignard, Pascal Battistella, Soulef Guendouz, Lucile Houyel, Marylou Para, Erwan Flecher, Arnaud Gay, Éric Épailly, Camille Dambrin, Kaitlyn Lam, Cally Ho Ka-Lai, Yang Hyun Cho, Jin-Oh Choi, Jae-Joong Kim, Louise Coats, David Steven Crossland, Lisa Mumford, Samer Hakmi, Cumaraswamy Sivathasan, Larissa Fabritz, Stephan Schubert, Jan Gummert, Michael Hübler, Peter Jacksch, Andreas Zuckermann, Günther Laufer, Helmut Baumgartner, Alessandro Giamberti, Hermann Reichenspurner, Paulus Kirchhof, Christoph Sinning, Elvin Zengin, Gerhard-Paul Diller, Francesco Onorati, María-Angeles Castel, Thibault Petit, Yih-Sharng Chen, Mauro Lo Rito, Carmelina Chiarello, Romain Guillemain, Karine Nubret-Le Coniat, Christina Magnussen, Dorit Knappe, Peter Moritz Becher, Benedikt Schrage, Jacqueline M Smits, Andreas Metzner, Christoph Knosalla, Felix Schoenrath, Oliver Miera, Mi-Young Cho, Alexander Bernhardt, Jessica Weimann, Alina Goßling, Amedeo Terzi, Antonio Amodeo, Sara Alfieri, Emanuela Angeli, Luca Ragni, Carlo Pace Napoleone, Gino Gerosa, Nicola Pradegan, Inez Rodrigus, Julia Dumfarth, Michel de Pauw, Katrien François, Olivier Van Caenegem, Arnaut Ancion, Johan Van Cleemput, Davor Miličić, Ajay Moza, Peter Schenker, Josef Thul, Michael Steinmetz, Gregor Warnecke, Fabio Ius, Susanne Freyt, Murat Avsar, Tim Sandhaus, Assad Haneya, Sandra Eifert, Diyar Saeed, Michael Borger, Henryk Welp, László Ablonczy, Bastian Schmack, Arjang Ruhparwar, Shiho Naito, Xiaoqin Hua, Nina Fluschnik, Moritz Nies, Laura Keil, Juliana Senftinger, Djemail Ismaili, Shinwan Kany, Dora Csengeri, Massimo Cardillo, Alessandra Oliveti, Giuseppe Faggian, Richard Dorent, Carine Jasseron, Alicia Pérez Blanco, José Manuel Sobrino Márquez, Raquel López-Vilella, Ana García-Álvarez, María Luz Polo López, Alvaro Gonzalez Rocafort, Óscar González Fernández, Raquel Prieto-Arevalo, Eduardo Zatarain-Nicolás, Katrien Blanchart, Aude Boignard, Pascal Battistella, Soulef Guendouz, Lucile Houyel, Marylou Para, Erwan Flecher, Arnaud Gay, Éric Épailly, Camille Dambrin, Kaitlyn Lam, Cally Ho Ka-Lai, Yang Hyun Cho, Jin-Oh Choi, Jae-Joong Kim, Louise Coats, David Steven Crossland, Lisa Mumford, Samer Hakmi, Cumaraswamy Sivathasan, Larissa Fabritz, Stephan Schubert, Jan Gummert, Michael Hübler, Peter Jacksch, Andreas Zuckermann, Günther Laufer, Helmut Baumgartner, Alessandro Giamberti, Hermann Reichenspurner, Paulus Kirchhof

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.

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.

© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.

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.

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