Abstract:Background and aims
Protein‐losing enteropathy (PLE) after Fontan surgery carries significant morbimortality. Its pathophysiology and association with other Fontan complications are poorly understood. Our aims were to examine whether Fontan‐PLE is associated with greater liver damage and to assess the presence of systemic and intestinal inflammation.
Methods
Fontan patients with PLE and Fontan controls without PLE matched for age and Fontan surgery procedure were included. Data were prospectively compiled on b… Show more
“…10 ) [ 59 ]. Nonetheless, elastography results must be interpreted with caution in the Fontan population because, unlike in other forms of chronic liver disease, no validated cut-offs of severe liver fibrosis are available, especially in pediatrics [ 55 , 60 ]. Fig.…”
“…Both protein-losing enteropathy and plastic bronchitis represent the clinical manifestation of chronic venous congestion induced by lymphatic insufficiency and abnormal lymphatic channels [ 70 ]. Protein-losing enteropathy affects 3–18% of Fontan patients and is a life-threatening disease when proteins leak into the small bowel [ 22 , 60 ]. The pathophysiological mechanisms of protein-losing enteropathy in Fontan circulation are incompletely understood and seem to differ compared to other forms.…”
Section: Veno-lymphatic Complicationsmentioning
confidence: 99%
“…The pathophysiological mechanisms of protein-losing enteropathy in Fontan circulation are incompletely understood and seem to differ compared to other forms. In a recent study by Rodriguez et al [ 60 ], protein-losing enteropathy seemed to be associated with advanced liver disease and markers of inflammation and intestinal permeability. Protein-losing enteropathy diagnosis is made by the evidence of elevated fecal α1-antitrypsin in combination with serum hypoalbuminemia and clinical edemas.…”
The Fontan operation is performed for various cardiac lesions with single-ventricle physiology. The survival rate of Fontan patients is increasing for adolescents and young adults, with an expected 30-year survival of >80%. Medical health care providers including specialists in organ systems and pediatric radiologists need to improve their knowledge about the Fontan circulation and the various organ complications to monitor care. In this review the author explains the basic anatomical and functional features of Fontan palliation and gives an overview of the multiple long-term organ complications that might present in the pediatric population. These include decreased physical capacity, ventricular dysfunction, atrioventricular valve regurgitation and arrhythmia, as well as protein-losing enteropathy, plastic bronchitis, growth/bone composition disturbances, renal dysfunction, and the recently recognized and important liver fibrosis (Fontan-associated liver disease). Neuropsychological and behavioral deficits occur frequently. This review focuses on the key role of radiology in making the diagnosis of these complications, monitoring therapy and predicting outcomes in the pediatric age group. The author discusses how and when radiology is important in Fontan patients, as well as how new techniques enabling quantitative measures in imaging with US, MRI and CT are adapted for pediatric use, and how they contribute to urgently needed surveillance strategies.
Electronic supplementary material
The online version of this article (10.1007/s00247-020-04682-5) contains supplementary material, which is available to authorized users.
