"Fontan procedure"

Growth Characteristics at Time of Fontan Procedure and Their Association with Long-Term Outcomes. To assess whether somatic growth may predict long-term success after a Fontan procedure. The National Death Index was used to track outcomes of Fontan procedures from 1982 through 2011, using data from the Pediatric Cardiac Care Consortium, a US-based registry. Kaplan-Meier plots and Cox models were %, p=0.02). High HAZ and middle WAZ tertiles were associated with lower mortality (aHR vs low tertiles: 0.42; 95%CI: 0.20-0.87, p=0.02 and 0.52; 95%CI: 0.28-0.97, p=0.04). Overweight (WHZ>2) was associated with increased mortality (aHR vs normal WHZ: 2.43; 95%CI: 1.15-5.14, p=0.02). Suboptimal growth is prevalent after Fontan procedure. Higher weight correlates with improved perioperative outcomes

Impact of aorto-pulmonary and veno-venous collaterals on the onset of plastic bronchitis following the Fontan procedure. This study aimed to clarify the association of the aorto-pulmonary/veno-venous collaterals in patients with plastic bronchitis after the Fontan procedure. All patients who underwent total cavopulmonary connection from 1994 to 2022 were reviewed. Aorto-pulmonary/veno-venous

Comparative Analysis of Respiratory and Functional Outcomes in Children Post-Fontan Procedure Versus Healthy Peers. Studies have shown that respiratory muscle training enhances functional capacity and pulmonary function in Fontan patients. However, diaphragm muscle characteristics in Fontan children have not been fully elucidated. The aim of this study was to compare respiratory function

Cardiovascular remodelling in response to exercise training in patients after the Fontan procedure: a pilot study. The cardiovascular adaptations associated with structured exercise training in Fontan patients remain unknown. We hypothesised that short-term training causes cardiac remodelling and parallel improvement in maximal exercise capacity (VO2 max) in these patients. Five patients, median

Liver Cirrhosis After the Fontan Procedure: Impact of Atrioventricular Valve Failure. Liver cirrhosis is now well recognized as a potential complication after the Fontan procedure, although associated risk factors and optimal timing of liver screening remain unclear. All patients who underwent an extracardiac conduit Fontan procedure at The Royal Children's Hospital, Melbourne, were identified using the Australia and New Zealand Fontan Registry. Cirrhosis was diagnosed based on liver biopsy, or a combination of imaging findings and clinical evaluation by a hepatologist. Between 1997 and 2020, 398 patients underwent an extracardiac conduit Fontan procedure at our center, and 276 had ongoing follow-up in Victoria. Ninety-five patients (34%) underwent liver assessment at a mean age of 18.2

Assessment of exercise capacity using field walking tests in patients after the Fontan procedure: A case-control study. Despite the frequent use of the 6-minute walk test (6MWT), exercise capacity has not been assessed with the incremental shuttle walk test (ISWT) in patients who have undergone the Fontan procedure. It is unclear whether these tests cause clinically relevant cardiorespiratory

Persistent Patent Vertical Vein After Repair of Total Anomalous Pulmonary Venous Connection (TAPVR): A Rare Cause of Hypoxemia Post-Fontan Procedure. Vertical vein (VV) ligation during total anomalous pulmonary venous return (TAPVR) repair is controversial. While some surgeons prefer ligation of the VV to prevent adverse sequelae of shunting across it and to promote flow through the newly created anastomosis, others leave it to serve as a "pop off valve" to the left heart structures, which are believed to be hypoplastic and noncompliant, presumably contributing to a more favorable post-operative outcome. We report two patients post-Fontan procedure, who underwent cardiac catheterization to explore the etiology of hypoxia and were found to have a persistent VV responsible for right to left shunting

Comparison of Functional Health Status and Health Related Quality of Life between Adult Patients with Fontan Procedure and Non-Congenital Heart Diseases. To compare functional health status and Health Related Quality of Life (HRQoL) between Fontan and non-congenital heart disease (CHD) patients in an Asian population. Functional health status and HRQoL (New York Heart Association (NYHA

Evaluation of Fontan-associated Liver Disease and Ethnic Disparities in Long-term Survivors of the Fontan Procedure: A Population-based Study. Fontan-associated liver disease (FALD) has emerged as a nearly universal chronic comorbidity in patients with univentricular congenital heart disease who undergo the Fontan procedure. There is a paucity of data reporting long-term outcomes and the impact of FALD in this population. Patients who underwent the Fontan procedure between 1992 and 2018 were identified using California registry data. Presumed FALD was assessed by a composite of liver disease codes. Primary outcomes were mortality and transplant. Multivariable regression and survival analyses were performed. Among 1436 patients post-Fontan, 75.9% studied were adults, with a median follow-up

Dosing Regimen Prediction and Confirmation With Rivaroxaban for Thromboprophylaxis in Children After the Fontan Procedure: Insights From the Phase III UNIVERSE Study. Thrombosis remains an important complication for children with single-ventricle physiology following the Fontan procedure, and effective thromboprophylaxis is an important unmet medical need. To obviate conventional dose-finding

Anticoagulation Therapy After the Fontan Procedure. The optimum postoperative anticoagulation therapy type and duration after the Fontan procedure remains unclear. This study aimed to evaluate whether our approach of administering only oral antiplatelets without warfarin due to serious bleeding complication risks in children is reasonable. We retrospectively reviewed the data of 249 patients who underwent the Fontan procedure. Total cavopulmonary connection with extracardiac conduit was performed in 230 patients (92%), the lateral tunnel Fontan procedure was performed in 18 patients (8%), and intraatrial conduit Fontan procedure was performed in one patient. Aspirin administration (initial dose: 5 mg/kg/day) was continued in all patients. Only 29 patients (11%) received combined administration

