Noninvasive surrogates are poor predictors of liver fibrosis in patients with Fontan circulationPatients with Fontan circulation exhibit a high incidence of liver fibrosis and cirrhosis. Transient elastography (TE) and the enhanced liver fibrosis (ELF) test have proven useful as noninvasive surrogate markers of liver fibrosis for other chronic liver diseases. We evaluated whether TE and the ELF score can predict the degree of liver fibrosis in patients with Fontan circulation.
Preoperative N-terminal pro-brain natriuretic peptide is associated with Fontan outcomesThe role of preoperative N-terminal pro-brain natriuretic peptide level in patient outcomes after the Fontan operation remains unclear.
Diastolic inflow is associated with inefficient ventricular flow dynamics in Fontan patientsThis study used cardiac magnetic resonance imaging to evaluate flow characteristics and ventricular hemodynamics for children with single right (hypoplastic left heart syndrome) and single left (hypoplastic right heart syndrome) systemic ventricle anatomy after Fontan palliation compared with normal biventricular controls.
Biventricular conversion after Fontan completion: A preliminary experienceTo assess the feasibility and outcomes of biventricular conversion following takedown of Fontan circulation.
Factors associated with mortality or transplantation versus Fontan completion after cavopulmonary shunt for patients with tricuspid atresiaTricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS–Fontan interstage death/transplantation versus transition to Fontan in TA.
Imposition of Fontan physiology: Effects on strain and global measures of ventricular functionWe sought to evaluate contractile function in single-ventricle patients before and after imposition of Fontan physiology.
Long-term outcomes of warfarin versus aspirin after Fontan surgeryBecause of the nature of the Fontan physiology, patients are at an increased risk of thromboembolic complications. As such, warfarin or aspirin is generally prescribed lifelong for thromboprophylaxis. This study aimed to compare long-term rates of cerebrovascular injury, thrombosis, bleeding, bone mineral density, and quality of life in people living with Fontan circulation receiving warfarin compared with aspirin.
Progression in Fontan conduit stenosis and hemodynamic impact during childhood and adolescenceTo characterize changes in Fontan conduit size over time and determine if cross-sectional area (CSA) affects cardiac output, pulmonary artery growth, and exercise capacity.
Long-term outcomes following Fontan takedown in Australia and New ZealandFontan takedown remains an option for the management of Fontan failure. We sought to evaluate early and late outcomes after Fontan takedown.
Protein-losing enteropathy and plastic bronchitis after the Fontan procedureProtein losing enteropathy and plastic bronchitis are severe complications in Fontan circulation, with 5-year survival ranging from 46% to 88%. We report risk factors and outcomes of protein losing enteropathy and plastic bronchitis in patients undergoing the Fontan.
A preoperative estimate of central venous pressure is associated with early Fontan failureEarly Fontan failure is a serious complication after total cavopulmonary connection, characterized by high central venous pressure, low cardiac output, and resistance to medical therapy. This study aimed to estimate postoperative central venous pressure in patients with total cavopulmonary connection using data routinely collected during preoperative assessment. We sought to determine if this metric correlated with measured postoperative central venous pressure and if it was associated with early Fontan failure.
Role of surgeon intuition and computer-aided design in Fontan optimization: A computational fluid dynamics simulation studyCustomized Fontan designs, generated by computer-aided design (CAD) and optimized by computational fluid dynamics simulations, can lead to novel, patient-specific Fontan conduits unconstrained by off-the-shelf grafts. The relative contributions of both surgical expertise and CAD to Fontan optimization have not been addressed. In this study, we assessed hemodynamic performance of Fontans designed by both surgeon's unconstrained modeling (SUM) and by CAD.
Fontan with lateral tunnel is associated with improved survival compared with extracardiac conduitThe study aim was to compare Fontan patients undergoing lateral tunnel (LT) versus extracardiac conduit (ECC) technique.
In vivo implantation of 3-dimensional printed customized branched tissue engineered vascular graft in a porcine modelThe customized vascular graft offers the potential to simplify the surgical procedure, optimize physiological function, and reduce morbidity and mortality. This experiment evaluated the feasibility of a flow dynamic–optimized branched tissue engineered vascular graft (TEVG) customized based on medical imaging and manufactured by 3-dimensional (3D) printing for a porcine model.
Cavopulmonary assist: Long-term reversal of the Fontan paradoxFontan circulatory inefficiency can be addressed by replacing the missing subpulmonary power source to reverse the Fontan paradox. An implantable cavopulmonary assist device is described that will simultaneously reduce systemic venous pressure and increase pulmonary arterial pressure, improving preload and cardiac output, in a univentricular Fontan circulation on a long-term basis.
Y-graft modification to the Fontan procedure: Increasingly balanced flow over timeThe use of Y-grafts for Fontan completion is hypothesized to offer more balanced hepatic flow distribution (HFD) and decreased energy losses. The purpose of this study was to evaluate the hemodynamic performance of Y-grafts over time using serial cardiac magnetic resonance data and to compare their performance with extracardiac Fontan connections.
An in vitro analysis of the PediMag and CentriMag for right-sided failing Fontan supportRight-sided mechanical circulatory support for failing Fontan physiology has been largely unsuccessful due to inherent hemodynamic differences between these patients and the target populations for most assist devices. This study uses advanced benchtop modeling of Fontan physiology to examine the use of PediMag and CentriMag to improve failing Fontan hemodynamics.
Evaluation of pulmonary endothelial function in Fontan patientsPulmonary endothelial dysfunction due to a loss of pulsatile pulmonary blood flow is thought to be a major contributor in the development of increased pulmonary vascular resistance (PVR) in patients with a Fontan circulation. We sought to evaluate pulmonary endothelial dysfunction by vasodilator response to acetylcholine in children and adolescents with Fontan hemodynamics.
The first cohort of prospective Fontan surgical planning patients with follow-up data: How accurate is surgical planning?Fontan surgical planning is an image-based, collaborative effort, which is hypothesized to result in improved patient outcomes. A common motivation for Fontan surgical planning is the progression (or concern for progression) of pulmonary arteriovenous malformations. The purpose of this study was to evaluate the accuracy of surgical planning predictions, specifically hepatic flow distribution (HFD), a known factor in pulmonary arteriovenous malformation progression, and identify methodological improvements needed to increase prediction accuracy.
Influence of aortic stiffness on ventricular function in patients with Fontan circulationElastic properties of the thoracic aorta are responsible for buffering systemic afterload, and may be particularly important in patients with Fontan circulation, in whom heart failure is a major source of attrition. The purpose of this study was to characterize regional stiffness in the ascending and descending aorta in patients with hypoplastic left heart syndrome and single left ventricle morphology after Fontan operation by cardiac magnetic resonance imaging, and to assess whether changes in aortic stiffness are associated with the ventricular function.
Long-term outcome of preadolescents, adolescents, and adult patients undergoing total cavopulmonary connectionPatients with a single ventricle infrequently undergo total cavopulmonary connection as preadolescents, adolescents, or adults. The purpose of this study was to clarify the characteristics of this cohort and to analyze the factors influencing outcomes.
High incidence of late infective endocarditis in bovine jugular vein valved conduitsBovine jugular vein (BJV) grafts (Medtronic, Inc, Minneapolis, Minn) are used to restore right ventricle-to-pulmonary artery continuity. Recent studies have associated these grafts with the development of infective endocarditis. The purpose of this study was to report the incidence of endocarditis in BJV grafts.
Impact of hemodynamics and fluid energetics on liver fibrosis after Fontan operationThe 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.