National trends in Fontan operation and in-hospital outcomes in the USA

Published on May 1, 2019in Heart5.213
· DOI :10.1136/HEARTJNL-2018-313680
Emmanuel Akintoye17
Estimated H-index: 17
(WSU: Wayne State University),
William R. Miranda14
Estimated H-index: 14
(Mayo Clinic)
+ 2 AuthorsAlexander C. Egbe20
Estimated H-index: 20
(Mayo Clinic)
Background National prevalence and outcomes of Fontan operation in the USA is unknown. Study objective was to determine trends (temporal change) in the annual volume of Fontan operations, in-hospital mortality, postoperative complications and type of hospital discharge. Methods Review of the Nationwide Inpatient Sample for patients that underwent Fontan operation from 2001 to 2014 using the International Classification of Diseases-Ninth Revision, Clinical Modification procedure code for Fontan operation, that is, 35.94. To evaluate for change in patients’ demographics over the years, we divided the patient population into four groups based on procedure year (2001–2004, 2005–2008, 2009–2011 and 2012–2014). Results An estimated 15 934 Fontan operations were performed in the USA from 2001 to 2014. Median (Q1–Q3) age was 3 (2–4) years and 39.8% were female. Hypoplastic left heart syndrome was the most common (29%) congenital heart disease diagnosis. An estimated 1175 procedures were performed in 2001 and 1340 in 2014, but there was no significant change in the number of procedures per year (p=0.47). There was significant decline in in-hospital mortality from 4.5% (53/1175) in 2001 to 1.1% (15/1340) in 2014 (p=0.009). When we compared event rates between 2001–2004 and 2012–2014 periods, there was significant decline in postoperative cardiac complications (12.6% (459/3640) to 8% (378/4706), p=0.007) and respiratory complications (17.1% (623/3640) to 10.2% (481/4706), p Conclusion On the average, 1062 Fontan operations are performed annually in the USA with no change in volume of procedures but a decrease in in-hospital mortality and postoperative complications over a 15-year period.
#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
#2William R. Miranda (Mayo Clinic)H-Index: 14
Last. Carole A. Warnes (Mayo Clinic)H-Index: 70
view all 9 authors...
Abstract Background The relationship between temporal progression of magnetic resonance elastography derived liver stiffness (MRE-LS) and progression of Fontan associated liver disease (FALD) is unknown. To assess this relationship, we hypothesized that progression of MRE-LS correlated with progression FALD severity and clinical outcomes. Methods Retrospective review of Fontan patients who had >1 liver MRE, 2010–2016. Annual change in MRE-LS was the quotient of the difference between baseline an...
21 CitationsSource
#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
#2Heidi M. Connolly (Cincinnati Children's Hospital Medical Center)H-Index: 75
Last. Barry A. Borlaug (Cincinnati Children's Hospital Medical Center)H-Index: 76
view all 7 authors...
Background Nonpulsatile pulmonary blood flow in Fontan circulation results in pulmonary vascular disease, but the potential relationships between pulmonary vascular resistance index (PVRI) and Fontan failure have not been studied. The objective was to determine whether the absence of subpulmonary ventricle in the Fontan circulation would make patients more vulnerable to even low-level elevations in PVRI, and when coupled with low cardiac index, this would identify patients at increased risk of F...
45 CitationsSource
#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
#2Arooj R. Khan (Mayo Clinic)H-Index: 9
Last. Heidi M. Connolly (Mayo Clinic)H-Index: 75
view all 9 authors...
Objective Limited data exist regarding the outcomes of non-cardiac procedures (NCPs) in adult patients after Fontan operations (Fontan patients). Methods To compare procedural outcomes after NCPs in Fontan patients with outcomes for two matched control groups: patients with repaired congenital heart disease and biventricular circulation (CHD-BiV) and patients with no heart disease (NHD). We defined cyanosis as oxygen saturation 10% from baseline. Results There were 538 NCPs in 154 Fontan patient...
13 CitationsSource
#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
#2Heidi M. Connolly (Mayo Clinic)H-Index: 75
Last. Christopher J. McLeod (Mayo Clinic)H-Index: 33
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Background The optimal management strategy for atrial tachyarrhythmia in the Fontan population is unknown. Methods Retrospective review of 264 adult Fontan patients with atrial tachyarrhythmia evaluating 3 clinically adopted scenarios: antiarrhythmic drug (AAD) therapy, catheter ablation (CA), and Fontan conversion (FC). These patients were followed up at Mayo Clinic from 1994 to 2014. The study objective was to compare freedom from atrial tachyarrhythmia recurrence (AR) and occurrence of compos...
