Prevalence and characterization of fibrosis in surveillance liver biopsies of patients with Fontan circulation

Surrey, Lea F.; Russo, Pierre; Rychik, Jack; Goldberg, David J.; Dodds, Kathryn; O’Byrne, Michael L.; Glatz, Andrew C.; Rand, Elizabeth B.; Lin, Henry C.

doi:10.1016/j.humpath.2016.07.006

Cited by 93 publications

(102 citation statements)

References 29 publications

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“…Use of a standardized protocol of reporting may be helpful in maintaining consistency of reporting. Our US results and the findings of others (23, 24) suggest that this test may be useful in following patients post-Fontan with the detection of portal hypertension or nodules, for example, being an indication for additional investigations and referral to a hepatologist.…”

Section: Discussionsupporting

confidence: 75%

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: Discussionsupporting

confidence: 75%

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 is highly prevalent, as these patients are exposed to systemic venous hypertension, hepatic congestion, and low cardiac output leading to various stages of liver disease ranging from clinically silent cirrhosis to hepatocellular carcinoma . A recent study found that adults with Fontan palliation universally have biopsy‐proven hepatic fibrosis despite normal synthetic function . How to best proceed with heart vs combined heart‐liver transplant remains a controversial and under studied area.…”

Section: Discussionmentioning

confidence: 99%

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

et al. 2020

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Introduction Treatment of adult congenital heart disease patients who require advanced therapies remains challenging due to high perioperative and wait‐list mortality and limited donors. Patients palliated with Fontan are at the highest risk of early mortality due to multiorgan involvement and few centers able to safely transplant them. We sought to evaluate the early outcomes of heart transplants in these adult Fontan patients. Methods Using the Nationwide Inpatient Sample database, we identified all adults aged at least 18 years old who underwent heart transplantation across U.S. hospitals from 2004 to 2014. We then identified those with specific ICD‐9 codes to include tricuspid atresia, hypoplastic left heart syndrome and common ventricle. Multivariate regression models were created to adjust for potential confounders. Results A total of 93 Fontan patients underwent heart transplant during the study time (0.5% of all heart transplants). Compared to non‐Fontan heart transplantations, Fontan patients were younger, with a higher incidence of liver disease and coagulopathy. Fontan patients receiving heart transplant had higher mortality during transplant hospitalization compared to non‐Fontan patients (26.3% vs 5.3% OR, 18.10, CI, 5.06‐65.0 P < .001). Extracorporeal membrane oxygenator (ECMO) usage and bleeding were also higher in the Fontan cohort with an OR of 5.30 (P = .016) and 5.32 (P = .015) for ECMO and bleeding, respectively. The remaining outcomes were similar for both cohorts. Conclusion Adults with Fontan palliation undergoing heart transplantation have exceptionally high inpatient mortality, which is nearly five times that of non‐Fontan heart transplant recipients, perhaps related to a delayed referral, surgical complexity, and coexistent, underrecognized liver failure.

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“…95 This may explain the poor correlation that has been described between histology and imaging findings, laboratory studies, and clinical outcomes in several studies of liver disease in patients with Fontan physiology. 94,95,99 Because of this, routine liver biopsy remains controversial, and consultation with a hepatologist may be helpful to weigh the pros and cons. However, targeted liver biopsy still plays an important role in the evaluation of nodules seen on hepatic imaging in patients with liver disease resulting from CHD because differences in hepatic blood flow from the patient's hemodynamic abnormalities may affect contrast uptake and enhancement and make accepted imaging characteristics of malignant nodules on CT and MR less reliable in this population.…”

Section: Managementmentioning

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

176

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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Prevalence and characterization of fibrosis in surveillance liver biopsies of patients with Fontan circulation

Cited by 93 publications

References 29 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

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

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

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