Imaging of Fontan-associated liver disease.
Children
Congestive hepatopathy
Fontan circulation
Fontan-associated liver disease
Liver
Magnetic resonance imaging
Single ventricle congenital heart disease
Ultrasound
Young adults
Journal
Pediatric radiology
ISSN: 1432-1998
Titre abrégé: Pediatr Radiol
Pays: Germany
ID NLM: 0365332
Informations de publication
Date de publication:
10 2020
10 2020
Historique:
received:
29
03
2020
accepted:
06
07
2020
revised:
01
06
2020
pubmed:
19
8
2020
medline:
16
6
2021
entrez:
19
8
2020
Statut:
ppublish
Résumé
The Fontan operation has dramatically altered the natural history of functionally single ventricle congenital heart disease. Patients who have undergone the Fontan operation are living longer and, thus, noncardiac morbidity resulting from the Fontan operation is increasingly being recognized. Fontan-associated liver disease (FALD), one of the chief morbidities following the Fontan operation, is believed to be a multifactorial process that manifests as hepatic congestion and fibrosis, portal hypertension, and development of focal liver lesions, including malignant tumors. This article reviews the imaging findings of FALD in the pediatric and young adult population, reviews the literature related to the imaging of FALD and discusses possible screening algorithms for this population. The need for further research to better understand the causes of FALD, to establish if early liver stiffness measurements (or their change over time) predict long-term outcomes and complications, and to define optimal liver screening procedures is highlighted.
Identifiants
pubmed: 32809067
doi: 10.1007/s00247-020-04776-0
pii: 10.1007/s00247-020-04776-0
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
1528-1541Références
Prakash A, Powell AJ, Geva T (2010) Multimodality noninvasive imaging for assessment of congenital heart disease. Circ Cardiovasc Imaging 3:112–125
pubmed: 20086225
Gewillig M, Brown SC (2016) The Fontan circulation after 45 years: update in physiology. Heart 102:1081–1086
pubmed: 27220691
pmcid: 4941188
Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248
pubmed: 5089489
pmcid: 1019078
de Leval MR, Kilner P, Gewillig M, Bull C (1988) Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg 96:682–695
pubmed: 3184963
Pekkan K, Dasi LP, de Zelicourt D et al (2009) Hemodynamic performance of stage-2 univentricular reconstruction: Glenn vs. hemi-Fontan templates. Ann Biomed Eng 37:50–63
pubmed: 18987974
Rychik J, Atz AM, Celermajer DS et al (2019) Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation 140:e234–e284
Akintoye E, Miranda WR, Veldtman GR et al (2019) National trends in Fontan operation and in-hospital outcomes in the USA. Heart 105:708–714
pubmed: 30377261
Iyengar AJ, Winlaw DS, Galati JC et al (2014) The Australia and New Zealand Fontan registry: description and initial results from the first population-based Fontan registry. Intern Med J 44:148–155
pubmed: 24393144
d'Udekem Y, Iyengar AJ, Cochrane AD et al (2007) The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation 116:I157–I164
pubmed: 17846297
Egbe AC, Poterucha JT, Warnes CA et al (2018) Hepatocellular carcinoma after Fontan operation. Circulation 138:746–748
pubmed: 30359134
Diller G-P, Kempny A, Alonso-Gonzalez R et al (2015) Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary Centre. Circulation 132:2118–2125
pubmed: 26369353
Rychik J, Veldtman G, Rand E et al (2012) The precarious state of the liver after a Fontan operation: summary of a multidisciplinary symposium. Pediatr Cardiol 33:1001–1012
pubmed: 22534759
pmcid: 3442163
Daniels CJ, Bradley EA, Landzberg MJ et al (2017) Fontan-associated liver disease: proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC. J Am Coll Cardiol 70:3173–3194
pubmed: 29268929
Pundi K, Pundi KN, Kamath PS et al (2016) Liver disease in patients after the Fontan operation. Am J Cardiol 117:456–460
pubmed: 26704027
DiPaola FW, Schumacher KR, Goldberg CS et al (2017) Effect of Fontan operation on liver stiffness in children with single ventricle physiology. Eur Radiol 27:2434–2442
pubmed: 27752831
Koehne de Gonzalez AK, Lefkowitch JH (2017) Heart disease and the liver: pathologic evaluation. Gastroenterol Clin N Am 46:421–435
Richter S, Mucke I, Menger MD, Vollmar B (2000) Impact of intrinsic blood flow regulation in cirrhosis: maintenance of hepatic arterial buffer response. Am J Physiol Gastrointest Liver Physiol 279:G454–G462
pubmed: 10915656
Kiesewetter CH, Sheron N, Vettukattill JJ et al (2007) Hepatic changes in the failing Fontan circulation. Heart 93:579–584
pubmed: 17005713
Rychik J, Goldberg D, Rand E et al (2013) End-organ consequences of the Fontan operation: liver fibrosis, protein-losing enteropathy and plastic bronchitis. Cardiol Young 23:831–840
pubmed: 24401255
Schwartz MC, Glatz AC, Daniels K et al (2015) Hepatic abnormalities are present before and early after the Fontan operation. Ann Thorac Surg 100:2298–2304
pubmed: 26343976
