Karina V. Bunting^1,2,*, Francesco Formisano³, Jennifer Green¹, Richard P. Steeds^1,2, Lucy Hudsmith¹, Paul Clift^1,2

Congenital Heart Disease, Vol.15, No.2, pp. 89-100, 2020, DOI:10.32604/CHD.2020.011376

Abstract Objective: To determine the accuracy of assessing univentricular function in adult Fontan patients using three-dimensional (3D) volumetric echocardiography. Design: A prospective observational study in an adult Fontan patient cohort. Setting: University Hospitals Birmingham, NHS Foundation Trust. Patients: 26 patients were enrolled in the study all aged over 18 years, possessing the Fontan anatomy, with no contraindications to Cardiac Magnetic Resonance (CMR) imaging and in sinus rhythm. Intervention: All patients underwent transthoracic echocardiography using a Philips EPIQ 7 and X5-1 transducer. End diastolic volume (EDV), end systolic volume (ESV), stroke volume (SV) and ejection fraction (EF) were obtained using two dimensional… More >

Matthew J. Lewis¹, Elizabeth Hecht², Jonathan Ginns¹, Joshua Benton², Martin Prince², Marlon S. Rosenbaum¹

Congenital Heart Disease, Vol.12, No.2, pp. 153-158, 2017, DOI:10.1111/chd.12422

Abstract Background: The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified.
Methods and Results: A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of clinically guided CMR. Two radiologists… More >

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 >

Justin Georgekutty¹, Amir Kazerouninia², YunFei Wang³, Peter R. Ermis⁴, Dhaval R. Parekh⁴, Wayne J. Franklin⁴, Wilson W. Lam⁴

Congenital Heart Disease, Vol.13, No.4, pp. 541-547, 2018, DOI:10.1111/chd.12603

Abstract Objective: Adult Fontan patients are at increased risk for thrombosis and thromboembolic complications leading to increased morbidity and mortality. Most are prescribed antiplatelet or anticoagulant therapy for thromboprophylaxis; novel oral anticoagulants (NOACs) are uncommonly used given lack of data on their use in this population and generalized concerns regarding Fontan patients’ abnormal coagulation. We report the largest single-center experience with the use of NOACs for treatment and prophylaxis of thrombosis and thromboembolism in adult Fontan patients.
Results: A retrospective chart review identified 21 patients (11 female, 10 male), median age 33 years (18-50) at first initiation, who were prescribed a… 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 >