Abstract
We hereby report on the case of F.C., a 17 years old male patient, who was initially referred to our Unit on the advice of his cardiologist at a tertiary referral center. He presented with an anatomic single ventricle, also known as univentricular heart, a condition known since birth. The univentricular heart is defined as the presence of only one well-developed ventricle and the other rudimentary ventricle (if present) with less than 30% of its expected volume. The patient underwent a first surgical procedure by Glenn in 2005, followed by Fontan surgery in 2008. The Fontan procedure, firstly described as a surgical palliation for a single ventricle with tricuspid atresia, has become the standard operation for all patients with single-ventricle physiology (e.g., hypoplastic left-heart syndrome, pulmonary atresia, unbalanced atrio-ventricular canal defects) [1–4].
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Rychik J, Atz AM, Celermajer DS, Gatzoulis MA, Gewillig MH, Hsia MA, et al. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the american heart association. Circulation. 2019; CIR0000000000000696.
Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019;73:e81.
Driscoll DJ. Long-term results of the Fontan operation. Pediatr Cardiol. 2007;28:438.
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26:240.
Shafer KM, Garcia JA, Babb TG, et al. The importance of the muscle and ventilatory blood pumps during exercise in patients without a subpulmonary ventricle (Fontan operation). J Am Coll Cardiol. 2012;60:2115.
Dunn GD, Hayes P, Breen KJ, Schenker S. The liver in congestive heart failure: a review. Am J Med Sci. 1973;265:174.
Wells ML, Fenstad ER, Poterucha JT, Hough DM, Young PM, Araoz PA, et al. Imaging findings of congestive hepatopathy. Radiographics. 2016;36:1024.
Dogan Y, Soylu A, Kilickesmez O, Demirtas T, Kilickesmez TA, Dogan SN, et al. The value of hepatic diffusion-weighted MR imaging in demonstrating hepatic congestion secondary to pulmonary hypertension. Cardiovasc Ultrasound. 2010;8:28.
Elsayes KM, Shaaban AM, Rothan SM, Javadi BL, Castillo RP, et al. A comprehensive approach to hepatic vascular disease. Radiographics. 2017;37:813.
Lemmer A, Van Wagner LB, Ganger D. Assessment of advanced liver fibrosis and the risk for hepatic decompensation in Patients with Congestive Hepatopathy. Hepatology. 2018;68:1633.
Kutty S, Peng Q, Danford D, Fletcher SE, Perry D, Talmon GA, et al. Increased hepatic stiffness as consequence of high hepatic afterload in the fontan circulation: a vascular doppler and elastography study. Hepatology. 2014;59:251–60.
Téllez L, Rodríguez de Santiago E, Minguez B, Payance A, Clemente A, Baiges A, et al. Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: The VALDIG Fonliver prospective cohort. J Hepatol. 2020;72(4):702–10.
Schleiger A, Salzmann M, Kramer P, Danne F, Schubert S, Bassir C, et al. Severity of Fontan-associated liver disease correlates with fontan hemodynamics. Pediatr Cardiol. 2020;41(4):736–46.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
1 Electronic Supplementary Material
Transient elastography (Fibroscan®): the two video sequences show the measurement of liver and spleen stiffness by means of dedicated probes (SSM@50Hz and SSM@100Hz respectively) (MP4 35066 kb)
Point shear-wave elastography of the liver and spleen (ElastPQ®) and shear-wave elastography of the liver (ElastQ®) (MP4 95028 kb)
Rights and permissions
Copyright information
© 2021 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Costantino, A., Fraquelli, M., Chessa, M., La Mura, V. (2021). Case 3: Congestive Hepatopathy with High Liver and Spleen Stiffness in a 17 Years Old Male Patient. In: Fraquelli, M. (eds) Elastography of the Liver and Beyond. Springer, Cham. https://doi.org/10.1007/978-3-030-74132-7_20
Download citation
DOI: https://doi.org/10.1007/978-3-030-74132-7_20
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-74131-0
Online ISBN: 978-3-030-74132-7
eBook Packages: MedicineMedicine (R0)