Results of the FUEL Trial.

Published on Feb 25, 2020in Circulation23.603
· DOI :10.1161/CIRCULATIONAHA.119.044352
David J. Goldberg28
Estimated H-index: 28
(Children's Hospital of Philadelphia),
Victor Zak17
Estimated H-index: 17
+ 39 AuthorsStephen M. Paridon42
Estimated H-index: 42
(Children's Hospital of Philadelphia)
Sources
Abstract
Background: The Fontan operation creates a total cavopulmonary connection, a circulation in which the importance of pulmonary vascular resistance is magnified. Over time, this circulation leads to ...
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References45
Newest
#1Hyuk Jae Chang (Yonsei University)H-Index: 59
#2Shinjeong Song (Yonsei University)H-Index: 4
Last. Duk Kyung Kim (SMC: Samsung Medical Center)H-Index: 23
view all 11 authors...
Abstract Purpose Udenafil is an oral phosphodiesterase-5 inhibitor approved for the treatment of erectile dysfunction. In a multicenter, placebo-controlled, randomized Phase IIa study, the reduction of pulmonary vascular resistance index was greater with a 50-mg baseline dose of udenafil than with the 100-mg dose, the cardiac index did not decrease at most points, and the safety was excellent, suggesting that 50-mg udenafil could be used in a Phase IIb trial. Methods In this 16-week, double-blin...
3 CitationsSource
#1Sebastian Udholm (Aarhus University Hospital)H-Index: 4
#2Nael Aldweib (Allegheny General Hospital)H-Index: 4
Last. Gruschen R. Veldtman (Cincinnati Children's Hospital Medical Center)H-Index: 33
view all 4 authors...
Objective Exercise impairment is common in Fontan patients. Our aim is to systematically review previous literature to determine the prognostic value of exercise capacity in older adolescent and adult Fontan patients with respect to late outcome. Additionally, we reviewed the determinants of exercise capacity in Fontan patients and changes in exercise capacity over time. Methods PubMed, CINAHL, Embase, The Cochrane Library and Scopus were searched systematically for studies reporting exercise ca...
19 CitationsSource
#1David J. Goldberg (Children's Hospital of Philadelphia)H-Index: 28
#2victor zakH-Index: 1
Last. Stephen M. Paridon (Children's Hospital of Philadelphia)H-Index: 42
view all 20 authors...
Abstract The Fontan operation creates a circulation characterized by elevated central venous pressure and low cardiac output. Over time, these characteristics result in a predictable and persistent decline in exercise performance that is associated with an increase in morbidity and mortality. A medical therapy that targets the abnormalities of the Fontan circulation might, therefore, be associated with improved outcomes. Udenafil, a phosphodiesterase type 5 inhibitor, has undergone phase I/II te...
13 CitationsSource
Abstract Background Long-term outcomes of Fontan patients who survive to age ≥16 years have not been well characterized. The Australian and New Zealand Fontan Registry (ANZFR) provides a unique opportunity to understand survival and complication rates in Fontan patients who transition to adult congenital heart disease centers. Objectives This study sought to describe the survival and complications of adult patients who have had a Fontan procedure. Methods The study analyzed outcomes in patients ...
62 CitationsSource
#1Brandon S Hays (Cincinnati Children's Hospital Medical Center)H-Index: 2
#2Michael Baker (Cincinnati Children's Hospital Medical Center)H-Index: 2
Last. Gruschen R. Veldtman (Cincinnati Children's Hospital Medical Center)H-Index: 33
view all 9 authors...
Objective Fontan circulations have obligatory venous hypertension, depressed cardiac output and abnormal arterial elastance. Ventriculovascular coupling is known to be abnormal, but the underlying mechanisms are poorly defined. We aim to describe the histopathological features of vascular remodelling encountered in the central arteries and veins in the Fontan circulation as a possible underlying pathological representation of abnormal ventriculovascular coupling. Methods Postmortem vasculature (...
7 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
#1Tacy E. Downing (Children's Hospital of Philadelphia)H-Index: 6
#2Kiona Y. Allen (Children's Hospital of Philadelphia)H-Index: 5
Last. David J. Goldberg (Children's Hospital of Philadelphia)H-Index: 28
view all 14 authors...
Abstract Objective Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort. Methods All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013. Results Follow-up rat...
85 CitationsSource
#1Jonathan W. Cunningham (Brigham and Women's Hospital)H-Index: 9
#2Ashwin S. Nathan (HUP: Hospital of the University of Pennsylvania)H-Index: 11
Last. Alexander R. Opotowsky (Brigham and Women's Hospital)H-Index: 43
view all 6 authors...
Background Peak oxygen consumption (pVO 2 ) measured by cardiopulmonary exercise test (CPET) predicts mortality in adults with a Fontan circulation. The purpose of this study was to assess the additive prognostic value of change in pVO 2 over time. Methods We analyzed a cohort of adults (≥18years-old) with a Fontan circulation who underwent at least 2 maximal CPETs separated by 6–30months at Boston Children's Hospital between 2000 and 2015. Survival analysis was performed to determine whether ch...
30 CitationsSource
#1Frederic Dallaire (Université de Sherbrooke)H-Index: 20
#2Rachel M. Wald (U of T: University of Toronto)H-Index: 24
Last. Ariane Marelli (McGill University)H-Index: 35
view all 3 authors...
Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. As a result of the surgical strategies employed at the time of initial repair, chronic pulmonary regurgitation (PR) is prevalent in this population. Despite sustained research efforts, patient selection and timing of pulmonary valve replacement (PVR) to address PR in young asymptomatic patients with repaired tetralogy of Fallot (rToF) remain a fundamental but as yet unanswered question in the field of congenital he...
20 CitationsSource
#1Andrew M. Atz (MUSC: Medical University of South Carolina)H-Index: 55
#2Victor ZakH-Index: 17
Last. Brian W. McCrindle (U of T: University of Toronto)H-Index: 114
view all 16 authors...
Abstract Background Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. Objectives This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Methods Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after ...
111 CitationsSource
Cited By38
Newest
#1Michael L. O'Byrne (UPenn: University of Pennsylvania)H-Index: 18
#2Jennifer Faerber (Children's Hospital of Philadelphia)H-Index: 15
Last. Andrew C. Glatz (UPenn: University of Pennsylvania)H-Index: 30
view all 11 authors...
Abstract null null BACKGROUND null Survivors of Fontan palliation are at life-long risk of thrombosis, arrhythmia, and circulatory failure. To our knowledge, no studies have evaluated current United States pharmaceutical prescription practice in this population. null null null METHODS null A retrospective observational study evaluating the prevalent use of prescription medications in children and adolescents with hypoplastic left heart syndrome or tricuspid atresia after Fontan completion (ident...
Source
#1Hannes Sallmon (Charité)H-Index: 14
#2Stanislav OvroutskiH-Index: 18
Last. Peter KramerH-Index: 8
view all 8 authors...
Abstract null null Background null The Fontan operation is a palliative procedure and a substantial number of patients eventually experiences late Fontan circulation failure. Previous concepts of Fontan failure implicate increasing pulmonary vascular resistance (PVR) as a key contributor to late circulatory failure. However, data to support this assumption are sparse. We sought to characterize longitudinal hemodynamic and echocardiographic findings in adult failing Fontan patients. null null nul...
Source
#1Ahmed Krimly (King Saud bin Abdulaziz University for Health Sciences)H-Index: 1
#2C. Charles Jain (Mayo Clinic)H-Index: 4
Last. Gruschen R. Veldtman (Jubilee Hospital)H-Index: 33
view all 7 authors...
Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous ...
Source
Source
The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with "stable" Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional...
Source
#1Kevin C. Harris (UBC: University of British Columbia)H-Index: 22
#2Andrew S. Mackie (U of A: University of Alberta)H-Index: 35
Last. Brian W. McCrindle (U of T: University of Toronto)H-Index: 114
view all 8 authors...
Pediatric cardiology has evolved over time with reductions in childhood mortality due to congenital heart disease. Surgical innovation drove early changes in care. Increasingly, the need for more robust evidence provided by randomized controlled trials (RCTs) has been recognized. While the number of RCTs increased, there remains a relative paucity of truly impactful trials in the field. However, those trials that have changed practice have demonstrated the potential and importance of this work. ...
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#1John M. Karamichalis (UTHSC: University of Tennessee Health Science Center)H-Index: 1
Source
#1Asim Al Balushi (U of A: University of Alberta)H-Index: 1
#2Konstantin Averin (U of A: University of Alberta)H-Index: 5
Last. Andrew S. Mackie (U of A: University of Alberta)H-Index: 35
view all 4 authors...
Introduction Preliminary animal and human data suggest that angiotensin-converting enzyme inhibition has a role in pulmonary vascular remodelling. We sought to assess the effect of ACEi versus placebo on pulmonary artery pressure and transpulmonary gradient amongst infants undergoing single-ventricle palliation. Materials and methods Using the publicly available Pediatric Heart Network Infant Single-Ventricle trial dataset, we compared mean PA pressure at pre-superior cavopulmonary connection ca...
Source
#1Mechthild Westhoff-Bleck (Hochschule Hannover)H-Index: 19
Das hohe Herzinsuffizienzrisiko des alternden Erwachsenen mit angeborenem Herzfehler (EMAH) beruht einerseits auf anatomischen, hamodynamischen sowie myokardialen Rest- und Folgezustanden der angeborenen Erkrankung. Andererseits werden mit zunehmendem Alter erworbene Erkrankungen mit ihrem additiven Herzinsuffizienzrisiko bedeutsam. Angesichts des relativ jungen Alters der Patienten rucken praventive Therapiestrategien in den Mittelpunkt des Interesses. Im Gegensatz zur medikamentosen Herzinsuff...
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#1Mathias Possner (University of Cincinnati Academic Health Center)H-Index: 4
#2Anisa Chaudhry (University of Cincinnati Academic Health Center)
Last. Gruschen R. Veldtman (University of Cincinnati Academic Health Center)H-Index: 33
view all 11 authors...
BACKGROUND Systemic venous hypertension and low cardiac output are believed to be important mediators of liver injury after the Fontan procedure. Pulmonary vasodilators have the potential to improve such haemodynamics. The aim of this study was to assess the acute effects of exercise on liver stiffness and venous pressures and to assess the impact of inhaled Treprostinil on this response. METHODS In this prospective, double-blind, placebo-controlled, crossover trial, 14 patients with a Fontan ci...
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