Relation of Magnetic Resonance Elastography to Fontan Circulatory Failure in a Cohort of Pediatric and Adult Patients.
Adults
Children
Fontan failure
Fontan-associated liver disease
Magnetic resonance elastography
Portal hypertension
Journal
Pediatric cardiology
ISSN: 1432-1971
Titre abrégé: Pediatr Cardiol
Pays: United States
ID NLM: 8003849
Informations de publication
Date de publication:
Dec 2021
Dec 2021
Historique:
received:
28
04
2021
accepted:
04
08
2021
pubmed:
28
8
2021
medline:
3
11
2021
entrez:
27
8
2021
Statut:
ppublish
Résumé
Elevated magnetic resonance elastography (MRE)-derived liver stiffness may be associated with worse outcomes in people with Fontan circulation. We sought to evaluate the association between liver stiffness and Fontan failure or portal hypertension. Single center cross-sectional retrospective study of people with Fontan circulation who underwent MRE between 2011 and 2020. The cohort was divided into adult (age ≥ 21 years) and pediatric (< 21 years) groups. Fontan circulatory failure (FF) was defined as any of the following: death, transplantation, ventricular assist device, heart failure symptoms requiring escalation of diuretics. Radiologic portal hypertension was defined as the presence of one or more of the following: splenomegaly, ascites, or gastrointestinal varices. 128 patients were included (average age = 22.6 ± 8.7 years) and 58 (45%) were children. Median liver stiffness was 4.3 kPa (interquartile range (IQR) 3.8-5.8) for the entire cohort. Thirty patients (23%) developed FF (16 adults, 14 children). Liver stiffness was higher in adults with FF compared to those without FF (4.9 (IQR 4.0-6.0) vs. 4.2 (IQR 3.8-4.7) kPa, p = 0.04). There was no difference in liver stiffness between pediatric patients with and without FF (4.4 (IQR 4.1-5.4) vs. 4.4 (IQR 3.8-5.0), p = 0.5). Adults with radiologic portal hypertension and adults with moderate or severe atrioventricular valve regurgitation had higher liver stiffness than adults without. MRE-derived liver stiffness is associated with atrioventricular valve regurgitation, portal hypertension, and poor clinical outcomes in adults with Fontan circulation. There was no association between liver stiffness and FF in pediatric patients. This difference may be due to the progressive nature of Fontan-associated liver disease.
Identifiants
pubmed: 34448042
doi: 10.1007/s00246-021-02707-w
pii: 10.1007/s00246-021-02707-w
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1871-1878Informations de copyright
© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
Références
Fredenburg TB, Johnson TR, Cohen MD (2011) The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics 31:453–463. https://doi.org/10.1148/rg.312105027
doi: 10.1148/rg.312105027
pubmed: 21415190
Alsaied T, Bokma JP, Engel ME et al (2017) Factors associated with long-term mortality after Fontan procedures: a systematic review. Heart 103:104–110
doi: 10.1136/heartjnl-2016-310108
Book WM, Gerardin J, Saraf A et al (2016) Clinical phenotypes of Fontan failure: implications for management. Congenit Heart Dis 11:296–308. https://doi.org/10.1111/chd.12368
doi: 10.1111/chd.12368
pubmed: 27226033
Gordon-Walker TT, Bove K, Veldtman G (2019) Fontan-associated liver disease: a review. J Cardiol 74:223–232
doi: 10.1016/j.jjcc.2019.02.016
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. Journal of the American College of Cardiology. Elsevier USA, pp 3173–3194
Morisaka H, Motosugi U, Ichikawa S et al (2018) Magnetic resonance elastography is as accurate as liver biopsy for liver fibrosis staging. J Magn Reson Imaging 47:1268–1275. https://doi.org/10.1002/jmri.25868
doi: 10.1002/jmri.25868
pubmed: 29030995
Munsterman ID, Duijnhouwer AL, Kendall TJ et al (2019) The clinical spectrum of Fontan-associated liver disease: results from a prospective multimodality screening cohort. Eur Heart J 40:1057–1068. https://doi.org/10.1093/eurheartj/ehy620
doi: 10.1093/eurheartj/ehy620
pubmed: 30346512
Silva-Sepulveda JA, Fonseca Y, Vodkin I et al (2019) Evaluation of Fontan liver disease: correlation of transjugular liver biopsy with magnetic resonance and hemodynamics. Congenit Heart Dis 14:600–608. https://doi.org/10.1111/chd.12770
doi: 10.1111/chd.12770
pubmed: 31038848
Ramachandran P, Serai SD, Veldtman GR et al (2019) Assessment of liver T1 mapping in fontan patients and its correlation with magnetic resonance elastography-derived liver stiffness. Abdom Radiol 44:2403–2408. https://doi.org/10.1007/s00261-019-01990-9
doi: 10.1007/s00261-019-01990-9
Evans WN, Acherman RJ, Ciccolo ML et al (2018) A composite noninvasive index correlates with liver fibrosis scores in post-Fontan patients: preliminary findings. Congenit Heart Dis 13:38–45. https://doi.org/10.1111/chd.12558
