fontan operation

Background: Advancements in palliative surgery of patients with single ventricle physiology have led to an increase in the need for deep sedation protocols for painful procedures. However, positive pressure ventilation during anesthesia can result in unfavorable cardiopulmonary interactions. This patient population may benefit from sedation from these painful procedures. Methods: This study aims to demonstrate the safety and efficacy of deep sedation by pediatric intensivists outside the operating room for children with single ventricle physiology...

OBJECTIVE: To identify risk factors for aortopulmonary collateral (APC) development and assess the impact of severe APCs in children undergoing staged single ventricle palliation. METHODS: Children undergoing a bidirectional Glenn operation between January 1, 2016, and March 31, 2021, at our center were included. All underwent angiography prior to Glenn and Fontan; APC flow was graded on a scale of 0 (no appreciable collateral flow) to 4 (severe burden). Demographic data, congenital diagnosis, clinical history, and outcomes were stratified by Glenn assessment; Fontan outcomes were stratified by pre-Fontan grade...

BACKGROUND: Superior cavopulmonary connection (SCPC) for stage II palliation of hypoplastic left heart syndrome (HLHS) is achieved most frequently by either a bidirectional Glenn (BDG) or hemi-Fontan (HF) operation. The comparison of flow hemodynamic efficiency at the region of surgical reconstruction and in proximal pulmonary arteries has been evaluated primarily using computational modeling techniques with conflicting reports. The purpose of this descriptive study was to compare flow hemodynamics following stage II (BDG vs HF) using 4-dimensional flow magnetic resonance imaging (4D-Flow MRI) with particular focus on flow-mediated viscous energy loss ( E L ' ) under matched hemodynamic conditions...

OBJECTIVE: To investigate the early and long-term outcomes of the deferred Norwood procedure by bilateral pulmonary artery banding (BPAB) versus the neonatal Norwood procedure. METHODS: This retrospective study examined 46 patients with hypoplastic left heart syndrome and its variants undergoing the Norwood procedure for single ventricle physiology between 2004 and 2022 at 3 institutions. The patients were divided into 2 groups: neonatal Norwood procedure (group N; n = 23) and staged Norwood procedure in infants following BPAB (group I; n = 23)...

The Fontan operation, the final palliative step after a series of complex operations in patients with univentricular hearts, has undergone multiple modifications throughout the last decades, with the goal of finding the method which combines the optimal hemodynamic effects of the Fontan circulation with minimal long term side effects. An understanding of the operative evolution and subsequent side effects, as well as the management thereof seems imperative. Since its inception by Francis Fontan the, now obsolete, initial atriopulmonary connection has passed through several milestones into having now reached the era of total cavopulmonary connection...

OBJECTIVES: Combined heart-liver transplantation (CHLT) is becoming increasingly frequent as a maturing population of patients with Fontan-palliated congenital heart disease develop advanced liver fibrosis or cirrhosis. The authors present their experience with CHLT for congenital and noncongenital indications, and identify characteristics associated with poor outcomes that may guide intervention in high-risk patients. DESIGN: This was a single-center retrospective cohort study...

BACKGROUND: Chronic liver disease leads to liver fibrosis, and an accurate diagnosis of the fibrosis stage is crucial for medical management. Connective tissue growth factor (CTGF) is produced by endothelial cells and platelets and plays a central role in inducing fibrosis in various organs. In the present study, we tested the validity of measuring the serum levels of two types of CTGF to estimate the biopsy-confirmed liver fibrosis stage. METHODS: We used two detection antibodies targeting the N- and C-terminal of CTGF to measure the serum levels of two forms of CTGF consisting of its full length and its N-terminal fragment...

OBJECTIVES: The goal of this retrospective multicentre study was to present late surgical outcomes of the treatment of children with double outlet right ventricle (DORV) coming from emerging countries. METHODS: The Mécénat Chirurgie Cardiaque brings to France for surgery selected children with simple and complex congenital diseases, including DORV. The patients are operated on in 9 hospitals that specialize in paediatric cardiac surgery. Data are collected from the Mécénat Chirurgie Cardiaque comprehensive database, with a strict postoperative follow-up...

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...

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Sinus node dysfunction (SND) with junctional rhythm (JR) is common after the Fontan operation. Atrial pacing (AP) restores atrioventricular (AV) synchrony, but the placement of a pacemaker carries significant morbidity. To study the impact of AP on echocardiographic parameters of function in Fontan patients with SND and JR. Nine Fontan patients with AP for SND and JR were prospectively studied with echocardiography in the following conditions-baseline paced rhythm, underlying JR and, if possible, slow-paced rhythm below their baseline paced rate (~ 10 bpm faster than their JR rate)...

