The PAtients pResenTing With COngenital HeaRt DIseAse Register (ARTORIA-R) (ARTORIA-R)
The PAtients pResenTing With COngenital HeaRt DIseAse Register (ARTORIA-R): A Global Register to Investigate Factors Associated With Morbidity and Mortality in Adult Patients With Congenital Heart Disease (ACHD) on the Waiting List for Heart or Heart/Lung Transplantation
Advances in surgical and medical care have led to improved outcomes in patients with congenital heart disease (CHD). As a consequence, the majority of patients nowadays survives to adulthood (adults with CHD, that is, adult CHD [ACHD]) with good quality of life. Despite the surgical success, the morbidity and mortality of ACHD is higher than in the general population and is linked to the development of heart failure (HF) in adulthood.
HF occurs in approximately 25% of patients with ACHD, even in those patients in whom the congenital mal-formation has been corrected successfully in childhood. The time course and presentation are heterogeneous owing to variable congenital malformation and limitation of treatment options. ACHD with an anatomic right ventricle as the systemic ventricle (e.g., atrial switch operation in patients with transposition of the great arteries [TGAs]) and those with a functional single ventricle (e.g., Fontan circulation) appear to be at higher risk of developing HF. Young age at initial corrective surgery-often in the first 2 years of life-and lack of specific medical therapies can contribute to a high and early demand for heart transplantation in patients with ACHD.
Study Overview
Status
Detailed Description
The ARTORIA-R is an international, observational research project collecting retrospective data from 18 countries in the period 1989 to 2024 and will thereafter include data prospectively (Figure 1). These data include patient profiles at time of evaluation for listing, listing, waiting list dynamics, post-transplant data and basic donor data. Future data will be updated annually in July each year and each institution willing to contribute anonymised data to the register is invited to participate. Furthermore, the institutions and organ allocation agencies have to obtain a positive vote of the ethic committee responsible for the data transmission of anonymized patient data.
The inclusion criteria are: a. The patient has to be listed as an adult transplant candidate in the country the data is obtained with an age ≥18 years b. The patient has to have a congenital heart defect or an inherited cardiomyopathy (specific; hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy or non-compaction cardiomyopathy) which is often included into the category ACHD c. Data is obtained from the first evaluation for listing or listing for heart-only or heart-combined organ transplantation d. Transfer of anonymised data e. The institution/organization agrees to the memorandum how data is managed, and scientific cooperation is planned between all institutions. The registry has the aim to achieve meticulous information regarding the underlying congenital heart defect and the previous treatment of the patient. With these information the patients can be divided into different cohorts with a systemic left ventricle, a systemic right ventricle or a single ventricle (which can be either an anatomic left or right ventricle). Further data is acquired regarding the medical treatment, the haemodynamic evaluation of the patient, laboratory testing to assess additional organ function of the kidney or liver, treatment in the intermediate care or intensive care unit. As it is of special relevance in ACHD patient treatment of arrhythmia, antiarrhythmic medication and use of ICD or cardiac resynchronization therapy are evaluated. Were available the data regarding imaging at listing with ejection fraction for the systemic ventricle with echocardiography or cardiac magnetic resonance imaging are obtained.
The exclusion criteria is: a. The patient is listed for a second heart transplantation (retransplantation) During the ongoing study additional institutions and organizations will be asked to include their data as well. Although the initial data is retrospective additional data into the register will be entered prospectively.