doi: 10.1111/chd.12558
pubmed: 29194972
Surrey LF, Russo P, Rychik J et al (2017) Defining the role of liver biopsy in the assessment of liver fibrosis in patients with Fontan circulation—reply. Hum Pathol 69:141
doi: 10.1016/j.humpath.2017.04.029
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. https://doi.org/10.1016/j.amjcard.2019.07.052
doi: 10.1016/j.amjcard.2019.07.052
pubmed: 31474329
Alsaied T, Moore RA, Lang SM et al (2020) Myocardial fibrosis, diastolic dysfunction and elevated liver stiffness in the Fontan circulation. Open Hear 7:1434. https://doi.org/10.1136/openhrt-2020-001434
doi: 10.1136/openhrt-2020-001434
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. https://doi.org/10.1161/JAHA.116.004809
doi: 10.1161/JAHA.116.004809
pubmed: 28947562
pmcid: 5634289
Egbe AC, Connolly HM, Niaz T et al (2017) Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation. Am Heart J 183:10–17. https://doi.org/10.1016/j.ahj.2016.09.014
doi: 10.1016/j.ahj.2016.09.014
pubmed: 27979032
Zafar F, Possner M, Trout AT et al (2020) Radiologic findings suggestive of portal hypertension may identify patients at risk for fontan failure. J Am Coll Cardiol 75:605. https://doi.org/10.1016/s0735-1097(20)31232-8
doi: 10.1016/s0735-1097(20)31232-8
Elder RW, Mccabe NM, Veledar E et al (2015) Risk factors for major adverse events late after fontan palliation. Congenit Heart Dis 10:159–168. https://doi.org/10.1111/chd.12212
doi: 10.1111/chd.12212
pubmed: 25130602
Diaz ES, Dillman JR, Veldtman GR, Trout AT (2019) MRI measured liver stiffness does not predict focal liver lesions after the Fontan operation. Pediatr Radiol 49:99–104. https://doi.org/10.1007/s00247-018-4264-z
doi: 10.1007/s00247-018-4264-z
pubmed: 30269160
Kim YS, Jang YN, Song JS (2018) Comparison of gradient-recalled echo and spin-echo echo-planar imaging MR elastography in staging liver fibrosis: a meta-analysis. Eur Radiol 28:1709–1718. https://doi.org/10.1007/s00330-017-5149-5
doi: 10.1007/s00330-017-5149-5
pubmed: 29164384
Serai SD, Dillman JR, Trout AT (2017) 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 282:761–770. https://doi.org/10.1148/radiol.2016160589
doi: 10.1148/radiol.2016160589
pubmed: 27715486
Trout AT, Anupindi SA, Gee MS et al (2020) Normal liver stiffness measured with MR elastography in children. Radiology 297:663–669. https://doi.org/10.1148/radiol.2020201513
doi: 10.1148/radiol.2020201513
pubmed: 32960728
Egbe AC, Poterucha JT, Warnes CA et al (2018) Hepatocellular carcinoma after fontan operation multicenter case series. Circulation 138:746–748
doi: 10.1161/CIRCULATIONAHA.117.032717
Dillman JR, Trout AT, Alsaied T et al (2020) Imaging of Fontan-associated liver disease. Pediatr Radiol 50:1528–1541
doi: 10.1007/s00247-020-04776-0
Schachter JL, Patel M, Horton SR et al (2018) FibroSURE and elastography poorly predict the severity of liver fibrosis in Fontan-associated liver disease. Congenit Heart Dis 13:764–770. https://doi.org/10.1111/chd.12650
doi: 10.1111/chd.12650
pubmed: 30101472
Talwalkar JA, Kurtz DM, Schoenleber SJ et al (2007) Ultrasound-based transient elastography for the detection of hepatic fibrosis: systematic review and meta-analysis. Clin Gastroenterol Hepatol 5:1214–1220. https://doi.org/10.1016/j.cgh.2007.07.020
doi: 10.1016/j.cgh.2007.07.020
pubmed: 17916549
Taniguchi T, Ohtani T, Kioka H et al (2019) Liver stiffness reflecting right-sided filling pressure can predict adverse outcomes in patients with heart failure. JACC Cardiovasc Imaging 12:955–964. https://doi.org/10.1016/j.jcmg.2017.10.022
doi: 10.1016/j.jcmg.2017.10.022
pubmed: 29361489
Piran S, Veldtman G, Siu S et al (2002) Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation 105:1189–1194. https://doi.org/10.1161/hc1002.105182
doi: 10.1161/hc1002.105182
pubmed: 11889012
Freedom RM, Hamilton R, Yoo SJ et al (2000) The Fontan procedure: analysis of cohorts and late complications. Cardiol Young 10:307–331. https://doi.org/10.1017/s1047951100009616
doi: 10.1017/s1047951100009616
pubmed: 10950328
Byrne RD, Weingarten AJ, Clark DE et al (2019) More than the heart: hepatic, renal, and cardiac dysfunction in adult Fontan patients. Congenit Heart Dis 14:765–771. https://doi.org/10.1111/chd.12820
doi: 10.1111/chd.12820
pubmed: 31282062
Singh S, Venkatesh SK, Wang Z et al (2015) Diagnostic performance of magnetic resonance elastography in staging liver fibrosis: a systematic review and meta-analysis of individual participant data. Clin Gastroenterol Hepatol 13:440-451.e6. https://doi.org/10.1016/j.cgh.2014.09.046
doi: 10.1016/j.cgh.2014.09.046
pubmed: 25305349