Validation of Cardiac Magnetic Resonance Sequences in Patients With Single Ventricles

NCT ID: NCT04017494

Last Updated: 2019-07-12

Basic Information

• Recruitment Status: UNKNOWN
• Total Enrollment: 63 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2019-09-01
• Study Completion Date: 2023-08-31

Brief Summary

Single ventricle defects make up the severe end of the congenital heart disease spectrum. The Fontan operation leads to a complete redirection of systemic venous blood outside of the heart and directly into the lungs. Patients with single ventricles suffer from multiple complications. Their survival has improved over the past decades, but is still severely compromised compared to the general population.

Their evaluation includes echocardiography and functional status by history and/or exercise testing. In longer intervals or if echocardiography does not allow visualization of all cardiovascular structures, cardiac magnetic resonance (CMR) is employed. Many patients also undergo more invasive cardiac catheterization.

In single ventricle patients, cardiac imaging has to address the questions of the patency of the Fontan pathways, i.e. all systemic veins, the Fontan conduit, and the pulmonary arteries, and of the function of the single ventricle (including myocardial function and valve function).

By using conventional imaging methods in Fontan patients, Ghelani et al. identified a CMR-based ventricular end-diastolic volume of > 125 ml/m2 and an echocardiographic global circumferential strain (GCS) value of higher than -17% to be strong predictors for a combined adverse outcome of death or heart transplantation. While interobserver reproducibility of single ventricle ejection fraction is similarly high by echocardiography, CMR is better in reliably measuring ventricular mass and diastolic volume and can provide additional information by MR feature tracking (strain), T1 mapping, and 4D flow measurements. Several substances that can be measured in the peripheral blood are being increasingly investigated as biomarkers of heart failure.

In conclusion, several advanced CMR sequences and new biomarkers have a potential role in the assessment and risk stratification of single ventricle patients. Every single published study has elucidated a particular use and aspect of these parameters, but broader correlations and prognostic values are still unclear.

The investigators hypothesize that myocardial strain (by feature tracking), myocardial fibrosis (by T1 mapping), and intracardiac flow disturbances (by 4D flow) along with biomarkers are diagnostic for single ventricle dysfunction and correlate with known prognostic factors.

This is a single center, prospective, observational cohort study. There will be no randomisation or blinding. Study setting: outpatients, cardiology clinic and radiology department, academic hospital. Every patient will be examined twice with a one-year interval (MR will only be repeated if clinically indicated).

Conditions

Single-ventricle

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Study Groups

Single ventricle

Patients with single ventricle lesions

Cardiac Magnetic Resonance Imaging

Intervention Type: DIAGNOSTIC_TEST

Cardiac Magnetic Resonance Imaging (non-invasive, with i.v. application of contrast), awake or (if clinically indicated) in general anesthesia

Blood draw for hematocrit and heart failure biomarkers

Intervention Type: DIAGNOSTIC_TEST

Approximately 10 ml of blood will be drawn before administration of contrast medium.

Cardiopulmonary exercise test

Intervention Type: DIAGNOSTIC_TEST

In patients 8 years of age or older: on a cycle ergometer with breath-by-breath analysis, continuous ECG and SpO2 monitoring during exercise, after a baseline spirometry and bodyplethysmography

Exhalomics

Intervention Type: DIAGNOSTIC_TEST

Measurement of exhaled molecules by mass spectrometry; patients breathe into a mouthpiece for 15 seconds 6 times (total time requirement: about 5 minutes)

Quality of life questionnaire

Intervention Type: DIAGNOSTIC_TEST

Questionnaire to be filled out by the Patient regarding quality of life perception

Interventions

Cardiac Magnetic Resonance Imaging

Cardiac Magnetic Resonance Imaging (non-invasive, with i.v. application of contrast), awake or (if clinically indicated) in general anesthesia

Intervention Type: DIAGNOSTIC_TEST

Blood draw for hematocrit and heart failure biomarkers

Approximately 10 ml of blood will be drawn before administration of contrast medium.

Intervention Type: DIAGNOSTIC_TEST

Cardiopulmonary exercise test

In patients 8 years of age or older: on a cycle ergometer with breath-by-breath analysis, continuous ECG and SpO2 monitoring during exercise, after a baseline spirometry and bodyplethysmography

Intervention Type: DIAGNOSTIC_TEST

Exhalomics

Measurement of exhaled molecules by mass spectrometry; patients breathe into a mouthpiece for 15 seconds 6 times (total time requirement: about 5 minutes)

Intervention Type: DIAGNOSTIC_TEST

Quality of life questionnaire

Questionnaire to be filled out by the Patient regarding quality of life perception

Intervention Type: DIAGNOSTIC_TEST

Eligibility Criteria

Inclusion Criteria

• Patients of any age with functionally single ventricle (patients under age 8 who need anesthesia for CMR will not be actively recruited. They may be approached to participate only if the anesthesia and CMR examination have been planned independently for clinical purposes)
• Written informed consent

Exclusion Criteria

• Inability to follow the procedures of the study, e.g. due to language problems, psychological disorders, inability to give assent or consent, etc. of the participant and/or his/her parents or legal caregivers
• MR-incompatible implanted or accidentally incorporated metal device or claustrophobia that prohibits use of magnetic resonance imaging (patient and guardians fill out a questionnaire).
• Pregnancy
• Participation in another study is not an exclusion criterion, e.g. in a therapeutic trial.

• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Barbara Burkhardt

OTHER

Sponsor Role: lead

Responsible Party

Barbara Burkhardt

Principal Investigator

Responsibility Role: SPONSOR_INVESTIGATOR

Principal Investigators

Barbara EU Burkhardt, MD

Role: PRINCIPAL_INVESTIGATOR

University of Zurich Children's Hospital

Locations

University Children's Hospital Zürich, Switzerland

Zurich, Canton of Zurich, Switzerland

Countries

Switzerland

Central Contacts

Barbara EU Burkhardt, MD

Role: CONTACT

barbara.burkhardt@kispi.uzh.ch

+41442667111

Silvia Hilfiker

Role: CONTACT

silvia.hilfiker@kispi.uzh.ch

+41442663339

Other Identifiers

SV-CMR

Identifier Type: -

Identifier Source: org_study_id