Study design and rationale of the pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R)
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Updated: Oct 20, 2025
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Christoph Sinning1,2, Elvin Zengin1, Gerhard-Paul Diller3
1Department of Cardiology, University Heart and Vascular Centre Hamburg, Hamburg, Germany.
Insights
Adults with congenital heart disease (ACHD) often develop advanced heart failure (HF) and need specialized care. The ARTORIA-R registry will gather data on ACHD patients awaiting heart transplantation to improve understanding of HF management and outcomes.
Area of Science:
- Cardiology
- Adult Congenital Heart Disease (ACHD)
- Heart Failure (HF) Management
Background:
- Improved childhood therapies have increased the number of adults with congenital heart disease (ACHD).
- ACHD patients frequently develop advanced heart failure (HF) due to complex anatomy or prior surgeries.
- Current HF trials often exclude ACHD, necessitating more data on their specific HF trajectories.
Purpose of the Study:
- To establish the pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R).
- To collect comprehensive data on ACHD patients evaluated for or listed for heart transplantation.
- To enhance understanding of HF management and outcomes in the ACHD population.
Main Methods:
- The ARTORIA-R will collect retrospective (1989-2020) and prospective data from 16 European and Asia/Pacific countries.
- Data will be sourced from ACHD patients undergoing evaluation or listed for heart transplantation.
- An electronic database will store variables crucial for patient listing and HF treatment assessment.
Main Results:
- The primary outcome is the composite of death or delisting due to clinical worsening while on the transplant waiting list.
- Secondary outcomes include delisting for clinical improvement and all-cause mortality post-transplantation.
- The registry aims to capture key data points relevant to ACHD HF management.
Conclusions:
- The ARTORIA-R registry will yield crucial insights into the management and outcomes of ACHD with advanced HF.
- This data will be vital for guiding clinical decisions and improving care for this vulnerable population.
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.
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