Abstract
Purpose
Patients with a Fontan circulation tend to develop liver fibrosis, liver cirrhosis and even hepatocellular carcinoma. A noninvasive ultrasound technique for liver fibrosis and cardiac function assessment in Fontan-associated liver disease (FALD) is needed to evaluate disease progression in real time. This study aimed to evaluate whether hepatic vein (HV) waveform analysis and elastography could be alternative markers to cardiac index (CI) in patients with FALD and assess factors influencing elastography measurements in FALD cases.
Methods
All patients underwent cardiac catheterization, B-mode ultrasound and ultrasound elastography measurement. Moreover, we measured serum markers related to fibrosis and examined HV blood flow using duplex Doppler ultrasonography.
Results
Forty-three patients (median age, 17 years; interquartile range, 12–25 years; 29 men, 6 with liver biopsy) were enrolled. The real-time tissue elastography (RTE) value was significantly higher in patients who underwent surgery > 7 years prior, suggesting that this value probably reflects the liver fibrosis due to FALD from the early fibrosis stage. The ultrasound elastography did not significantly correlate with hemodynamic parameters. The area under the receiver operating curve for the diagnosis of CI < 2.2 L/min/m2 using HV waveform was superior to the results from elastography and calculated fibrosis indices.
Conclusion
HV waveform can be used as a noninvasive measurable surrogate marker for CI. The RTE value increased overtime after the operation and would reflect liver fibrosis. The combination of RTE and HV waveform type could be useful noninvasive tools to evaluate clinical conditions in FALD patients in real time.
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Acknowledgments
This work was partly supported by Japan Society for the Promotion of Science (JSPS) KAKENHI Grant Number 16K19825 to Y.K., 18K07634 to M.H. and JP18K08007 to Y.H.
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Yohei Koizumi, Masashi Hirooka, Takaaki Tanaka, Takao Watanabe, Osamu Yoshida, Yoshio Tokumoto, Takashi Higaki, Mariko Eguchi, Masanori Abe and Yoichi Hiasa declare that they have no conflicts of interest.
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All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1964 and later versions. This study was approved by the Ethical Review Board of Ehime University (permit number: 1607006). Informed consent to be included in the study was obtained from all patients.
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Koizumi, Y., Hirooka, M., Tanaka, T. et al. Noninvasive ultrasound technique for assessment of liver fibrosis and cardiac function in Fontan-associated liver disease: diagnosis based on elastography and hepatic vein waveform type. J Med Ultrasonics 48, 235–244 (2021). https://doi.org/10.1007/s10396-020-01078-8
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DOI: https://doi.org/10.1007/s10396-020-01078-8