Congenital: Fontan
Noninvasive surrogates are poor predictors of liver fibrosis in patients with Fontan circulation
Patients 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.Biventricular conversion after Fontan completion: A preliminary experience
To 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 atresia
Tricuspid 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 function
We 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 surgery
Because 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 adolescence
To characterize changes in Fontan conduit size over time and determine if cross-sectional area (CSA) affects cardiac output, pulmonary artery growth, and exercise capacity.Protein-losing enteropathy and plastic bronchitis after the Fontan procedure
Protein 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 failure
Early 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.Cavopulmonary assist: Long-term reversal of the Fontan paradox
Fontan 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 time
The 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 support
Right-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.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.Impact of hemodynamics and fluid energetics on liver fibrosis after Fontan operation
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.
