Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.

Published on Aug 6, 2019in Circulation23.603
· DOI :10.1161/CIR.0000000000000696
Jack Rychik69
Estimated H-index: 69
,
Andrew M. Atz55
Estimated H-index: 55
+ 17 AuthorsYves d'Udekem45
Estimated H-index: 45
Sources
Abstract
It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array o...
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References425
Newest
#1Karen K. Stout (American Association for Thoracic Surgery)H-Index: 25
#2Curt J. Daniels (American Association for Thoracic Surgery)H-Index: 19
Last. George F. Van HareH-Index: 58
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Glenn N. Levine, MD, FACC, FAHA, Chair Patrick T. O’Gara, MD, MACC, FAHA, Chair-Elect Jonathan L. Halperin, MD, FACC, FAHA, Immediate Past Chair Nancy M. Albert, PhD, RN, FAHA[§§][1] Sana M. Al-Khatib, MD, MHS, FACC, FAHA Joshua A. Beckman, MD, MS, FAHA Kim K. Birtcher, PharmD, MS,
471 CitationsSource
#1David M. BikoH-Index: 13
#2Aaron G. DeWittH-Index: 4
Last. Yoav DoriH-Index: 27
view all 18 authors...
Patients with single ventricle physiology and greater extension and distribution of lymphatic channels in the chest and neck on T2-weighted MRI had worse outcomes after planned Fontan surgery.
32 CitationsSource
#1Michael Daley (Royal Children's Hospital)H-Index: 4
#2Yves d'Udekem (University of Melbourne)H-Index: 45
5 CitationsSource
#1Michael V. Di Maria (University of Colorado Denver)H-Index: 11
#2David W. Brown (Harvard University)H-Index: 69
Last. Adel K. Younoszai (University of Colorado Denver)H-Index: 19
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More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan c...
13 CitationsSource
#1Thomas G. Wilson (University of Melbourne)H-Index: 3
#2Yves d'Udekem (University of Melbourne)H-Index: 45
Last. Ajay J. Iyengar (University of Melbourne)H-Index: 18
view all 25 authors...
Abstract Background Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. Methods A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99m DTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR). Results Mean age and time since Fontan w...
31 CitationsSource
#1Jessie J. Hu (Mayo Clinic)H-Index: 1
#2M. Yasir Qureshi (Mayo Clinic)H-Index: 5
Last. Frank Cetta (Mayo Clinic)H-Index: 46
view all 11 authors...
Abstract Patients with congenital heart disease with a pressure-overloaded right ventricle can develop liver disease and would benefit from non-invasive diagnostic modalities such as ultrasound shear wave elastography (US SWE). We sought to investigate the ability of US SWE to measure dynamic changes in liver stiffness with an acute fluid bolus in an animal model. Three piglets underwent surgical intervention to create a pressure-overloaded right ventricle and, 12 wk later, underwent US SWE, bot...
4 CitationsSource
#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
#2Joseph T. Poterucha (Mayo Clinic)H-Index: 18
Last. Gruschen R. Veldtman (Cincinnati Children's Hospital Medical Center)H-Index: 33
view all 21 authors...
Data regarding hepatocellular carcinoma (HCC) in patients with Fontan palliation are derived predominantly from case reports and small case series. Consequently little is known about the clinical characteristics and outcomes of HCC in Fontan patients. The purpose of this study was to describe
39 CitationsSource
#1Sudheer R. Gorla (UM: University of Miami)H-Index: 3
#2Nataley K. Jhingoeri (UM: University of Miami)H-Index: 1
Last. Sethuraman Swaminathan (UM: University of Miami)H-Index: 8
view all 8 authors...
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...
6 CitationsSource
#1Phillip M. Trusty (Georgia Institute of Technology)H-Index: 10
#2Zhenglun Wei (Georgia Institute of Technology)H-Index: 5
Last. Ajit P. Yoganathan (Georgia Institute of Technology)H-Index: 82
view all 8 authors...
Abstract Objective The staged Fontan procedure has shown promising short-term outcomes in patients with single ventricles. However, Fontan-associated liver disease is a marked problem as patients age. The purpose of this study is to investigate the relationship between hemodynamics and liver fibrosis in patients undergoing the Fontan. Methods A total of 33 patients undergoing the Fontan with liver fibrosis were included in this study. Cardiac magnetic resonance and phase-contrast cardiac magneti...
