The PAtients pResenTing With COngenital HeaRt DIseAse Register (ARTORIA-R)

NCT04848844 | Recruiting DMC

• 1 other identifier: 2023-101119-BO-ff
• Study Type: observational
• Target: 2,000
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Congenital Heart Disease; Heart Failure; Transplant; Complication, Failure; Arrythmia; Ventricular Dysfunction

Outcome Measures

Primary Outcomes (1)

• Delisting due to clinical worsening or death on the waiting list

  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.

  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.

Secondary Outcomes (1)

• Delisting due to clinical improvement

  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.

Other Outcomes (2)

• All-cause mortality following transplantation

  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.

• Implantation of a ventricular assist device as a bridge to transplantation, bridge to candidacy, or destination therapy

  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.

Study Arms (3)

Patient transplanted due to ACHD and heart failure

Patients successfully transplanted due to congenital heart failure.

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.

ACHD evaluated for heart transplantation

All patients evaluated for heart or heart and combined organ transplantation at the hospital level

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The PAtients pResenTing with COngenital HeaRt DIseAse Register (ARTORIA-R) will collect data from patients with ACHD evaluated or listed for heart or heart-combined organ transplantation from 19 countries in Europe and the Asia/Pacific region. We plan retrospective collection of data from 1989 - 2024 and will include patients prospectively. 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.

You may qualify if:

• 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

You may not qualify if:

• a. The patient is listed for a second heart transplantation (retransplantation)

Sponsors & Collaborators

• Universitätsklinikum Hamburg-Eppendorf: lead
• Deutsches Zentrum für Herz-Kreislauf-Forschung (DZHK): collaborator

Study Sites (1)

University Heart and Vascular Center Hamburg

Hamburg, Free and Hanseatic City of Hamburg, 20246, Germany

RECRUITING

Related Publications (2)

• 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.

  PMID: 34510806 BACKGROUND

• 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.

  PMID: 32778365 BACKGROUND

MeSH Terms

Conditions

Heart Defects, Congenital; Heart Failure; Arrhythmias, Cardiac; Ventricular Dysfunction

Condition Hierarchy (Ancestors)

Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Study Officials

• Christoph Sinning, MF

  University Heart & Vascular Center Hamburg

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Christoph Sinning, MD

CONTACT

004915222817675; c.sinning@uke.de

Christina Magnussen, MD

CONTACT

c.magnussen@uke.de

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 30 Years
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: April 7, 2021
• First Posted: April 19, 2021
• Study Start: September 2, 2020
• Primary Completion (Estimated): July 30, 2030
• Study Completion (Estimated): July 30, 2030
• Last Updated: July 1, 2026

Record last verified: 2026-05

Data Sharing

• IPD Sharing: Will share
• Shared Documents: STUDY PROTOCOL, SAP, ICF
• Time Frame: The data will be analysed at the University Heart and Vascular Center Hamburg. Researchers who did submit data to the registry are eligible to send as well a research proposal utilizing the whole data base. All statistical calculations will be done at the Department of Statistics of the Heart Center in Hamburg
• Access Criteria: Researchers entering data into the registry will do so using REDCap and will see only patients of the own center.

Locations

DE (1)