Schwartz MC, Sullivan L, Cohen MS et al (2012) Hepatic pathology may develop before the Fontan operation in children with functional single ventricle: an autopsy study. J Thorac Cardiovasc Surg 143:904–909
pubmed: 21955477
Ofei S, Gariepy C (2017) When the cause of liver disease is the heart. J Pediatr Gastroenterol Nutr 64:3–7
pubmed: 27540709
Gordon-Walker TT, Bove K, Veldtman G (2019) Fontan-associated liver disease: a review. J Cardiol 74:223–232
Goldberg DJ, Surrey LF, Glatz AC et al (2017) Hepatic fibrosis is universal following Fontan operation, and severity is associated with time from surgery: a liver biopsy and hemodynamic study. J Am Heart Assoc 6:e004809
pubmed: 28446492
pmcid: 5524062
Kim S-O, Lee S-Y, Jang S-I et al (2018) Hepatic stiffness using shear wave elastography and the related factors for a Fontan circulation. Pediatr Cardiol 39:57–65
pubmed: 28932977
Wallihan DB, Podberesky DJ (2013) Hepatic pathology after Fontan palliation: spectrum of imaging findings. Pediatr Radiol 43:330–338
pubmed: 23052733
Kim T-H, Yang HK, Jang H-J et al (2018) Abdominal imaging findings in adult patients with Fontan circulation. Insights Imaging 9:357–367
pubmed: 29623675
pmcid: 5991003
Alsaied T, Possner M, Lubert AM et al (2019) Relation of magnetic resonance elastography to Fontan failure and portal hypertension. Am J Cardiol 124:1454–1459
pubmed: 31474329
Kendall TJ, Stedman B, Hacking N et al (2008) Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study. J Clin Pathol 61:504–508
pubmed: 17965217
Sherlock S (1951) The liver in heart failure; relation of anatomical, functional, and circulatory changes. Br Heart J 13:273–293
pubmed: 14848381
pmcid: 479419
Surrey LF, Russo P, Rychik J et al (2016) Prevalence and characterization of fibrosis in surveillance liver biopsies of patients with Fontan circulation. Hum Pathol 57:106–115
pubmed: 27476041
Yin M, Glaser KJ, Manduca A et al (2017) Distinguishing between hepatic inflammation and fibrosis with MR Elastography. Radiology 284:694–705
pubmed: 28128707
pmcid: 5529282
Egbe A, Miranda WR, Connolly HM et al (2018) Temporal changes in liver stiffness after Fontan operation: results of serial magnetic resonance elastography. Int J Cardiol 258:299–304
pubmed: 29433966
Wallihan DB, Podberesky DJ, Marino BS et al (2014) Relationship of MR elastography determined liver stiffness with cardiac function after Fontan palliation. J Magn Reson Imaging 40:1328–1335
pubmed: 24408379
Elder RW, McCabe NM, Hebson C et al (2013) Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. Int J Cardiol 168:3764–3769
pubmed: 23849105
pmcid: 3805740
Anstee QM, Dhar A, Thursz MR (2011) The role of hypercoagulability in liver fibrogenesis. Clin Res Hepatol Gastroenterol 35:526–533
pubmed: 21570930
Alsaied T, Possner M, Brown N et al (2020) Lymphopenia in adults after the Fontan operation: prevalence and associations. Cardiol Young 30:641–648
pubmed: 32248856
Engelhardt EM, Trout AT, Sheridan RM et al (2019) Focal liver lesions following Fontan palliation of single ventricle physiology: a radiology-pathology case series. Congenit Heart Dis 14:380–388
pubmed: 30569564
Horvat N, Rocha MS, Chagas AL et al (2018) Multimodality screening of hepatic nodules in patients with congenital heart disease after Fontan procedure: role of ultrasound, ARFI elastography, CT, and MRI. AJR Am J Roentgenol 211:1212–1220
pubmed: 30247977
Diaz ES, Dillman JR, Veldtman GR et al (2019) MRI measured liver stiffness does not predict focal liver lesions after the Fontan operation. Pediatr Radiol 49:99–104
pubmed: 30269160
Tellez L, Rodriguez de Santiago E, Minguez B et al (2020) Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: the VALDIG Fonliver prospective cohort. J Hepatol 72:702–710
pubmed: 31726116
Ghaferi AA, Hutchins GM (2005) Progression of liver pathology in patients undergoing the Fontan procedure: chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thorac Cardiovasc Surg 129:1348–1352
pubmed: 15942576
Asrani SK, Warnes CA, Kamath PS (2013) Hepatocellular carcinoma after the Fontan procedure. N Engl J Med 368:1756–1757
pubmed: 23635071
Josephus Jitta D, Wagenaar LJ, Mulder BJ et al (2016) Three cases of hepatocellular carcinoma in Fontan patients: review of the literature and suggestions for hepatic screening. Int J Cardiol 206:21–26
pubmed: 26773766
Wells ML, Hough DM, Fidler JL et al (2017) Benign nodules in post-Fontan livers can show imaging features considered diagnostic for hepatocellular carcinoma. Abdom Radiol (NY) 42:2623–2631
American College of Radiology (2018) CT/MR LI-RADS® v2018 core. https://www.acr.org/-/media/ACR/Files/RADS/LI-RADS/LI-RADS-2018-Core.pdf?la=en . Accessed 29 Mar 2020
Di Maria MV, Brown DW, Cetta F et al (2019) Surveillance testing and preventive care after Fontan operation: a multi-institutional survey. Pediatr Cardiol 40:110–115
pubmed: 30159585
Schleiger A, Salzmann M, Kramer P et al (2020) Severity of Fontan-associated liver disease correlates with Fontan hemodynamics. Pediatr Cardiol 41:736–746
pubmed: 32006084
pmcid: 7256101