Jessica L. Schachter¹, Manisha Patel², Samuel R. Horton³, A. Mike Devane⁴, Alex Ewing⁵, Gary A. Abrams⁶

Congenital Heart Disease, Vol.13, No.5, pp. 764-770, 2018, DOI:10.1111/chd.12650

Abstract Objective: As the population of patients with Fontan circulation surviving into adult‐ hood increases, hepatic cirrhosis has grown to be a significant cause of morbidity and mortality. Early detection of advanced hepatic fibrosis is imperative for proper inter‐ vention and consideration for heart or combined heart/liver transplantation. Noninvasive biomarkers and elastography have been evaluated for their diagnostic utility with variable results in the Fontan population.
Design: The cohort included 14 patients age 26.4 SD 7.5 who underwent Fontan surgery. All patients were evaluated with FibroSURE, shear wave elastography (SWE), hepatic duplex sonography, and liver biopsy. Liver fibrosis on biopsy was… More >

Michael V. Di Maria¹, Lindsey Silverman², Adel K. Younoszai¹, Mariana L. Meyers³, Kelley E. Capocelli⁴, Michael R. Narkewicz⁵

Congenital Heart Disease, Vol.13, No.3, pp. 444-449, 2018, DOI:10.1111/chd.12594

Abstract Background: The current management paradigm for children with single ventricle heart disease involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results in a high central venous pressure, and in the setting of single ventricle heart disease, results in hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse (ARFI), has been utilized in this cohort. The effect of poor cardiovascular efficiency, as measured by cardiopulmonary… More >

William N. Evans^1,2, Ruben J. Acherman^1,2, Michael L. Ciccolo^1,3, Sergio A. Carrillo^1,2, Alvaro Galindo^1,2, Abraham Rothman^1,2, Gary A. Mayman^1,2, Elizabeth A. Adams^1,2, Leigh C. Reardon^1,4, Brody J. Winn⁵, Noel S. Yumiaco⁵, Lesley Shimuizu¹, Yoko Inanaga¹, Rowena J. Deleon¹, Humberto Restrepo^1,2

Congenital Heart Disease, Vol.13, No.1, pp. 38-45, 2018, DOI:10.1111/chd.12558

Abstract Objective: We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements, either alone or in combination with other noninvasive variables, might correlate with liver-biopsy fibrosis scores in patients post-Fontan.
Methods: Between March 2012 and February 2017, we identified patients post-Fontan that underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography. Utilizing the results of elastography, laboratory testing, and time post-Fontan, we constructed a composite Fontan hepatic index as a sum of elastography measurements in kilopascals, model for end-stage liver disease excluding INR scores, and the square root of the… More >

Masashi Nishida, Shingo Kubo, Yuma Morishita, Kosuke Nishikawa, Kazuyuki Ikeda, Toshiyuki Itoi, Hajime Hosoi

Congenital Heart Disease, Vol.14, No.6, pp. 1087-1093, 2019, DOI:10.1111/chd.12853

Abstract Objective: This study aims to investigate the changes in renal function and levels of urinary biomarkers before and after cardiac angiography in children with congenital heart disease (CHD).
Setting: Children with CHD are at a risk for kidney injury during contrast exposure in cardiac angiography.
Outcome Measures: We measured urinary protein, albumin, N‐acetyl‐β‐D‐glucosaminidase (NAG), β2‐microglobulin (BMG), and liver‐type fatty acid‐binding protein (L‐FABP) levels, as well as serum creatinine and cystatin C levels, before and after cardiac angiography in 33 children with CHD.
Results: No significant decrease was noted in either the creatinine‐based or cystatin C‐based estimated glomerular filtration rate at… More >

Alexander Lemmer¹, Lisa VanWagner^1,2, Zaira Gasanova³, Steve Helmke^4,5, Gregory T. Everson^4,5, Daniel Ganger¹

Congenital Heart Disease, Vol.14, No.6, pp. 978-986, 2019, DOI:10.1111/chd.12831

Abstract Background & Aims: Fontan surgery for single ventricle congenital heart disease leads to Fontan‐associated liver disease (FALD). Typical laboratory tests, imaging, and histopathology cannot predict clinical severity in FALD. HepQuant SHUNT is a proprietary serum test of hepatic function and physiology that has not yet been evaluated in FALD.
Methods: Fourteen adult FALD patients at a single urban tertiary care center who underwent a Fontan procedure in childhood received HepQuant SHUNT testing between September 2015 and April 2018. The HepQuant SHUNT disease severity index (DSI) assesses global liver function and physiology from systemic and portal hepatic filtration rates (HFRs, clearances… More >

