Congenital Heart Disease, Fibrosis; Heart, Fibrosis Myocardial
Conditions
Keywords
Late gadolinium enhancement, 3D late gadolinium enhancement, atrial late gadolinium enhancement, Cardiac MRI, Congenital heart disease
Brief summary
In this research study the investigators want to learn more about how well the investigators can visualize scar tissue in the heart by MRI. In patients with congenital heart disease who need a procedure in the electrophysiology laboratory, how the MRI findings match the findings in the electrophysiology laboratory is not known. This study works to answer these questions. Participants will undergo a cardiac MRI as part of the routine clinical care that was ordered by their doctors and additional imaging by cardiac MRI will be performed.
Detailed description
Atrial arrhythmias including intra-atrial reentrant tachycardia (IART) and atrial fibrillation (AF) routinely develop after surgical repair of congenital heart disease (CHD), contributing to heart failure exacerbation, increased hospital resource use, and reduced health-related quality of life. The combination of atriotomy scars, intra-cardiac suture lines, and chronic pressure or volume overload from residual lesions creates the necessary milieu of heterogeneous atrial fibrosis capable of supporting wavefront reentry. While catheter ablation has become a primary tool in the management of IART and AF, long-term ablation outcomes have stagnated over the preceding decade despite advances in mapping and ablative technologies. Left atrial (LA) fibrosis analysis using 3-dimensional (3D) late gadolinium enhancement (LGE) cardiac magnetic resonance imaging (CMR) has shown utility in the management of adults with structurally normal hearts and atrial fibrillation (AF), having associations with endocardial bipolar voltage amplitude, likelihood of maintenance of sinus rhythm after ablation, and thromboembolic risk. Excellent reproducibility of LA fibrosis quantification has been demonstrated in adults with structurally normal hearts and AF. To date, the use of 3D LGE in CHD has been limited to the ventricles. Prior studies have described altered LA function in adolescent and young adult patients with rTOF. Additionally, right atrial (RA) functional abnormalities have also been described in patients with rTOF. No prior studies have attempted to validate this technology in the atrium of patients with congenital heart disease. Our studies aims to investigate the reproducibility of atrial fibrosis quantification by cardiac MRI and may provide insights to correlations with voltage mapping in the electrophysiology laboratory.
Interventions
DIAGNOSTIC_TESTAtrial 3D late gadolinium enhancement
We will use cardiac MRI to take a picture of the atria. This picture will be used to identify scar tissue within the atria.
Sponsors
Boston Children's Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
13 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patients \>13 years old with congenital heart disease referred for cardiac MRI and receiving gadolinium as part of routine clinical care or for pre-ablation planning will be included.
Exclusion criteria
* Those with self-reported anxiety or claustrophobia and/or the presence of a permanent pacemaker or implantable cardioverter defibrillator will be excluded.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Total atrial fibrosis content from 3D-LGE-CMR | 1 year | Atrial 3D-LGE-CMR images will be post-processed using the Circle ADAS 3D software module (Circle Cardiovascular Inc., Calgary, AB, Canada) and analyzed to identify fibrotic tissue. Results are portrayed as 3D-colored maps and as a calculated percentage of atrial fibrosis relative to total atrial surface area. All CMR images will be analyzed by two observers for the calculation of interrater reliability of the percent of atrial LGE. One observer will perform contours twice for the purposes of intra-observer agreement. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Total atrial fibrosis content from catheter-derived endocardial bipolar voltage map. | 2 years | In patients who have a cardiac MRI and then an electrophysiology study as part of their clinical care, the 3D-CMR image will be compared to the catheter-generated electro-anatomic voltage mapping obtained in the electrophysiology laboratory. |
| Sustained atrial arrhythmia, defined as IART/AF lasting >30 seconds detected on ECG, ambulatory monitor, or inpatient telemetry | 5 years | Following 3D-LGE-CMR acquisition, CMR and clinical data will be entered into a deidentified database to be used for clinical research. After 5 years patients without a prior history of electrophysiology issues will be analyzed prospectively (over a 5 year period) for the development of a sustained atrial arrhythmia using time-to-event analysis. |
Countries
United States
Contacts
Primary ContactDaniel A Castellanos, MD
Backup ContactEdward O'Leary, MD
Outcome results
None listed