22 CitationsSource
#1Fred M. Wu (Brigham and Women's Hospital)H-Index: 13
#2Brian Kogon (Emory University)H-Index: 29
Last. Michael J. Landzberg (Brigham and Women's Hospital)H-Index: 73
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Abstract Objectives Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. Methods Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained. Results Of ...
64 CitationsSource
#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
#2Heidi M. Connolly (Mayo Clinic)H-Index: 75
Last. David J. Driscoll (Mayo Clinic)H-Index: 71
view all 9 authors...
Background There are limited studies of thrombotic and embolic complications (TEC) in the adult Fontan population. The purpose of the study was to determine the prevalence, risk factors, and outcomes of TECs in this population. Methods Retrospective review of adults with a previous Fontan operation, with follow-up at Mayo Clinic, 1994-2014. Systemic TEC was defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus. Nonsystemic TEC was defined as Fontan conduit/right atrial ...
32 CitationsSource
#1Masamichi Ono (TUM: Technische Universität München)H-Index: 6
#2Jelena Kasnar-Samprec (TUM: Technische Universität München)H-Index: 12
Last. Rüdiger Lange (TUM: Technische Universität München)H-Index: 56
view all 12 authors...
This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome.Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed.Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92.3%. Freedom from tachyarrhythmia at 15 years was...
45 CitationsSource
#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
#2Heidi M. Connolly (Mayo Clinic)H-Index: 75
Last. David J. Driscoll (Mayo Clinic)H-Index: 71
view all 9 authors...
Abstract Background There are limited data about the risk of thrombotic and embolic complication (TEC) in adults with atrial arrhythmia after Fontan operation. Objectives This study sought to determine the risk of TEC in this population and the role of anticoagulation therapy in TEC prevention. Methods This was a retrospective review of adults with atrial arrhythmia after Fontan operation who were evaluated at the Mayo Clinic between 1994 to 2014. TEC was classified into 2 groups: systemic TEC, ...
48 CitationsSource
#1Marc Gewillig (Katholieke Universiteit Leuven)H-Index: 76
#2Stephen C. Brown (University of the Free State)H-Index: 17
The Fontan operation was first performed in 1968. Since then, this operation has been performed on thousands of patients worldwide. Results vary from very good for many decades to very bad with a pleiad of complications and early death. A good understanding of the physiology is necessary to further improve results. The Fontan connection creates a critical bottleneck with obligatory upstream congestion and downstream decreased flow; these two features are the basic cause of the majority of the ph...
200 CitationsSource
#1Toshihide Nakano (Boston Children's Hospital)H-Index: 14
#2Hideaki Kado (Boston Children's Hospital)H-Index: 27
Last. Shiro Ishikawa (Boston Children's Hospital)H-Index: 11
view all 10 authors...
OBJECTIVES: This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC).
64 CitationsSource
Cited By13
#1Fulvio GasparriniH-Index: 1
#2Ilaria LenciH-Index: 15
Last. Antonio OrlacchioH-Index: 30
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#1John D. Serfas (Duke University)H-Index: 3
#2Dylan Thibault (Durham University)H-Index: 9
Last. Kevin D. Hill (Duke University)H-Index: 32
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Abstract Background Fontan failure often requires surgical therapy in the form of Fontan revision or heart transplantation. We sought to characterize national trends in the surgical burden of Fontan failure and identify risk factors for adverse outcomes. Methods Fontan patients undergoing Fontan revision or transplant from 1/2010-6/2018 were included. We evaluated baseline characteristics and outcomes and used multivariable logistic regression to identify risk factors for operative mortality and...
1 CitationsSource
#1Thomas G. Cotter (U of C: University of Chicago)H-Index: 12
#2Jennifer Wang (U of C: University of Chicago)H-Index: 3
Last. Michael Charlton (U of C: University of Chicago)H-Index: 84
view all 10 authors...
