Liver Disease in Patients After the Fontan Operation

Pundi, Krishna; Kamath, Patrick S.; Cetta, Frank; Li, Zhuo; Poterucha, Joseph T.; Driscoll, David J.; Johnson, Jonathan N.

doi:10.1016/j.amjcard.2015.11.014

Cited by 149 publications

(112 citation statements)

References 23 publications

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“…Since FALD appears to be a progressive disease with increasing severity over time (17), we compared results from children to a group of adult Fontan patients as a surrogate for time post-Fontan. We also examined differences between groups of patients who were greater or less than 10 years post-Fontan to identify markers that potentially change over time and could therefore indicate FALD progression.…”

Section: Discussionmentioning

confidence: 99%

Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients

Fidai

Dallaire

Alvarez

et al. 2017

Front. Cardiovasc. Med.

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Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8.4 ± 4.3 and 5.4 ± 4.1 years post-Fontan) and 8 adults (average age 31.5 ± 8.9 and 21.1 ± 4 years post-Fontan) using standard serum laboratory investigations assessing hepatic integrity and function, the FibroTest, liver ultrasound, and transient elastography (FibroScan). In adult Fontan patients, hemoglobin, C-reactive protein, and gamma-glutamyl transpeptidase were significantly increased, and white blood cell and platelet counts were significantly decreased in comparison to the pediatric cohort. International normalized ratio was mildly elevated in both children and adults. FibroTest results were suggestive of fibrosis regardless of time post-Fontan. FibroScan measurements were significantly correlated with time post-Fontan, but the incidence of ultrasound-detected liver abnormalities was variable. No cases of hepatocellular carcinoma were identified. Abnormalities suggestive of FALD occur in both children and adults post-Fontan. Select laboratory tests, and possibly ultrasound and FibroScan in some patients, appear to have the most promise for the non-invasive detection of FALD.

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Section: Discussionmentioning

confidence: 99%

Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients

Fidai

Dallaire

Alvarez

et al. 2017

Front. Cardiovasc. Med.

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“…A recent study reviewed liver data including histopathology and biochemistry 40. Cirrhosis was found to be a late complication with a high mortality (1-year, 43%; 2-year, 65%).…”

Section: Predictors Of Long-term Mortalitymentioning

confidence: 99%

Factors associated with long-term mortality after Fontan procedures: a systematic review

Alsaied

Bokma

Engel

et al. 2016

Heart

119

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“…[96][97][98][99] Epidemiologic data are limited in other CHD populations in which routine surveillance is uncommon and recognition therefore is less likely.…”

Section: -95mentioning

confidence: 99%

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Lui¹,

Saïdi²,

Bhatt³

et al. 2017

Circulation

175

136

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ABSTRACT:Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

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Liver Disease in Patients After the Fontan Operation

Cited by 149 publications

References 23 publications

Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients

Non-invasive Investigations for the Diagnosis of Fontan-Associated Liver Disease in Pediatric and Adult Fontan Patients

Factors associated with long-term mortality after Fontan procedures: a systematic review

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

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