Reichert, M. (Marjolein) (2018) Paediatric Patients with Single Ventricle Congenital Heart Disease are at Risk for Myocardial and Hepatic Fibrotic Remodelling and Dysfunction. thesis, Medicine.
Full text available on request.Abstract
Introduction: Patients with single ventricle circulations are at risk for developing myocardial and liver fibrosis. Its extent and prevalence in children with a bidirectional cavo-pulmonary connection (BCPC) and Fontan circulation has not been evaluated. Extracellular volume fraction (ECV) and T1 values, derived from cardiac magnetic resonance (CMR), reflect fibrotic remodeling in the liver and the heart. Hypothesis: Patients after BCPC and Fontan completion have increased CMR markers of fibrosis. Methods: Native T1 and ECV were measured retrospectively; results were compared to a control group and correlated to demographic, surgical, clinical and hemodynamic information. Results: Hepatic T1 times were increased in Fontan patients (n=62, 11.4±4.4 years, 76264 ms) vs. BCPC (n=20, 2.8±0.9 years, 645±43 ms, p=0.04) and vs. controls (n=44, 13.7±2.9 years, 604±54 ms, p<0.001 against Fontan and BCPC). ECV in the Fontan and BCPC groups were 39.9±5.3% and 33.2±5.1% respectively, p=0.01. Myocardial T1 was increased in Fontan patients (1048±67 ms) vs. controls (986±49, p<0.001) and ECV was increased in both Fontan and BCPC (29±3 and 27±6% respectively) vs. in controls (21±3%, both p<0.001). In Fontan patients hepatic T1 correlated with cardiopulmonary bypass duration and inversely with oxygen saturation (R=0.273, p=0.033; R=-0.271, p=0.035) Myocardial T1 correlated with aorto-pulmonary collateral flow and inversely with age (R=0.440, p=0.001; R=-0.402, p=0.001; ECV correlated with end-diastolic and end-systolic volume (R=0.324, p=0.010; R=0.372, p=0.003) and inversely with ejection fraction (R=-0.294, p=0.020). Conclusion: Imaging biomarkers of myocardial and hepatic fibrosis are elevated, particularly in Fontan patients. Operation-related damage and hypoxia are associated with elevated liver fibrosis markers and abnormal loading conditions with elevated myocardial fibrosis markers. Longitudinal studies are required to test the prognostic significance of these findings.
| Item Type: | Thesis (Thesis) |
|---|---|
| Supervisor name: | Faculty supervisor: and Roofthooft, Marc |
| Supervisor name: | Local supervisor: and Grosse-Wortmann, Lars and Department of Pediatric Cardiology. The Hospital for the Sic |
| Faculty: | Medical Sciences |
| Date Deposited: | 25 Jun 2020 11:03 |
| Last Modified: | 25 Jun 2020 11:03 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/2338 |
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