M. Elisabeth Heal¹, Lanier B. Jackson², Andrew M. Atz², Ryan J. Butts³

Congenital Heart Disease, Vol.12, No.4, pp. 399-402, 2017, DOI:10.1111/chd.12451

Abstract Cardiopulmonary exercise testing (CPET) aids in clinical assessment of patients with Fontan circulation. Effects of persistent fenestration on CPET variables have not been clearly defined. Associations between fenestration and CPET variables at anaerobic threshold (AT) and peak exercise were explored in the Pediatric Heart Network Fontan Cross-Sectional Study cohort. Fenestration patency was associated with a greater decrease in oxygen saturation from rest to peak exercise (fenestration -4.9 ± 3.8 v. nonfenestration -3 ± 3.5; P < .001). Physiological dead space at peak exercise was higher in fenestrated v. nonfenestrated (25.2 ± 16.1 v. 21.4 ± 15.2; P = .03). There… More >

Moira B. Hilscher¹, Jonathan N. Johnson^2,3, Frank Cetta^2,3, David J. Driscoll^2,3, John J. Poterucha¹, William Sanchez¹, Heidi M. Connolly³, Patrick S. Kamath¹

Congenital Heart Disease, Vol.12, No.2, pp. 124-132, 2017, DOI:10.1111/chd.12446

Abstract The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population. 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 >

Keri M. Shafer^1,2, Alexander R. Opotowsky^1,2, Jonathan Rhodes¹

Congenital Heart Disease, Vol.13, No.6, pp. 903-910, 2018, DOI:10.1111/chd.12661

Abstract Objective: Risk prediction using cardiopulmonary exercise testing (CPET) in complex congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single institution over the same time period to determine CPET variables associated with mortality.
Design: All Fontan and tetralogy of Fallot (TOF) subjects with CPET between November 1, 2002 and December 31, 2014 and subsequently died were identified (cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and timing of exercise test.
Results: Of the 42 cases, 27 had a Fontan… More >

Sudheer R. Gorla¹, Nataley K. Jhingoeri¹, Abhishek Chakraborty¹, Kishore R. Raja¹, Ashish Garg¹, Satinder Sandhu¹, Eliot R. Rosenkranz², Sethuraman Swaminathan¹

Congenital Heart Disease, Vol.13, No.5, pp. 776-781, 2018, DOI:10.1111/chd.12652

Abstract Introduction: The Fontan operation is the final stage of single ventricle palliation in patients with complex congenital heart disease. Fenestration in the Fontan conduit, providing an atrial level right to left shunt, has been shown to reduce early postoperative morbidity. However, there is limited data on the long‐term fate of this fenestration. The aim of this study is to define the rate of spontaneous closure of the fenestration in the Fontan conduit and factors predictive of the fate of the fenestration.
Methods: This was a retrospective study reviewing the medical records of the patients who underwent fenestrated Fontan operation at… 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 >

Mi Kyoung Song¹, Gi Beom Kim¹, Eun Jung Bae¹, Young Ah Lee¹, Hyun-Young Kim², Seung-Kee Min³, Jung Hee Kim⁴, Jae-Kyung Won⁵

Congenital Heart Disease, Vol.13, No.4, pp. 608-616, 2018, DOI:10.1111/chd.12625

Abstract Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center.
Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children’s Hospital.
Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5%… More >

Christopher R. Broda¹, Hamsini Sriraman², Devanshi Wadhwa², YunFei Wang¹, Hari Tunuguntla¹, Ayse Akcan-Arikan^3,4, Peter R. Ermis¹, Jack F. Price¹

Congenital Heart Disease, Vol.13, No.4, pp. 602-607, 2018, DOI:10.1111/chd.12617

Abstract Purpose: Elevated central venous pressure (CVP) has deleterious effects on several organ systems in patients with Fontan circulation. However, the relationship between CVP and estimated glomerular filtration rate (eGFR) has not been assessed in patients with Fontan circulation.
Methods: Patients with Fontan circulation whose hemodynamics were assessed by catheterization between 1987 and 2015 and had a serum creatinine measured within 72 hours prior to the procedure were included for analysis. Patients with primary kidney disease were excluded. Renal function was calculated by “bedside Schwartz” equation in children (< 18 years) and Modification of Diet in Renal Disease equation in adults.… 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 >

Shaji C. Menon¹, Ragheed Al-Dulaimi¹, Brian W. McCrindle², David J. Goldberg³, Ritu Sachdeva⁴, Bryan H. Goldstein⁵, Thomas Seery⁶, Karen C. Uzark⁷, Anjali Chelliah⁸, Ryan Butts⁹, Heather Henderson¹⁰, Tiffanie Johnson¹¹, Richard V. Williams¹

Congenital Heart Disease, Vol.13, No.3, pp. 463-469, 2018, DOI:10.1111/chd.12597

Abstract Introduction: We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors.
Methods: This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL). Mixed effects regression modeling adjusting for clustering by center was used to evaluate factors associated with abnormal anthropometry and delayed puberty and associations with QoL.
Results: Of the 299 subjects, 42% were female.… More >