A 3-year-old boy with Glenn physiology exhibited refractory heart failure with reduced ejection fraction. To improve the patient's oxygen saturation, he underwent ventricular assist device (VAD) implantation with concomitant Fontan completion. The extracardiac conduit Fontan operation was performed with a 4-mm fenestration. For VAD implantation, Berlin Heart cannulas were positioned at the left ventricular apex and the neo-aorta. Following weaning from cardiopulmonary bypass, a temporary continuous-flow VAD, equipped with an oxygenator, was utilized for support...

BACKGROUND: Haemodynamic changes in caval venous flow distribution occurring during bidirectional cavopulmonary anastomosis operation are still largely unknown. METHODS: Transit time flow measurements were performed in 15 cavopulmonary anastomosis operations. Superior and inferior caval vein flows were measured before and after the cavopulmonary anastomosis. Ratio of superior caval vein to overall caval veins flow was calculated. RESULTS: Mean superior caval vein flow ratio before cavopulmonary anastomosis was higher than previously reported for healthy children...

OBJECTIVE: Intra-atrial reentrant tachycardia (IART) is an important late-onset complication in Fontan patients. However, the protective effects of prophylactic cryoablation against late-onset IART are unclear. This study investigated the late development of IART in lateral tunnel (LT) Fontan patients and the role of prophylactic cryoablation. METHODS: This was a single-center retrospective cohort study of patients who underwent the LT Fontan procedure between 1988 and 2003...

BACKGROUND: Ventricular dyssynchrony and its relationship to clinical outcomes is not well characterized in patients following Fontan palliation. METHODS: Single-center retrospective analysis of cardiac magnetic resonance (CMR) imaging of patients with a Fontan circulation and an age-matched healthy comparison cohort as controls. Feature tracking was performed on all slices of a ventricular short-axis cine stack. Circumferential and radial strain, strain rate, and displacement were measured; and multiple dyssynchrony metrics were calculated based on timing of these measurements (including standard deviation of time-to-peak, maximum opposing wall delay, and maximum base-to-apex delay)...

BACKGROUND: The main objective measure to assess the health of the Fontan circulation is the pressure measurement of the superior vena cava or pulmonary arteries. We reviewed the literature for benefits of measuring resting pressure in the Fontan circuit and explored whether dynamic measurement by volume loading or exercise has the potential to refine this diagnostic tool. METHODS: PubMed was searched for articles showing a relationship between resting post-operative central venous pressure or pulmonary artery pressure and Fontan failure...

BACKGROUND: The bidirectional Glenn (BDG) shunt operation serves as temporary surgery for the treatment of single-ventricle physiology with the eventual Fontan procedure. In some cases, the procedure can be performed without the support of a cardiopulmonary bypass (CPB) machine. In this study, we present the surgical outcomes of off-pump BDG operation with the use of a temporary veno-atrial shunt to decompress the superior vena cava (SVC) during clamping time. METHODS: A cohort of 23 patients underwent off-pump BDG operations at Cardiovascular Center, E Hospital...

Distal radius fractures are common injuries. Satisfactory outcomes are typically achieved with appropriate nonoperative or operative treatment. A proportion of these injuries develop symptomatic malunions, which may be treated surgically with distal radius corrective osteotomy. A thorough understanding of the anatomy, biomechanics, radiographic parameters, and indications is needed to provide appropriate treatment. Factors, including surgical approach, osteotomy type, use of bone graft, fixation construct, management of associated tendon and/or nerve conditions, soft tissue contracture releases, and need for ulnar-sided procedures, should be considered...

BACKGROUND: Fontan-associated liver disease (FALD) is characterized by hepatic congestion and progressive hepatic fibrosis in patients with the Fontan operation. This condition is generally clinically silent until late, necessitating techniques for early detection. Liver T1 mapping has been used to screen for FALD, but without consideration of regional variations in T1 values. METHODS: Liver T1 measured with a liver-specific T1 mapping sequence (PROFIT1) in Fontan patients was compared with cohorts of patients with biventricular congenital heart disease (BiV-CHD) and controls with normal cardiac function and anatomy...

The gold standard for determining the severity of liver disease in Fontan patients is now liver biopsy. Since it is an invasive procedure, this study determined the possibility of applying mitochondrial function from isolated peripheral blood mononuclear cells (PBMCs) as a non-invasive indicator of liver fibrosis. Fontan patients (n = 37) without known liver disease were analysed cross-sectionally. Patients were classified according to their histology using the METAVIR score as follows; F0/F1-no/mild fibrosis; F2-moderate fibrosis; and F3/F4-cirrhosis...