Post-operative Morbidity and Mortality After Fontan Procedure in Patients with Heterotaxy and Other Situs Anomalies. Heterotaxy is a complex, multisystem disorder associated with single ventricle heart disease and decreased survival. Ciliary dysfunction is common in heterotaxy and other situs abnormalities (H/SA) and may increase post-operative complications. We hypothesized that patients with H /SA have increased respiratory and renal morbidities and increased in-hospital mortality after Fontan procedure. We queried the Pediatric Health Information System database for hospitalizations with ICD-9/10 codes for Fontan procedure in patients aged 1 through 11 years from 2004 to 2019. H/SA was identified by codes for dextrocardia, situs inversus, asplenia/polysplenia, or atrial isomerism

Hemodynamic Characteristics After Fontan Procedure in Patients with Down's Syndrome. Patients with Down's syndrome (DS) are generally regarded as not being good candidates for the Fontan procedure. However, detailed hemodynamic changes over time are not fully clarified. A retrospective chart review of all patients with DS who underwent the Fontan procedure and 5 times that number of Fontan patients without DS performed in Fukuoka Children's Hospital and Kyushu University Hospital. Seven Fontan patients with DS were identified, and 35 Fontan patients without DS were recruited. During the mean observational periods of 14.7 years and 15.0 years (DS and non-DS, respectively) after the Fontan procedure, only one DS patient died. Central venous pressure (CVP) and transpulmonary pressure gradient

Valve-Sparing Reimplantation for Right Coronary Artery Compression After Fontan Procedure. An incidental finding of right coronary artery compression between the dilated aortic root and the sternum was detected on follow-up cardiac catheterization after Fontan completion for tricuspid atresia with transposition of the great arteries. Although this 31-year-old man had no subjective symptoms

Three-dimensional imaging of pulmonary arterial vasa vasorum using optical coherence tomography in patients after bidirectional Glenn and Fontan procedures. We evaluated pulmonary arterial (PA) vasa vasorum (VV) in Fontan candidate patients with a novel three-dimensional (3D) imaging technique using optical coherence tomography (OCT). This prospective study assessed the development in the BDG than in the control group (3.38% vs. 0.61%; P < 0.001). Although the VV volume ratio decreased significantly after the Fontan procedure (2.64%, P = 0.005 vs. BDG), the ratio remained higher than in the control group (P < 0.001 vs. control). 3D OCT imaging is a novel method that can be used to evaluate adventitial PA VV and may provide pathophysiological insight into the role of the PA VV

Thromboprophylaxis for Children Post-Fontan Procedure: Insights From the UNIVERSE Study. Background Patients with single-ventricle physiology who undergo the Fontan procedure are at risk for thrombotic events associated with significant morbidity and mortality. The UNIVERSE Study evaluated the efficacy and safety of a novel liquid rivaroxaban formulation, using a body weight-adjusted dosing regimen, versus acetylsalicylic acid (ASA) in children post-Fontan. Methods and Results The UNIVERSE Study was a randomized, multicenter, 2-part, open-label study of rivaroxaban, in children who had undergone a Fontan procedure, to evaluate its dosing regimen, safety, and efficacy. Part A was the single-arm part of the study that determined the pharmacokinetics/pharmacodynamics and safety of rivaroxaban

Pre-Fontan Cardiac Catheterization Data as a Predictor of Prolonged Hospital Stay and Post-Discharge Adverse Outcomes Following the Fontan Procedure: A Single-Center Study. Despite improved outcomes following modifications to the Fontan technique, significant morbidity and mortality persist. We sought to determine if abnormal pre-Fontan catheterization hemodynamic data will predict postoperative prolonged hospital stay (PHLOS) and adverse post-discharge outcomes. This is a retrospective study of patients who underwent the Fontan procedure at Children's Hospital of New Orleans from 2008 to 2018. PHLOS was defined as ≥ 14 and ≥ 21 days to discharge post Fontan. We defined post-discharge adverse outcomes as thromboembolic phenomena requiring anticoagulation therapy, protein-losing enteropathy

A Comparison of Bidirectional Glenn vs. Hemi-Fontan Procedure: An Analysis of the Single Ventricle Reconstruction Trial Public Use Dataset. Patients with single ventricle (SV) heart defects have two primary surgical options for superior cavopulmonary connection (SCPC): bidirectional Glenn (BDG) and hemi-Fontan (HF). Outcomes based on type of SCPC have not been assessed in a multi-center cohort

Clinical Significance of Central Venous Pressure During Exercise After Fontan Procedure. Various late complications are associated with the Fontan procedure. In patients who undergo the Fontan procedure, the central venous pressure (CVP) tends to be higher than normal. However, the relationship between CVP (at rest and during exercise) and late complications associated with the Fontan procedure is unknown. Thirty-four patients who underwent the Fontan procedure were enrolled in this study. The median age was 19.3 years, and the median time after the Fontan procedure was 12.7 years. With exercise, the CVP increased from a median of 11 to 19 mmHg, and the cardiac index increased from a median of 2.1 to 4.4 l/min/m. In 38% of the patients, CVP measured at the resting condition and during exercise

Reduced brain mammillary body volumes and memory deficits in adolescents who have undergone the Fontan procedure. Adolescents with single ventricle heart disease (SVHD) who have undergone the Fontan procedure show cognitive/memory deficits. Mammillary bodies are key brain sites that regulate memory; however, their integrity in SVHD is unclear. We evaluated mammillary body (MB) volumes