Study design and rationale of the pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA‐R)
Authors: Sinning Christoph , Zengin Elvin , Diller Gerhard‐Paul , Onorati Francesco , Castel María‐Angeles , Petit Thibault , Chen Yih‐Sharng , Lo Rito Mauro , Chiarello Carmelina , Guillemain Romain , Coniat Karine Nubret‐Le , Magnussen Christina , Knappe Dorit , Becher Peter Moritz , Schrage Benedikt , Smits Jacqueline M. , Metzner Andreas , Knosalla Christoph , Schoenrath Felix , Miera Oliver , Cho Mi‐Young , Bernhardt Alexander , Weimann Jessica , Goßling Alina , Terzi Amedeo , Amodeo Antonio , Alfieri Sara , Angeli Emanuela , Ragni Luca , Napoleone Carlo Pace , Gerosa Gino , Pradegan Nicola , Rodrigus Inez , Dumfarth Julia , de Pauw Michel , François Katrien , Van Caenegem Olivier , Ancion Arnaut , Van Cleemput Johan , Miličić Davor , Moza Ajay , Schenker Peter , Thul Josef , Steinmetz Michael , Warnecke Gregor , Ius Fabio , Freyt Susanne , Avsar Murat , Sandhaus Tim , Haneya Assad , Eifert Sandra , Saeed Diyar , Borger Michael , Welp Henryk , Ablonczy László , Schmack Bastian , Ruhparwar Arjang , Naito Shiho , Hua Xiaoqin , Fluschnik Nina , Nies Moritz , Keil Laura , Senftinger Juliana , Ismaili Djemail , Kany Shinwan , Csengeri Dora , Cardillo Massimo , Oliveti Alessandra , Faggian Giuseppe , Dorent Richard , Jasseron Carine , Blanco Alicia Pérez , Márquez José Manuel Sobrino , López‐Vilella Raquel , García‐Álvarez Ana , López María Luz Polo , Rocafort Alvaro Gonzalez , Fernández Óscar González , Prieto‐Arevalo Raquel , Zatarain‐Nicolás Eduardo , Blanchart Katrien , Boignard Aude , Battistella Pascal , Guendouz Soulef , Houyel Lucile , Para Marylou , Flecher Erwan , Gay Arnaud , Épailly Éric , Dambrin Camille , Lam Kaitlyn , Ka‐lai Cally Ho , Cho Yang Hyun , Choi Jin‐Oh , Kim Jae‐Joong , Coats Louise , Crossland David Steven , Mumford Lisa , Hakmi Samer , Sivathasan Cumaraswamy , Fabritz Larissa , Schubert Stephan , Gummert Jan , Hübler Michael , Jacksch Peter , Zuckermann Andreas , Laufer Günther , Baumgartner Helmut , Giamberti Alessandro , Reichenspurner Hermann , Kirchhof Paulus 
Published: September 2021

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.