Study Type
Enrollment (Estimated)
Contacts and Locations
Study Contact
- Name: Christoph Sinning, MD
- Phone Number: 004915222817675
- Email: c.sinning@uke.de
Study Contact Backup
- Name: Christina Magnussen, MD
- Email: c.magnussen@uke.de
Study Locations
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Free and Hanseatic City of Hamburg
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Hamburg, Free and Hanseatic City of Hamburg, Germany, 20246
- Recruiting
- University Heart and Vascular Center Hamburg
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Contact:
- Christoph Sinning, MD
- Phone Number: 004915222817675
- Email: c.sinning@uke.de
-
Contact:
- Christina Magnussen, MD
- Email: c.magnussen@uke.de
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Participation Criteria
Eligibility Criteria
Ages Eligible for Study
Accepts Healthy Volunteers
Sampling Method
Study Population
Description
Inclusion Criteria:
- The patient has to be listed as an adult transplant candidate in the country the data is obtained with an age ≥18 years
- The patient has to have a congenital heart defect or an inherited cardiomyopathy (specific; hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy or non-compaction cardiomyopathy) which is often included into the category ACHD
- Data is obtained from the first evaluation for listing or listing for heart-only or heart-combined organ transplantation
- Transfer of anonymised data
- The institution/organization agrees to the memorandum how data is managed, and scientific cooperation is planned between all institutions
Exclusion Criteria:
a. The patient is listed for a second heart transplantation (retransplantation)
Study Plan
How is the study designed?
Design Details
Cohorts and Interventions
Group / Cohort |
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Patient transplanted due to ACHD and heart failure
Patients successfully transplanted due to congenital heart failure.
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ACHD listed due to heart failure for heart or heart and combined organ transplantation
Patients on the actual waiting list for heart or heart and combined organ transplantation.
They can have either outcome transplantation or the primary outcome death on the waiting list or delisting due to clinical worsening.
The secondary outcome is as well delisting due to clinical improvement.
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ACHD evaluated for heart transplantation
All patients evaluated for heart or heart and combined organ transplantation at the hospital level
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What is the study measuring?
Primary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
|---|---|---|
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Delisting due to clinical worsening or death on the waiting list
Time Frame: From the date of listing until delisting due to clinical worsening, death on the waiting list, transplantation, or last available follow-up, assessed up to 15 years.
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Patients who experience clinical worsening due to advanced heart failure may be removed from the waiting list.
Patients may also die while on the waiting list.
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From the date of listing until delisting due to clinical worsening, death on the waiting list, transplantation, or last available follow-up, assessed up to 15 years.
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Secondary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
|---|---|---|
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Delisting due to clinical improvement
Time Frame: From the date of listing until the date of delisting due to clinical improvement, transplantation, death, or last available follow-up, whichever occurs first, assessed up to 15 years.
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Patients may improve after listing and be removed from the waiting list because they no longer fulfill the criteria for heart or combined organ transplantation.
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From the date of listing until the date of delisting due to clinical improvement, transplantation, death, or last available follow-up, whichever occurs first, assessed up to 15 years.
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Other Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
|---|---|---|
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All-cause mortality following transplantation
Time Frame: From the date of transplantation until the date of death from any cause or last available follow-up, whichever occurs first, assessed up to 15 years after transplantation.
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Patients who undergo successful transplantation are followed at the transplant center, and vital status is recorded during follow-up.
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From the date of transplantation until the date of death from any cause or last available follow-up, whichever occurs first, assessed up to 15 years after transplantation.
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Implantation of a ventricular assist device as a bridge to transplantation, bridge to candidacy, or destination therapy
Time Frame: From the date of registry inclusion until the date of ventricular assist device implantation or last available follow-up, whichever occurs first, assessed up to 15 years.
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Some patients will receive a ventricular assist device as a bridge to transplantation or candidacy; others may receive the device as destination therapy.
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From the date of registry inclusion until the date of ventricular assist device implantation or last available follow-up, whichever occurs first, assessed up to 15 years.
