Key result
Myocardial ECV correlates with systemic ventricular end-diastolic pressure and liver stiffness in Fontan patients.
Why the study?
The relationship between adverse haemodynamics and end-organ fibrosis in adolescents and young adults with Fontan circulation had not been investigated.
Observational (n=25)
No
Effect estimate: r=0.42
p-value: p=0.03
In Fontan patients, myocardial fibrosis measured by cardiac MRI correlates with diastolic dysfunction, liver stiffness, and circulating profibrotic biomarkers, suggesting a systemic profibrotic milieu.
Myocardial ECV may mark systemic fibrosis in Fontan patients; hypothesis-generating and should not yet change practice.
INTRODUCTION: Single ventricle diastolic dysfunction and hepatic fibrosis are frequently observed in patients with a Fontan circulation. The relationship between adverse haemodynamics and end-organ fibrosis has not been investigated in adolescents and young adults with Fontan circulation. METHODS: Prospective observational study of Fontan patients who had a cardiac catheterisation. Cardiac MRI with T1 mapping was obtained to measure extracellular volume (ECV), a marker of myocardial fibrosis. Hepatic magnetic resonance elastography was performed to assess liver shear stiffness. Serum biomarkers of fibrosis including matrix metalloproteinases (MMPs) and tissue inhibitor of metalloproteinases (TIMPs) were measured. Very high ECV was defined as >30% and elevated serum biomarkers as >75th percentile for each biomarker. RESULTS: 25 Fontan patients (52% female) with mean age of 16.3±6.8 years were included. Mean ECV was 28%±5%. There was a significant correlation between ECV and systemic ventricular end-diastolic pressure (r=0.42, p=0.03) and between ECV and liver stiffness (r=0.45, p=0.05). Patients with elevated ECV demonstrated elevations in MMPs and TIMPs. Similarly, patients with elevated MMPs and TIMPs had greater liver stiffness compared with patients with normal levels of these biomarkers. CONCLUSIONS: In Fontan patients, cardiac magnetic resonance evidence of myocardial fibrosis is associated with diastolic dysfunction, increased liver stiffness and elevated circulating biomarkers of fibrosis. These findings suggest the presence of a profibrotic milieu, with end-organ implications, in some patients with Fontan circulation.
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Alsaied et al. (2020) conducted an observational in Fontan circulation (n=25). Myocardial extracellular volume (ECV) was evaluated on Correlation between extracellular volume (ECV) and baseline systemic ventricular end-diastolic pressure (r=0.42, p=0.03). In patients with Fontan circulation, myocardial extracellular volume significantly correlated with baseline systemic ventricular end-diastolic pressure (r=0.42, p=0.03) and liver stiffness (r=0.45, p=0.05).
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