Fontan associated liver disease is associated with morbidity and mortality in palliated
single-ventricle congenital heart disease patients. Magnetic resonance elastography
(MRE) provides a quantitative assessment of liver stiffness in Fontan patients. We
hypothesized that MRE liver stiffness correlates with liver enzymes, hemodynamics,
portal hypertension, and Fontan failure (FF). All adult Fontan patients who had MRE
between 2011 and 2018 were included. Radiologic portal hypertension was defined as
splenomegaly, ascites, and/or varices. FF was defined as death, transplantation, or
heart failure symptoms requiring escalation of diuretics. Seventy patients with a
median age of 24.7 years and a median follow-up from MRE of 3.9 years were included.
The median liver stiffness was 4.3 kPa (interquartile range [IQR]: 3.8 to 5.0 kPa).
There was a weak, positive correlation between liver stiffness and Fontan pathway
pressure (r = 0.34, p = 0.03). There was a moderate negative correlation of liver
stiffness with ventricular ejection fraction (r = −0.52, p = 0.03). Liver stiffness
was weakly positively correlated with liver transaminases and gamma glutamyl transferase.
Patients with portal hypertension had higher liver stiffness compared to patients
without (5.2 ± 1.3 vs 4.2 ± 0.8 kPa, p = 0.03). At MRE or during follow-up, 13 patients
(19%) met definition of FF and had significantly higher liver stiffness compared to
patients without FF (5.1 [IQR: 4.3 to 6.3] vs 4.2 [IQR: 3.7 to 4.7] kPa, p = 0.01).
Liver stiffness above 4.5 kPa differentiated FF with a sensitivity of 77% and specificity
of 77%. In conclusion, elevated MRE-derived liver stiffness is associated with worse
hemodynamics, liver enzymes and clinical outcomes in Fontan patients. This measure
may serve as a global imaging biomarker of Fontan health.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to American Journal of CardiologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Fontan-associated liver disease: proceedings from the American College of Cardiology stakeholders meeting, October 1 to 2, 2015, Washington DC.J Am Coll Cardiol. 2017; 70: 3173-3194
- Factors associated with long-term mortality after Fontan procedures: a systematic review.Heart. 2017; 103: 104-110
- Clinical phenotypes of Fontan failure: implications for management.Congenit Heart Dis. 2016; 11: 296-308
- Fontan-associated liver disease: a review.J Cardiol. 2019; 74: 223-232https://doi.org/10.1016/j.jjcc.2019.02.016
- Hepatic changes in the failing Fontan circulation.Heart (British Cardiac Society). 2007; 93: 579-584
- Fontan liver disease: review of an emerging epidemic and management options.Curr Treat Options Cardiovasc Med. 2015; 17: 51
- End-organ consequences of the Fontan operation: liver fibrosis, protein-losing enteropathy and plastic bronchitis.Cardiol Young. 2013; 23: 831-840
- The clinical spectrum of Fontan-associated liver disease: results from a prospective multimodality screening cohort.Eur Heart J. 2019; 40: 1057-1068
- Evaluation of Fontan liver disease: correlation of transjugular liver biopsy with magnetic resonance and hemodynamics.Cong Heart Dis. 2019; 14: 600-608https://doi.org/10.1111/chd.1277
- Percutaneous liver biopsy in Fontan patients.Pediatr Radiol. 2019; 49: 342-350
- Assessment of liver T1 mapping in fontan patients and its correlation with magnetic resonance elastography-derived liver stiffness.Abdom Radiol (NY). 2019;
- A composite noninvasive index correlates with liver fibrosis scores in post-Fontan patients: preliminary findings.Cong Heart Dis. 2018; 13: 38-45
- Defining the role of liver biopsy in the assessment of liver fibrosis in patients with Fontan circulation-reply.Human Path. 2017; 69: 141
- Relationship of MR elastography determined liver stiffness with cardiac function after Fontan palliation.J Magn Reson Imaging. 2014; 40: 1328-1335
- Hepatic changes in the Fontan circulation: identification of liver dysfunction and an attempt to streamline follow-up screening.Pediatr Cardiol. 2018; 39: 1604-1613
- Hemodynamics of Fontan failure: the role of pulmonary vascular disease.Circ Heart Fail. 2017; : 10
- Evaluation of hemostatic and coagulation factor abnormalities in patients undergoing the Fontan operation.J Thoracic Cardiovasc Surg. 2000; 120: 778-782
- Protein C activity and postoperative metabolic liver function after liver transplantation.Transplant Proc. 2012; 44: 1336-1340
- Comparison of gradient-recalled echo and spin-echo echo-planar imaging MR elastography in staging liver fibrosis: a meta-analysis.Eur Radiol. 2018; 28: 1709-1718
- MRI measured liver stiffness does not predict focal liver lesions after the Fontan operation.Pediatr Radiol. 2019; 49: 99-104
- Spin-echo echo-planar imaging MR elastography versus gradient-echo MR elastography for assessment of liver stiffness in children and young adults suspected of having liver disease.Radiology. 2017; 282: 761-770
- Temporal changes in liver stiffness after Fontan operation: results of serial magnetic resonance elastography.Int J Cardiol. 2018; 258: 299-304
- Transient elastography may identify Fontan patients with unfavorable hemodynamics and advanced hepatic fibrosis.Cong Heart Dis. 2014; 9: 438-447
- Liver disease in patients after the Fontan operation.Am J Cardiol. 2016; 117: 456-460
- Ultrasound-based transient elastography for the detection of hepatic fibrosis: systematic review and meta-analysis.Clin Gastroenterol Hepatol. 2007; 5: 1214-1220
- FibroSURE and elastography poorly predict the severity of liver fibrosis in Fontan-associated liver disease.Cong Heart Dis. 2018; 13: 764-770
- Risk factors and serological markers of liver cirrhosis after Fontan procedure.Heart Vessels. 2016; 31: 1514-1521
- Magnetic resonance elastography of liver: clinical applications.J Comput Assist Tomogr. 2013; 37: 887-896
Article info
Publication history
Published online: August 07, 2019
Received in revised form:
July 24,
2019
Received:
June 8,
2019
Footnotes
Funding: Academic and research committee of Cincinnati Children's Hospital grant support for the Fontan multidisciplinary clinic.
Identification
Copyright
© 2019 Elsevier Inc. All rights reserved.