BACKGROUND There are more adults than children living with congenital heart disease (CHD) in the US, with a growing proportion requiring heart-liver transplantation (HLT). Our aim was to ascertain the frequency, outcomes and prognostic factors in this patient population. METHODS UNOS data on adult patients who underwent heart transplantation (HT) from 2009 through March 2020 were analyzed. The primary study outcome was patient survival. Cox proportional-hazards modeling assessed for mortality as...
4 CitationsSource
#1Karl Julius Thrane (Oslo University Hospital)H-Index: 1
#2Lil Sofie Ording Müller (Oslo University Hospital)H-Index: 6
Last. Charlotte de Lange (Oslo University Hospital)H-Index: 7
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PURPOSE Patients with Fontan circulation are at risk of developing hepatic fibrosis/cirrhosis. The mechanisms and disease development are unclear and early secondary liver cancer is a concern. This study will describe hepatic imaging findings in a national cohort of adolescents with Fontan circulation. METHODS The patients prospectively underwent abdominal contrast enhanced magnetic resonance imaging (MRI) including diffusion-weighted imaging. Images were assessed for criteria of fibrosis/cirrho...
1 CitationsSource
Abstract Background The size of the Fontan population with end-stage heart failure is growing. In this population, heart transplant has been the only option. In the present study sought to investigate the efficacy of ventricular assist device (VAD) support in Fontan patients. Methods We conducted a retrospective study of Fontan patients in the ACTION Learning Network. We evaluated patient characteristics, clinical and physiologic outcomes after VAD implant. Results We identified 45 VAD-implanted...
3 CitationsSource
#1Susan R. Hopkins (UCSD: University of California, San Diego)H-Index: 44
#2Rui Carlos Sá (UCSD: University of California, San Diego)H-Index: 12
Last. Rebecca J. Theilmann (UCSD: University of California, San Diego)H-Index: 30
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KEY POINTS The distribution of pulmonary perfusion is affected by gravity, vascular branching structure and active regulatory mechanisms, which may be disrupted by cardiopulmonary disease, but this is not well studied, particularly in rare conditions. We evaluated pulmonary perfusion in patients who had undergone Fontan procedure, patients with pulmonary arterial hypertension (PAH) and two groups of controls using a proton magnetic resonance imaging technique, arterial spin labelling to measure ...
1 CitationsSource
#1Enrique Rodríguez de Santiago (University of Alcalá)H-Index: 8
#2Luis Téllez (University of Alcalá)H-Index: 11
Last. Agustín Albillos (University of Alcalá)H-Index: 55
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AIM Fontan surgery is often the procedure of choice for patients with congenital single effective ventricle. In the long term, elevated systemic venous pressure and chronic ischemia following this procedure could lead to advanced chronic liver disease and there is also a risk of hepatocellular carcinoma (HCC). This review systematically summarizes the characteristics and outcomes of this rare condition. METHODS PubMed and Embase databases were searched from inception to January 2020 for studies ...
4 CitationsSource
The number of Fontan patients with circulatory failure and systolic dysfunction is growing rapidly. The last decade has demonstrated that ventricular assist device (VAD) is an effective therapy in properly selected patients. Herein, we discuss the current approach to patient selection, implantation, and patient management.
#1Irim SalikH-Index: 2
#2Bhupen Mehta (WMC: Westchester Medical Center)
Last. Shashikanth Ambati (Albany Medical College)
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The bidirectional Glenn (BDG) and hemi-Fontan are surgical techniques used to create superior cavopulmonary anastomosis, the second stage repair in Fontan completion. They are performed in patients with an anatomic or functional single ventricle. Whether it is right or left, the single ventricle must therefore provide blood supply to the higher resistance systemic circulation and the lower resistance pulmonary circulation until surgical correction is undertaken. The BDG procedure or hemi-Fontan ...
#1Jonathan R. Dillman (University of Cincinnati Academic Health Center)H-Index: 16
#2Andrew T. Trout (University of Cincinnati Academic Health Center)H-Index: 26
Last. Adam M. Lubert (University of Cincinnati Academic Health Center)H-Index: 5
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The Fontan operation has dramatically altered the natural history of functionally single ventricle congenital heart disease. Patients who have undergone the Fontan operation are living longer and, thus, noncardiac morbidity resulting from the Fontan operation is increasingly being recognized. Fontan-associated liver disease (FALD), one of the chief morbidities following the Fontan operation, is believed to be a multifactorial process that manifests as hepatic congestion and fibrosis, portal hype...
3 CitationsSource