Hepatocellular Carcinoma After Fontan Operation

Egbe, Alexander C.; Poterucha, Joseph T.; Warnes, Carole A.; Connolly, Heidi M.; Baskar, Shankar; Ginde, Salil; Clift, Paul; Kogon, Brian; Book, Wendy; Walker, Niki L.; Wagenaar, Lodewijk J.; Moe, Tabitha G.; Oechslin, Erwin; Kay, W. Aaron; Norris, Mark D.; Gordon‐Walker, Timothy T.; Dillman, Jonathan R.; Trout, Andrew T.; Anwar, Nadeem; Hoskoppal, Arvind; Veldtman, Gruschen

doi:10.1161/circulationaha.117.032717

Cited by 86 publications

(64 citation statements)

References 5 publications

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“…7,17 The most devastating complication of FALD remains hepatocellular carcinoma (HCC), and it has been previously recognized as a long-term complication of the Fontan procedure. 7,21 In a recent retrospective study, it was found that 1.3% of 2,470 cases (n=33) had biopsy-proven HCC, with the average age at time of diagnosis being 30 years, and the average length of time from Fontan operation to HCC diagnosis being 22 years. 21 Of the 33 patients identified, 18 (55%) died within 26 months.…”

Section: Hepatic Systemmentioning

confidence: 99%

“…7,21 In a recent retrospective study, it was found that 1.3% of 2,470 cases (n=33) had biopsy-proven HCC, with the average age at time of diagnosis being 30 years, and the average length of time from Fontan operation to HCC diagnosis being 22 years. 21 Of the 33 patients identified, 18 (55%) died within 26 months.…”

Section: Hepatic Systemmentioning

confidence: 99%

“…However, in contrast to other patients with HCC, only 50% of the singleventricle patients diagnosed with HCC previously carried a diagnosis of hepatic cirrhosis, in contrast to 80% of patients with two ventricles developing HCC. 21,22 This suggested either an underdiagnosis of hepatic cirrhosis in patients with Fontan circulation, or an increased risk of HCC in non-cirrhotic livers in these patients. Unfortunately, advanced tumor burden at time of diagnosis gives many patients only the option of palliative care.…”

Section: Hepatic Systemmentioning

confidence: 99%

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Single-Ventricle Physiology

Taranto

Moe

2020

US Cardiology Review

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Single-ventricle physiology occurs in patients with hypoplastic ventricular heart defects, either on the right or left, who have undergone stepwise palliation surgeries ending with the Fontan procedure. After Fontan completion, these patients are dependent on passive venous return to the pulmonary circulation. The implications of passive flow are potentially devastating to the patient. We discuss some of the basic changes to the patient’s experience after a Fontan procedure, as well as the common complications. We also touch on some of the emerging management strategies for the common complications.

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Section: Hepatic Systemmentioning

confidence: 99%

Section: Hepatic Systemmentioning

confidence: 99%

Section: Hepatic Systemmentioning

confidence: 99%

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Single-Ventricle Physiology

Taranto

Moe

2020

US Cardiology Review

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“…It is important to differentiate these nodules from hepatocellular carcinoma (HCC). A recent review found 33/2470 patients (1.3%) developed biopsy‐proven HCC at a median age of 30 years (interquartile range, 12‐53 years), 22 years (2‐36 years) after Fontan completion . Surgical management of these tumors is complicated by the high systemic venous pressure required to sustain an adequate preload and CO, which predisposes to bleeding during hepatectomy.…”

Section: Chd and The Livermentioning

confidence: 99%

“…A recent review found 33/2470 patients (1.3%) developed biopsy-proven HCC at a median age of 30 years (interquartile range, 12-53 years), 22 years (2-36 years) after Fontan completion. (26) Surgical management of these tumors is complicated by the high systemic venous pressure required to sustain an adequate preload and CO, which predisposes to bleeding during hepatectomy. Nonsurgical treatments for locoregional control may therefore be preferable, such as transarterial chemoembolization or radiofrequency ablation.…”

Section: Fontan Circulationmentioning

confidence: 99%

Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation

et al. 2019

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There is close interaction between the functions of the liver and heart affecting the presentation, diagnosis, and outcome of acute and chronic cardiac and liver disease. Conditions affecting both organ systems should be considered when proposing transplantation because the interaction between cardiac disease and liver disease has implications for diagnosis, management, selection for transplantation, and, ultimately, for longterm outcomes after liver transplantation (LT). The combination of cardiac and liver disease is well recognized in adults but is less appreciated in pediatric patients. The focus of this review is to describe conditions affecting both the liver and heart and how they affect selection and management of LT in the pediatric population.

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