“…10 ) [ 59 ]. Nonetheless, elastography results must be interpreted with caution in the Fontan population because, unlike in other forms of chronic liver disease, no validated cut-offs of severe liver fibrosis are available, especially in pediatrics [ 55 , 60 ]. Fig.…”
“…Both protein-losing enteropathy and plastic bronchitis represent the clinical manifestation of chronic venous congestion induced by lymphatic insufficiency and abnormal lymphatic channels [ 70 ]. Protein-losing enteropathy affects 3–18% of Fontan patients and is a life-threatening disease when proteins leak into the small bowel [ 22 , 60 ]. The pathophysiological mechanisms of protein-losing enteropathy in Fontan circulation are incompletely understood and seem to differ compared to other forms.…”
Section: Veno-lymphatic Complicationsmentioning
confidence: 99%
“…The pathophysiological mechanisms of protein-losing enteropathy in Fontan circulation are incompletely understood and seem to differ compared to other forms. In a recent study by Rodriguez et al [ 60 ], protein-losing enteropathy seemed to be associated with advanced liver disease and markers of inflammation and intestinal permeability. Protein-losing enteropathy diagnosis is made by the evidence of elevated fecal α1-antitrypsin in combination with serum hypoalbuminemia and clinical edemas.…”
The Fontan operation is performed for various cardiac lesions with single-ventricle physiology. The survival rate of Fontan patients is increasing for adolescents and young adults, with an expected 30-year survival of >80%. Medical health care providers including specialists in organ systems and pediatric radiologists need to improve their knowledge about the Fontan circulation and the various organ complications to monitor care. In this review the author explains the basic anatomical and functional features of Fontan palliation and gives an overview of the multiple long-term organ complications that might present in the pediatric population. These include decreased physical capacity, ventricular dysfunction, atrioventricular valve regurgitation and arrhythmia, as well as protein-losing enteropathy, plastic bronchitis, growth/bone composition disturbances, renal dysfunction, and the recently recognized and important liver fibrosis (Fontan-associated liver disease). Neuropsychological and behavioral deficits occur frequently. This review focuses on the key role of radiology in making the diagnosis of these complications, monitoring therapy and predicting outcomes in the pediatric age group. The author discusses how and when radiology is important in Fontan patients, as well as how new techniques enabling quantitative measures in imaging with US, MRI and CT are adapted for pediatric use, and how they contribute to urgently needed surveillance strategies.
Electronic supplementary material
The online version of this article (10.1007/s00247-020-04682-5) contains supplementary material, which is available to authorized users.
“…Liver function is usually preserved for many years and the first manifestation of FALD frequently overlaps the dysfunction of another organ, such as protein-losing enteropathy or a drop in functional class. 22 As shown in ►Table 1, FALD comprises three main stages. 23 The first stage starts before Fontan surgery and continues into childhood.…”
Section: Natural History Of Fontan-associated Liver Diseasementioning
Fontan-associated liver disease is the term used to encompass the disorders arising from abnormal hemodynamic alterations and systemic venous congestion after the Fontan procedure. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease. While the natural history of this form of liver disease is poorly established, many Fontan patients ultimately develop portal hypertension-related complications such as ascites, esophageal varices, malnutrition, and encephalopathy. Fontan survivors also show an elevated risk of hepatocellular carcinoma. Adequate staging of the liver damage is essential to anticipate screening strategies and improve global management.
“…4,5 Thus, despite its limitations, liver biopsy remains the accepted gold standard for evaluating the degree of FALD-related hepatic fibrosis. 6 Nevertheless, frequent liver biopsy is impractical. An alternative to repetitive biopsy could be composite models comprised of factors that correlate with hepatic pathology.…”
Objective: We investigated a relationship between a composite index comprised of Fontan-circuit anatomical features and hepatic fibrosis scores from biopsy.Methods: We identified living extracardiac Fontan patients, ≥7 years old and ≥5 but <20 years postoperative, that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2020. We divided patients into anatomical groups and applied a risk score to each patient. We compared average anatomical risk scores with average hepatic total fibrosis scores by group.
Results:We identified 111 patients that met inclusion criteria. After excluding four patients, we assigned 107 to one of 12 anatomical variant groups (n ≥ 3). For the 107, the average age at liver biopsy was 14 ± 6 years old. Of the 107, 105 (98%)were New York Heart Association Class 1. We found average anatomical risk scores by group correlated with average total fibrosis scores by group (R = 0.8; p = .005). An average Fontan duration to biopsy of 10 ± 1 years was similar for all 12 anatomical groups. We found no other clinical variables, laboratory, or hemodynamic values that trended with anatomical risk scores or hepatic total fibrosis scores.Conclusions: In a cohort of relatively young, stable extracardiac Fontan patients, average composite anatomical risk scores strongly correlated with average hepatic total fibrosis scores by anatomical group. These findings suggest that some anatomical variants in extracardiac Fontan patients are associated with higher Fontanassociated liver disease (FALD)-related hepatic total fibrosis scores than others, despite similar Fontan durations.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
