Liver health in adults with Fontan circulation: A multicenter cross-sectional study

Wu, Fred; Kogon, Brian; Earing, Michael G.; Aboulhosn, Jamil; Broberg, Craig S.; John, Anitha S.; Harmon, Amy; Sainani, Nisha I.; Hill, Andrew J.; Odze, Robert D.; Johncilla, Melanie; Ukomadu, Chinweike; Gauvreau, Kimberlee; Valente, Anne Marie; Landzberg, Michael J.

doi:10.1016/j.jtcvs.2016.10.060

Cited by 121 publications

(103 citation statements)

References 38 publications

(55 reference statements)

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“…Swartz et al reported 100% sinusoidal and 92% portal based fibrosis in biopsies of only 13 patients. A multicenter study by Wu et al examined 68 biopsies and found 97% with sinusoidal fibrosis and 90% with portal fibrosis . The congestive hepatic fibrosis score (CHS) does not address the clinically relevant range of portal fibrosis between central fibrosis (CHS 2B) and cirrhosis (CHS 4).…”

Section: Discussionmentioning

confidence: 99%

“…At the same time, early central fibrosis is not evaluated in traditional scoring systems, such as the Ishak score and METAVIR, which ignore specific characteristic of the Fontan liver. Due to the limitations of applying existing histologic scoring systems to Fontan associated liver disease, most studies examining liver biopsies have relied on reporting multiple scoring systems and even adding fibrosis scores when evaluating Fontan liver fibrosis . These studies highlight the importance of creating a common histologic scoring system for the Fontan population that considers the specific characteristics of the Fontan liver and also the use of noninvasive imaging to evaluate the Fontan liver disease.…”

Section: Discussionmentioning

confidence: 99%

“…Establishing the Fontan circuit results in elevated hepatic venous pressure and low cardiac output causing hepatic congestion, hypoxia, and decreased portal blood flow . These factors lead to a unique pattern of injury characterized by both sinusoidal and portal fibrosis which is distinctly different when compared to injury related to standard causes of chronic liver disease and other forms of congestive hepatopathy . Liver disease is associated with long‐term morbidity and mortality in this population and development of hepatocellular carcinoma has been reported as a consequence .…”

Section: Introductionmentioning

confidence: 99%

“…There is not a clear consensus in determining the most appropriate scoring system for the Fontan liver . Most studies examining liver biopsies have relied on reporting various scoring systems at different institutions and even adding fibrosis scores without taking into account the unique characters of the Fontan liver …”

Section: Introductionmentioning

confidence: 99%

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Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics

Silva-Sepulveda

Fonseca

Vodkin

et al. 2019

Congenital Heart Disease

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Introduction Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics. Methods Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic resonance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modified Ishak congestive hepatic fibrosis score. Results Median age 17.8 years, median time since Fontan 15.2 years. Primary diagnosis and Fontan type were variables, but predominantly LV morphology (30/49), lateral tunnel Fontan (29/49), originally fenestrated (37/49), and 11/49 had a pacemaker. Histologic fibrosis correlated with MRE (R = 0.62, P ≤ .001). Histologic fibrosis and MRE correlated with Fontan pressure (R = 0.38, P = .008 & R = 0.59, P ≤ .001). Morphology of the single ventricle did not correlate with liver fibrosis. The presence of a fenestration resulted in a higher cardiac index (P = .026) but did not resulted in lower liver fibrosis (P = .64). Conclusion Noninvasive tests, such as MRE, may be suitable for longitudinal follow‐up in patients with single ventricle physiology. Our data suggest that there is reasonable correlation of MRE liver stiffness with biopsy scoring systems and Fontan pressures. We demonstrated the feasibility of TJLB in the setting of Fontan palliation and demonstrated its correlation with noninvasive measures particularly MRE. We recommend selective use of TJLB when MRE score is >5 KPa or when there are other clinical signs of cirrhosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics

Silva-Sepulveda

Fonseca

Vodkin

et al. 2019

Congenital Heart Disease

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“…13 In addition, abnormal biomarkers of hepatic fibrosis and specimen-proven hepatic fibrosis are common in adults with Fontan circulation. Fontan Associated Liver Disease (FALD).…”

Section: Ac Tive Re S E Arch In Fontan Failure ( Anitha John MD mentioning

confidence: 99%

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary

Ephrem

Hebson

John

et al. 2018

Congenital Heart Disease

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The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, Georgia, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation." The purpose of the second "Frontiers in Fontan Failure" was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 "Frontiers in Fontan Failure: Innovation and Improving Outcomes" was sponsored by Children's Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic-related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to. K E Y W O R D S failure, Fontan, innovation, outcomes | 129 EphrEm Et al.

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Congestive hepatopathy: Differentiating congestion from fibrosis

Lemmer¹,

VanWagner²,

Ganger³

2017

Clinical Liver Disease

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Liver health in adults with Fontan circulation: A multicenter cross-sectional study

Cited by 121 publications

References 38 publications

Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics

Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary

Congestive hepatopathy: Differentiating congestion from fibrosis

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