23 CitationsSource
#1Jessica L. Bean Jaworski (Boston Children's Hospital)H-Index: 1
#2Matthew T. White (Boston Children's Hospital)H-Index: 16
Last. Adam R. Cassidy (Boston Children's Hospital)H-Index: 9
view all 6 authors...
ABSTRACTAmong the most significant factors affecting quality of life in individuals with critical congenital heart disease (CCHD) are neurodevelopmental challenges, including deficits in visuospatial processing and academic achievement. Few studies have compared outcomes across CCHD subgroups, despite their significant differences in anatomy/physiology and medical/surgical courses. This study compared visuospatial processing abilities using the Developmental Scoring System for the Rey–Osterrieth...
16 CitationsSource
Cited By175
Newest
#1Sven Dittrich (FAU: University of Erlangen-Nuremberg)H-Index: 25
#2Anja Weise (FAU: University of Erlangen-Nuremberg)
Last. Martin Glöckler (FAU: University of Erlangen-Nuremberg)H-Index: 11
view all 8 authors...
Background Increased central venous pressure is inherent in Fontan circulation but not strongly related to Fontan complication. Abnormalities of the lymphatic circulation may play a crucial role in early Fontan complications. Methods This was a retrospective, single-center study of patients undergoing Fontan operation from 2008 to 2015. The primary outcome was significant early Fontan complication defined as secondary in-hospital treatment due to peripheral edema, ascites, pleural effusions, pro...
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#1Wardah Hassan (DUHS: Dow University of Health Sciences)
#2Sohny Kotak (DUHS: Dow University of Health Sciences)H-Index: 1
Last. Taj M. Khan (BCM: Baylor College of Medicine)H-Index: 1
view all 7 authors...
As the population of patients with Fontan palliation grows, so does the number of patients with Fontan failure, necessitating heart transplantation. However, due to mainly small-sized studies, outcomes after heart transplantation in these patients remain unclear. The objective of this study was to review the available literature and conduct a meta-analysis to provide well-powered and generalizable estimates of outcomes after heart transplantation in patients with a failing Fontan. PubMed, Embase...
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#1Maria Victoria Ordonez (UoB: University of Bristol)H-Index: 3
#2Giovanni Biglino (UoB: University of Bristol)H-Index: 17
Last. Radwa Bedair (University Hospitals Bristol NHS Foundation Trust)H-Index: 4
view all 3 authors...
There is no consensus on the clinical utility of ‘routine’ diagnostic cardiac catheterisation in patients with Fontan palliation in the absence of symptoms or haemodynamic lesions. We sought to evaluate whether diagnostic cardiac catheterisation for a variety of indications led to a change in the clinical management of patients with a Fontan circulation. All adult patients (≥16 years) with Fontan palliation undergoing diagnostic cardiac catheterisation at our institution from 2016 to 2019 were i...
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Fontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinates an increased morbidity and mortality. Currently, there is no consensus on how to stage FALD. We report here our experience by an observational study in 52 patients with SV-CHD after Fontan operation...
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#1Kriti Puri (BCM: Baylor College of Medicine)H-Index: 8
#2Martin SchweigerH-Index: 19
Last. Joseph W. Rossano (Children's Hospital of Philadelphia)H-Index: 42
view all 3 authors...
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#1Michael Khoury (U of A: University of Alberta)H-Index: 3
#2Samuel G. Wittekind (Cincinnati Children's Hospital Medical Center)H-Index: 8
Last. Danielle S. Burstein (Children's Hospital of Philadelphia)H-Index: 5
view all 8 authors...
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#1Ondřej Materna (Charles University in Prague)H-Index: 4
#2Vojtěch Illinger (Charles University in Prague)
Last. Jan Janoušek (Charles University in Prague)H-Index: 7
view all 10 authors...
INTRODUCTION Fenestration in the total cavopulmonary connection system may improve the outcome of patients with significant risk factors for Fontan haemodynamics. Our study aims to analyse the difference in long-term survival between non-fenestrated and fenestrated patients. METHODS All consecutive patients (n = 351) who underwent total cavopulmonary connection between 1992 and 2016 were identified. Six early deaths were excluded resulting in a group of 345 patients. Median (interquartile range,...
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#1Fahad Alfares (UM: University of Miami)
#2Satinder K. Sandhu (UM: University of Miami)H-Index: 3
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