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 >

Alessia Callegari^1,2,4, Rhoia Neidenbach², Ornella Milanesi¹, Biagio Castaldi¹, Martin Christmann⁴, Masamichi Ono³, Jan Müller², Peter Ewert², Alfred Hager²

Congenital Heart Disease, Vol.14, No.2, pp. 147-155, 2019, DOI:10.1111/chd.12694

Abstract Aim: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and qual‐ ity of life is unknown and objective of this study.
Methods: This multicenter retrospective/cross‐sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connec‐ tion). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assess‐ ment (SF‐36 questionnaire) were performed between 2003 and 2015.
Results: Overall, mean forced expiratory volume… More >

Neil C. Zaki^1,2, Michael S. Kelleman¹, W. James Parks^1,3, Timothy C. Slesnick^1,3, Michael E. McConnell^1,3, Matthew E. Oster^1,3

Congenital Heart Disease, Vol.14, No.2, pp. 140-146, 2019, DOI:10.1111/chd.12692

Abstract Objective: Gated cardiac MRI offers the most detailed and accurate noninvasive method of assessing cardiac anatomy, particularly in patients with complex congenital heart disease. The proposed benefits of using cMRI as a routine screening tool in the Fontan population include early recognition of asymptomatic, postoperative anatomic and physiologic changes. In 2011, we therefore instituted at our center a recommended practice of cMRI screening in patients with Fontan physiology at 3 and 8 years postFontan operation. The purpose of this study was to determine the impact of this standardized practice of cMRI screening on the clinical management of a Fontan population.
… More >

Emmanuel Akintoye¹, Gruschen R. Veldtman², William R. Miranda³, Heidi M. Connolly³, Alexander C. Egbe³

Congenital Heart Disease, Vol.14, No.2, pp. 138-139, 2019, DOI:10.1111/chd.12690

Abstract The purpose of this study was to determine the optimal age for performing Fontan operation using data from the National Inpatient Sample. Our results showed that although the Fontan operation was most commonly performed at age 2 in the United States, age 3 is the optimum age for this procedure as evident by lower rate of in‐hospital mortality, procedure‐related complications, and rate of nonroutine home discharge when procedure is performed at age 3 years. More >

Georges Ephrem¹, Camden Hebson², Anitha John³, Estella Moore⁴, Maan Jokhadar⁴, Ryan Ford⁵, Gruschen Veldtman⁶, Yoav Dori⁷, Michelle Gurvitz^8,9, Brian Kogon¹⁰, Adrienne Kovacs¹¹, Meghan Roswick, Michael McConnell¹², Wendy M. Book⁴, Fred Rodriguez III^4,12

Congenital Heart Disease, Vol.14, No.2, pp. 128-137, 2019, DOI:10.1111/chd.12685

Abstract The initial “Frontiers in Fontan Failure” conference in 2015 in Atlanta, Georgia, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with “Failing Fontan” physiology. Four types of “Fontan Failure” were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan proce‐ dure “imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation.” The purpose of the second “Frontiers in Fontan Failure” was to… More >

Camden Hebson^1,2, Wendy Book¹, Robert W. Elder³, Ryan Ford⁴, Maan Jokhadar¹, Kirk Kanter⁵, Brian Kogon⁵, Adrienne H. Kovacs⁶, Rebecca D. Levit¹, Michael Lloyd¹, Kevin Maher², Preeti Reshamwala⁴, Fred Rodriguez III^1,2, Rene Romero⁷, Thor Tejada¹, Anne Marie Valente⁸, Gruschen Veldtman⁹, Michael McConnell^1,2

Congenital Heart Disease, Vol.12, No.1, pp. 6-16, 2017

Abstract “Frontiers in Fontan Failure” was the title of a 2015 conference sponsored by Children’s Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions. More >

Kavitha N. Pundi¹, Krishna N. Pundi², Jonathan N. Johnson^1,3, Joseph A. Dearani⁴, Zhuo Li, BS⁵, David J. Driscoll¹, Philip L. Wackel¹, Christopher J. McLeod³, Frank Cetta^1,3, Bryan C. Cannon^1,3

Congenital Heart Disease, Vol.12, No.1, pp. 17-23, 2017

Abstract Objectives: We sought to examine the incidence and predictors of arrhythmias and sudden cardiac death (SCD) after Fontan operation.
Background: Arrhythmias and SCD have been reported following operations for congenital heart disease, but the incidence and risk factors have not been well defined in patients after a Fontan operation.
Methods: We reviewed records of all patients who had a Fontan operation from 1973 to 2012 (n 5 1052) at our institution. A questionnaire was mailed to patients who were not known to be deceased at the initiation of the study. Late arrhythmias were classified as bradyarrhythmias or tachyarrhythmias requiring treatment… More >

Hoi Lam She^*, Arno A.W. Roest^†, Emmeline E. Calkoen^†, Pieter J. van den Boogaard^‡, Rob J. van der Geest^‡, Mark G. Hazekamp^§, Albert de Roos^‡, Jos J.M. Westenberg^‡

Congenital Heart Disease, Vol.12, No.1, pp. 40-48, 2017

Abstract Purpose. To evaluate the inflow pattern and flow quantification in patients with functional univentricular heart after Fontan’s operation using 4D flow magnetic resonance imaging (MRI) with streamline visualization when compared with the conventional 2D flow approach.
Method. Seven patients with functional univentricular heart after Fontan’s operation and twenty-three healthy controls underwent 4D flow MRI. In two orthogonal two-chamber planes, streamline visualization was applied, and inflow angles with peak inflow velocity (PIV) were measured. Transatrioventricular flow quantification was assessed using conventional 2D multiplanar reformation (MPR) and 4D MPR tracking the annulus and perpendicular to the streamline inflow at PIV, and they… More >