Cardiac Magnetic Resonance Stress-perfusion Study in Patinets with Fontan Circulation

NCT06735521 | Recruiting

• 1 other identifier: Dnr 2022-04574-01
• Study Type: observational
• Target: 30
• Locations: 1 country
• Sites: 1

Brief Summary

Univentricular heart (UVH) is a severe congenital heart disease. Accurate advanced non-invasive diagnostic methods is limited. Cardiovascular magnetic resonance (CMR) imaging has evolved as a particularly useful tool for the study of patients with adult congenital heart disease (ACHD) considering its ability to determine detailed anatomy and detect early cardiac dysfunction without the need for radiation exposure. Most of contemporary treatment recommendations are based on consensus opinions/documents and small studies from local, or national registries. Improved knowledge is needed in all these areas to facilitate clinical decisions regarding treatment, monitoring and follow-up. This study seeks to answer if early detection of deterioration in cardiac function, venous pressure and microvascular dysfunction can identify patients before the symptoms progress and thus help to initiate early treatment. The hypothesis is that quantitative myocardial stress-perfusion maps improves the pathophysiological insight in patients with UVH. The overall goal with this research proposal is to implement combined advanced CMR imaging for a comprehensive non-invasive mapping of functional cardiovascular behavior in patients with complex UVH disease. The outcome of this research may benefit this young adult patient population due to early detection of cardiac disease, less hospitalizations because of heart failure, and eventually decrease morbidity and mortality.

Conditions

Univentricular Heart; Microvascular Dysfunction; Heart Failure; Magnetic Resonance

Keywords

Fontan circulation; CMR; stress-perfusion; tissue characterization; univentricle heart; TCPC

Outcome Measures

Primary Outcomes (1)

• Detection of microvascular dysfunction in patients with UVH

  This project aims to evaluate microvascular dysfunction using quantitative stress-perfusion CMR in patients with different morphological systemic chamber (left vs right).

  Through study completion, an average of 1 year

Secondary Outcomes (1)

• To detect and compare tissue characterization.

  Through study completion, an average of 1 year

Other Outcomes (1)

• Venous pressure in TCPC

  Through study completion, an average of 1 year

Study Arms (1)

Univentricle heart, operated with TCPC (total cavo pulmonary connection)

Diagnostic Test: Cardiac magnetic resonance stress-perfusion

Interventions

Cardiac magnetic resonance stress-perfusion DIAGNOSTIC_TEST

Included patients will be examined at the Karolinska University Hospital in a 1.5T Siemens Sola magnetic resonance camera at rest and during adenosine stress with specific CMR sequences (adenosine stress-perfusion, myocardial velocities, and tissue characterization) for non-invasive determination of macro- and microvascular dysfunction, global myocardial function, pulmonary artery pressure and scarring of the myocardium. Adenosine or Regadenoson will be used as stress medication which is according to clinical routine. Adenosine will be infused over approximately 5 minutes (110-140 μg / kg/min), Regadenoson (5 ml) will be a 10-second injection (400 μg single dosage). Intravenous gadolinium-based contrast agents will be administered, Gadovist (1 mmol/ml, gadobuterol), 0.15 mmol/kg during stress and rest.

Univentricle heart, operated with TCPC (total cavo pulmonary connection)

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

The project is planned as a prospective cohort study where approximately 30 patients with Fontan circulation will be included. The cohort is out-clinic patients at the tertiary adult congenital heart disease center at the Karolinska University Hospital in Stockholm, Sweden. The patients will be asked to participate in the study when they have a clinical visit at Karolinska by the treating physician or nurse. Inclusion and exclusion criteria are shown above. Additional data will be collected from the patient cohort; age, gender, underlying diagnoses, interventions, medication, symptoms, NYHA functional class, ECG, cardiac pulmonary exercise results, self-reported level of physical activity, pacemaker / implantable cardioverter defibrillator (ICD), and quality of life assessed by EuroQoL 5-dimensional questionnaire (EQ-5D).

You may qualify if:

• Patients who had undergone total cavo-pulmonary connection (TCPC) surgery resulting in Fontan circulation.
• NYHA (New York Heart Assosiation) class I-II.
• Understanding the study information (signed informed consent).
• \>18 years old.

You may not qualify if:

• Device therapy (pacemaker, ICD).
• Failing Fontan (NYHA III-IV).
• Kidney failure (GFR\<30ml/h).
• Arrythmia (atrial fibrillation).
• Pregnancy.

Sponsors & Collaborators

• Karolinska Institutet: lead
• Karolinska University Hospital: collaborator

Study Sites (1)

Karolinska University Hospital

Stockholm, 17176, Sweden

RECRUITING

Related Publications (3)

• Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH, Hsia TY, Hsu DT, Kovacs AH, McCrindle BW, Newburger JW, Pike NA, Rodefeld M, Rosenthal DN, Schumacher KR, Marino BS, Stout K, Veldtman G, Younoszai AK, d'Udekem Y; American Heart Association Council on Cardiovascular Disease in the Young and Council on Cardiovascular and Stroke Nursing. Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association. Circulation. 2019 Aug 6;140(6):e234-e284. doi: 10.1161/CIR.0000000000000696. Epub 2019 Jul 1.

  PMID: 31256636 BACKGROUND

• Di Salvo G, Miller O, Babu Narayan S, Li W, Budts W, Valsangiacomo Buechel ER, Frigiola A, van den Bosch AE, Bonello B, Mertens L, Hussain T, Parish V, Habib G, Edvardsen T, Geva T, Baumgartner H, Gatzoulis MA; 2016-2018 EACVI Scientific Documents Committee. Imaging the adult with congenital heart disease: a multimodality imaging approach-position paper from the EACVI. Eur Heart J Cardiovasc Imaging. 2018 Oct 1;19(10):1077-1098. doi: 10.1093/ehjci/jey102.

  PMID: 30084968 BACKGROUND

• Dalen M, Odermarsky M, Liuba P, Johansson Ramgren J, Synnergren M, Sunnegardh J. Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study. J Am Heart Assoc. 2024 Mar 19;13(6):e031722. doi: 10.1161/JAHA.123.031722. Epub 2024 Mar 18.

  PMID: 38497454 BACKGROUND

MeSH Terms

Conditions

Univentricular Heart; Heart Failure

Condition Hierarchy (Ancestors)

Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Central Study Contacts

Peder Sorensson, MD, Associate professor

CONTACT

+46 731 400059; peder.sorensson@ki.se

Ayse-Gul Ozturk, MD

CONTACT

ayse-gul.ozturk@ki.se

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 1 Year
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Senior Consultant in Cardiology

Study Record Dates

• First Submitted: October 16, 2024
• First Posted: December 16, 2024
• Study Start: April 15, 2024
• Primary Completion: June 30, 2025
• Study Completion: September 1, 2025
• Last Updated: December 16, 2024

Record last verified: 2024-11

Data Sharing

• IPD Sharing: Will share
• Shared Documents: STUDY PROTOCOL, CSR
• Time Frame: April 2024 - April 2026.
• Access Criteria: On written request to the PI from researchers in the same field of interests.

Locations

SE (1)