T1 mapping to assess hepatic and myocardial characteristics in children with single ventricle circulation

Abstract Background Single ventricle (SV) palliation, culminating with the Fontan operation, results in passive systemic venous blood flow directly to the pulmonary circulation. Resulting inevitable hepatic venous congestion can lead to hepatic fibrosis. Previous studies suggest hepatic changes can occur prior to the Fontan completion. Besides fibrotic myocardial remodeling may lead to systolic and diastolic ventricular dysfunction, transmitting back pressure to the pulmonary system. Purpose To compare quantitative T1 cardiovascular magnetic resonance (CMR) imaging of the myocardium and liver between SV patients and controls, as a potential measure of myocardial and hepatic fibrosis. Methods Retrospective review of 16 SV patients with dominant single left ventricle (SLV, n=6) or single right ventricle (SRV, n=10), at various stages of palliation (pre-Glenn=6, post-Glenn=3, Fontan=7) underwent CMR with myocardial T1 mapping with the liver also in the plane of view. Biventricular patients found to have structurally normal hearts and normal cardiac function on CMR were used as controls (n=21). Native T1 times using a modified Look-Locker inversion recovery (MOLLI) approach in free-wall of the dominant ventricle at a mid-ventricular short axis in SV and the ventricular septum in controls and, a region of interest in the liver (avoiding any vessels) were measured in all patients. Median and inter-quartile ranges of continuous variables were compared between SV and controls using the Mann-Whitney U test. Results As compared to controls SV patients were (1) significantly younger, (2) had lower ejection fraction, (3) higher median myocardial T1, and (4) higher median liver T1. Also, there was no difference between SLV vs. SRV median myocardial T1 (1056 vs. 1065ms, p=0.43) or liver T1 (678 vs. 729ms, p=0.30) Conclusion Despite younger age, findings of increased myocardial T1 may suggest an element of myocardial fibrosis responsible for the ventricular dysfunction in this population, and that raised liver T1 may be an earlier marker of liver fibrosis, which warrants further study. Results Variable SV Controls P-value Median IQR Median IQR Age (years) 1.6 0.42–11.0 12.0 9.0–15.0 0.003 EF (%) 47.0 43.5–52.8 59.5 56.4–62.5 0.012 Myocardial T1 (ms) 1066 1041–1078 1014 995–1032 0.0002 Liver T1 (ms) 705 654–759 606 581–634 0.0006 EF, ejection fraction; IQR, inter-quartile range; ms, milliseconds; sSV, single ventricle. Funding Acknowledgement Type of funding source: None