Validation of Cardiac Magnetic Resonance Sequences in Patients With Single Ventricles

NCT04017494 | Unknown

• 1 other identifier: SV-CMR
• Study Type: observational
• Target: 63
• Locations: 1 country
• Sites: 1

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

Keywords

Fontan; Congenital heart defects; Non-invasive cardiac imaging; Biomarkers

Outcome Measures

Primary Outcomes (2)

• Strain correlation with ventricular volume

  Strain correlation with ventricular volume measured by magnetic resonance imaging

  1 year

• Strain correlation with clinical parameters

  Strain correlation with clinical parameters such as presence of arrhythmias on Holter-EKG, maximal oxygen consumption on cardio-pulmonary exercise testing (ml/(kg\*min))

  1 year

Secondary Outcomes (3)

• T1 values compared to previously established normal cohort

  1 year

• Blood and exhaled biomarkers of heart failure correlation with T1 mapping

  1 year

• Intraventricular blood flow correlation with cardiac function

  1 year

Study Arms (1)

Single ventricle

Patients with single ventricle lesions

Diagnostic Test: Cardiac Magnetic Resonance Imaging; Diagnostic Test: Blood draw for hematocrit and heart failure biomarkers; Diagnostic Test: Cardiopulmonary exercise test; Diagnostic Test: Exhalomics; Diagnostic Test: Quality of life questionnaire

Interventions

Cardiac Magnetic Resonance Imaging DIAGNOSTIC_TEST

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

Single ventricle

Blood draw for hematocrit and heart failure biomarkers DIAGNOSTIC_TEST

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

Single ventricle

Cardiopulmonary exercise test 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

Single ventricle

Exhalomics 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)

Single ventricle

Quality of life questionnaire DIAGNOSTIC_TEST

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

Single ventricle

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with single ventricle physiology

You may qualify if:

• 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

You may not qualify if:

• 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

Sponsors & Collaborators

• Barbara Burkhardt: lead

Study Sites (1)

University Children's Hospital Zürich, Switzerland

Zurich, Canton of Zurich, 8032, Switzerland

Location

MeSH Terms

Conditions

Univentricular Heart; Heart Defects, Congenital

Interventions

Blood Specimen Collection; Hematocrit; Exercise Test

Condition Hierarchy (Ancestors)

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

Intervention Hierarchy (Ancestors)

Specimen Handling; Clinical Laboratory Techniques; Diagnostic Techniques and Procedures; Diagnosis; Punctures; Surgical Procedures, Operative; Investigative Techniques; Hematologic Tests; Hemorheology; Blood Physiological Phenomena; Circulatory and Respiratory Physiological Phenomena; Heart Function Tests; Diagnostic Techniques, Cardiovascular; Respiratory Function Tests; Diagnostic Techniques, Respiratory System; Ergometry

Study Officials

• Barbara EU Burkhardt, MD

  University of Zurich Children's Hospital

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Barbara EU Burkhardt, MD

CONTACT

+41442667111; barbara.burkhardt@kispi.uzh.ch

Silvia Hilfiker

CONTACT

+41442663339; silvia.hilfiker@kispi.uzh.ch

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR INVESTIGATOR
• PI Title: Principal Investigator

Study Record Dates

• First Submitted: July 8, 2019
• First Posted: July 12, 2019
• Study Start: September 1, 2019
• Primary Completion: August 31, 2023
• Study Completion: August 31, 2023
• Last Updated: July 12, 2019

Record last verified: 2019-07

Data Sharing

• IPD Sharing: Will not share

Locations

CH (1)