Ryan D. Byrne¹, Angela J. Weingarten², Daniel E. Clark², Shi Huang³, Roman E. Perri⁴, Andrew E. Scanga⁴, Jonathan N. Menachem², Larry W. Markham⁵, Benjamin P. Frischhertz²

Congenital Heart Disease, Vol.14, No.5, pp. 765-771, 2019, DOI:10.1111/chd.12820

Abstract Setting: Fontan‐associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra‐cardiac dysfunction as measured by MELD and MELD‐XI.
Objective: We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD‐XI scores.
Design: Single‐center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function… More >

Natalie Bottega¹, Isabelle Malhamé², Liming Guo¹, Raluca Ionescu‐Ittu¹, Judith Therrien¹, Ariane Marelli¹

Congenital Heart Disease, Vol.14, No.5, pp. 735-744, 2019, DOI:10.1111/chd.12811

Abstract Background: The number of women with congenital heart disease (CHD) of reproductive age is increasing, yet a description of trends in pregnancy and delivery outcomes in this population is lacking.
Objective: To assess secular trends in pregnancy rates, delivery outcomes, and related health care utilization in the adult female CHD population in Quebec, Canada.
Methods: The Quebec CHD database was used to construct a cohort with all women with CHD aged 18‐45 years between 1992 and 2004. Pregnancy and delivery rates were determined yearly and compared to the general population. Secular trends in pregnancy and delivery rates were assessed with… More >

Emily M. Engelhardt¹, Andrew T. Trout^2,3, Rachel M. Sheridan⁴, Gruschen R. Veldtman⁵, Jonathan R. Dillman^2,3

Congenital Heart Disease, Vol.14, No.3, pp. 380-388, 2019, DOI:10.1111/chd.12730

Abstract Purpose: Patients who have undergone Fontan palliation of single ventricle physiol‐ ogy congenital heart disease are prone to developing focal liver lesions. In our experi‐ ence, the variety of lesions occurring in this population is greater than that described in the literature. The purpose of this study was to describe the breadth of biopsy‐ proven liver lesions in patients post–Fontan palliation of single ventricle physiology cared for at our institution.
Methods: We retrospectively identified patients who had previously undergone the Fontan operation and had a focal liver lesion biopsied between January 2000 and June 2018. Medical records were reviewed for… More >

Yuguo YI, Jiamin ZHENG, Yang ZHOU, Zhiqin LIU, Dan WENG^*

BIOCELL, Vol.44, No.1, pp. 7-11, 2020, DOI:10.32604/biocell.2020.08686

Abstract Nonalcoholic fatty liver disease (NAFLD) represents a huge threat to public health of the whole world. Around 25% of NAFLD patients will progress to nonalcoholic steatohepatitis (NASH), which has been predicted to be the main reason for liver transplantation in the United States in 2020. Extensive effort has been devoted to investigating the underlying molecular mechanisms of NASH pathogenesis and developing new promising treatments. Recent studies have demonstrated that pyroptosis, an inflammatory programmed cell death mediated by inflammasome and gasdermin-D (GSDMD), is involved in the development and progression of NASH. This review aims to summarize the recent findings regarding the… More >

Yi Shen¹, Victor S. Sheng^{1, 2, *}, Lei Wang¹, Jie Duan¹, Xuefeng Xi¹, Dengyong Zhang³, Ziming Cui¹

CMC-Computers, Materials & Continua, Vol.62, No.3, pp. 1233-1247, 2020, DOI:10.32604/cmc.2020.07450

Abstract Accurate segmentation of CT images of liver tumors is an important adjunct for the liver diagnosis and treatment of liver diseases. In recent years, due to the great improvement of hard device, many deep learning based methods have been proposed for automatic liver segmentation. Among them, there are the plain neural network headed by FCN and the residual neural network headed by Resnet, both of which have many variations. They have achieved certain achievements in medical image segmentation. In this paper, we firstly select five representative structures, i.e., FCN, U-Net, Segnet, Resnet and Densenet, to investigate their performance on liver… More >