Hepatic and Renal Consequences of Single-Ventricle Physiology Palliated With the Fontan Operation

Rathgeber, Steven L.; Lam, C.; Harris, Kevin C.; GrewalMD, Jasmine

doi:10.1016/j.cjca.2022.04.022

Cited by 9 publications

(11 citation statements)

References 49 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The pathologic changes include findings of passive congestion and fibrosis and are associated with development of benign and malignant masses. Biopsy data in all Fontan patients consistently demonstrate liver pathology across all patient ages, with severity increasing from the time of Fontan palliation 6 . Liver injury may become severe in adulthood with some patients requiring simultaneous heart and liver transplantation due to advanced liver disease, portal hypertension, or hepatocellular carcinoma (HCC) 9 …”

Section: Pathophysiology and Clinical Features Of Fontan-associated L...mentioning

confidence: 96%

“…Biopsy data in all Fontan patients consistently demonstrate liver pathology across all patient ages, with severity increasing from the time of Fontan palliation. 6 Liver injury may become severe in adulthood with some patients requiring simultaneous heart and liver transplantation due to advanced liver disease, portal hypertension, or hepatocellular carcinoma (HCC). 9 The source of injury to the liver in the Fontan patient is thought to be complex and influenced by hypoxemic conditions even before the Fontan is performed.…”

Section: Pathophysiology and Clinical Features Of Fontan-associated L...mentioning

confidence: 99%

“…Portal inflow is further decreased by the low cardiac output often found in Fontan patients. The liver maintains some ability to compensate for the decrease in portal flow via the hepatic artery buffer; however, it is vulnerable to ischemia in times of decreased cardiac output 6,12 …”

Section: Pathophysiology and Clinical Features Of Fontan-associated L...mentioning

confidence: 99%

“…The liver maintains some ability to compensate for the decrease in portal flow via the hepatic artery buffer; however, it is vulnerable to ischemia in times of decreased cardiac output. 6,12 Hepatic fibrosis occurs universally in the Fontan population. 13 Because of the multifactorial sources of liver injury, a combination of portal, centrilobular, and sinusoidal patterns of fibrosis is often found at pathology.…”

Section: Pathophysiology and Clinical Features Of Fontan-associated L...mentioning

confidence: 99%

“…5 However, the Fontan circulation is associated with elevated central venous pressure and decreased cardiac output, which predisposes patients to multiple long-term health consequences, a topic gaining interest as survival increases post-Fontan surgery. 4,6 Long-term cardiac complications include arrhythmias, valvular insufficiency, and heart failure. 4 In addition, dysfunction of other organs is common with patients developing plastic bronchitis, thromboembolic disease, venous insufficiency, and protein-losing enteropathy.…”

mentioning

confidence: 99%

See 4 more Smart Citations

Imaging of Fontan-Associated Liver Disease

Brown

Kolbe

Hull

et al. 2023

J Comput Assist Tomogr

View full text Add to dashboard Cite

The Fontan procedure is the definitive treatment for patients with single-ventricle physiology. Surgical advances have led to a growing number of patients surviving into adulthood. Fontan-associated liver disease (FALD) encompasses a spectrum of pathologic liver changes that occur secondary to altered physiology including congestion, fibrosis, and the development of liver masses. Assessment of FALD is difficult and relies on using imaging alongside of clinical, laboratory, and pathology information. Ultrasound, computed tomography, and magnetic resonance imaging are capable of demonstrating physiologic and hepatic parenchymal abnormalities commonly seen in FALD. Several novel imaging techniques including magnetic resonance elastography are under study for use as biomarkers for FALD progression. Imaging has a central role in detection and characterization of liver masses as benign or malignant. Benign FNH-like masses are commonly encountered; however, these can display atypical features and be mistaken for hepatocellular carcinoma (HCC). Fontan patients are at elevated risk for HCC, which is a feared complication and has a poor prognosis in this population. While imaging screening for HCC is widely advocated, no consensus has been reached regarding an optimal surveillance regimen.

show abstract

Section: Pathophysiology and Clinical Features Of Fontan-associated L...mentioning

confidence: 96%

Section: Pathophysiology and Clinical Features Of Fontan-associated L...mentioning

confidence: 99%

Section: Pathophysiology and Clinical Features Of Fontan-associated L...mentioning

confidence: 99%

Section: Pathophysiology and Clinical Features Of Fontan-associated L...mentioning

confidence: 99%

mentioning

confidence: 99%

See 3 more Smart Citations