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Collaborators and Investigators
Collaborators
Investigators
- Principal Investigator: Christoph Sinning, MF, University Heart & Vascular Center Hamburg
Publications and helpful links
General Publications
- Sinning C, Zengin E, Diller GP, Onorati F, Castel MA, Petit T, Chen YS, Lo Rito M, Chiarello C, Guillemain R, Coniat KN, Magnussen C, Knappe D, Becher PM, Schrage B, Smits JM, Metzner A, Knosalla C, Schoenrath F, Miera O, Cho MY, Bernhardt A, Weimann J, Gossling A, Terzi A, Amodeo A, Alfieri S, Angeli E, Ragni L, Napoleone CP, Gerosa G, Pradegan N, Rodrigus I, Dumfarth J, de Pauw M, Francois K, Van Caenegem O, Ancion A, Van Cleemput J, Milicic D, Moza A, Schenker P, Thul J, Steinmetz M, Warnecke G, Ius F, Freyt S, Avsar M, Sandhaus T, Haneya A, Eifert S, Saeed D, Borger M, Welp H, Ablonczy L, Schmack B, Ruhparwar A, Naito S, Hua X, Fluschnik N, Nies M, Keil L, Senftinger J, Ismaili D, Kany S, Csengeri D, Cardillo M, Oliveti A, Faggian G, Dorent R, Jasseron C, Blanco AP, Marquez JMS, Lopez-Vilella R, Garcia-Alvarez A, Lopez MLP, Rocafort AG, Fernandez OG, Prieto-Arevalo R, Zatarain-Nicolas E, Blanchart K, Boignard A, Battistella P, Guendouz S, Houyel L, Para M, Flecher E, Gay A, Epailly E, Dambrin C, Lam K, Ka-Lai CH, Cho YH, Choi JO, Kim JJ, Coats L, Crossland DS, Mumford L, Hakmi S, Sivathasan C, Fabritz L, Schubert S, Gummert J, Hubler M, Jacksch P, Zuckermann A, Laufer G, Baumgartner H, Giamberti A, Reichenspurner H, Kirchhof P. Study design and rationale of the pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R). ESC Heart Fail. 2021 Dec;8(6):5542-5550. doi: 10.1002/ehf2.13574. Epub 2021 Sep 12.
- Becher PM, Schrage B, Weimann J, Smits J, Magnussen C, Reichenspurner H, Gossling A, Rodrigus I, Dumfarth J, de Pauw M, Francois K, van Caenegem O, Ancion A, Van Cleemput J, Milicic D, Moza A, Schenker P, Rohrich L, Schonrath F, Thul J, Steinmetz M, Schmack B, Ruhparwar A, Warnecke G, Rojas SV, Sandhaus T, Haneya A, Eifert S, Welp H, Ablonczy L, Wagner F, Westermann D, Bernhardt AM, Knappe D, Blankenberg S, Kirchhof P, Zengin E, Sinning C. Clinical characteristics and outcomes of patients with adult congenital heart disease listed for heart and heart-lung transplantation in the Eurotransplant region. J Heart Lung Transplant. 2020 Nov;39(11):1238-1249. doi: 10.1016/j.healun.2020.07.012. Epub 2020 Jul 25.
Study record dates
Study Major Dates
Study Start (Actual)
Primary Completion (Estimated)
Study Completion (Estimated)
Study Registration Dates
First Submitted
First Submitted That Met QC Criteria
First Posted (Actual)
Study Record Updates
Last Update Posted (Actual)
Last Update Submitted That Met QC Criteria
Last Verified
More Information
Terms related to this study
Additional Relevant MeSH Terms
- Cardiovascular Diseases
- Pathologic Processes
- Heart Diseases
- Congenital Abnormalities
- Cardiovascular Abnormalities
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities
- Pathological Conditions, Signs and Symptoms
- Heart Failure
- Heart Defects, Congenital
- Ventricular Dysfunction
- Arrhythmias, Cardiac
Other Study ID Numbers
- 2023-101119-BO-ff
Plan for Individual participant data (IPD)
Plan to Share Individual Participant Data (IPD)?
IPD Plan Description
IPD Sharing Time Frame
IPD Sharing Access Criteria
IPD Sharing Supporting Information Type
- STUDY_PROTOCOL
- SAP
- ICF
Drug and device information, study documents
Studies a U.S. FDA-regulated drug product
Studies a U.S. FDA-regulated device product
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