Failed fontan

Objectives: Fontan circulation presents significant challenges for patients with congenital heart disease, often necessitating heart transplantation (HTX) due to deteriorating functionality across multiple organ systems. However, the impact of prior Fontan palliation on HTX outcomes remains poorly understood, with early mortality rates suggesting a heightened risk. The aim of our study is to evaluate the long-term results after heart transplantation in patients with univentricular congenital heart disease previously palliated with Fontan circulation...

Lymphatic flow abnormalities are central to the development of protein losing enteropathy, plastic bronchitis, ascites and pleural effusions in patients palliated to the Fontan circulation. These complications can occur in isolation or multicompartmental (two or more). The treatment of multicompartmental lymphatic failure aims at improving thoracic duct drainage. Re-routing the innominate vein to the pulmonary venous atrium decompresses the thoracic duct, as atrial pressure is lower than systemic venous pressure in Fontan circulation...

INTRODUCTION: As the adult Fontan population with Fontan associated liver disease continues to increase, more patients are being referred for transplantation, including combined heart and liver transplantation. METHODS: We report updated mortality and morbidity outcomes after combined heart and liver transplant in a retrospective cohort series of 40 patients (age 14 to 49 years) with Fontan circulation across two centers from 2006-2022. RESULTS: The 30-day, 1-year, 5-year and 10-year survival rate was 90%, 80%, 73% and 73% respectively...

Univentricular heart anomalies represent a group of severe congenital heart defects necessitating early surgical intervention in infancy. The Fontan procedure, the final stage of single-ventricle palliation, establishes a serial connection between systemic and pulmonary circulation by channeling venous return to the lungs. The absence of the subpulmonary ventricle in this peculiar circulation progressively eventuates in failure, primarily due to chronic elevation in inferior vena cava (IVC) pressure. This study experimentally validates the effectiveness of an intracorporeally-powered venous ejector pump (VEP) in reducing IVC pressure in Fontan patients...

Fontan physiology creates a chronic state of decreased cardiac output and systemic venous congestion, leading to liver cirrhosis/malignancy, protein-losing enteropathy, chylothorax, or plastic bronchitis. Creating a fenestration improves cardiac output and relieves some venous congestion. The anatomic connection of the thoracic duct to the subclavian-jugular vein junction exposes the lymphatic system to systemic venous hypertension and could induce plastic bronchitis. To address this complication, two techniques have been developed...

BACKGROUND: In patients with a total cavopulmonary connection in Fontan circulation, the access to the common atrium (CA) during a catheter ablation can be challenging, even in the presence of fenestration in an intra-atrial lateral tunnel (IALT). In our department, the fenestration is typically marked with metal clips (MCs). To the best of our knowledge, there is no previous report of balloonoplasty of clipped fenestration. CASE SUMMARY: A 19-year-old male with hypoplastic left heart syndrome (HLHS) was scheduled for catheter ablation of recurrent atrial tachycardia...

We report a case of an alternative transcatheter use of the modified Medtronic microvascular plug to modify fenestration stent flow in a patient with a rapidly deteriorating clinical condition. This four-year-old boy developed severe cyanosis following fenestration stent insertion, initially placed due to prolonged drainage post-Fontan with extra-cardiac conduit. In April 2023, he underwent urgent cardiac catheterisation and had partial occlusion of fenestration stent with a modified 9Q microvascular plug. His oxygen saturations improved from 50 to 89% in room air with no re-emergence of raised cavopulmonary pressures...

Background A systemic inflammatory response is triggered in patients undergoing cardiothoracic surgery with cardiopulmonary bypass. This response is particularly evident in pediatric patients, especially if of low weight and after undergoing long cardio-pulmonary by-pass (CPB), and can severely impair surgical result. Objectives Adsorptive blood purification techniques have been proposed to limit this systemic inflammatory response. To test its efficacy, we added the hemoadsorption filter Jafron HA 380 to CPB in a very compromised pediatric patient who underwent heart transplantation...

OBJECTIVES: The two opposing inflows and two outflows in a total cavopulmonary connection (TCPC) makes mechanical circulatory support (MCS) extremely challenging. We have previously reported a novel convergent cavopulmonary connection (CCPC) Fontan design that improves baseline characteristics and provides a single inflow and outflow, thus simplifying MCS. This study aims to assess the feasibility of MCS of this novel configuration using axial flow pumps in an in vitro benchtop model...

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...

Purpose To develop an end-to-end deep learning (DL) pipeline for automated ventricular segmentation of cardiac MRI data from a multicenter registry of patients with Fontan circulation (Fontan Outcomes Registry Using CMR Examinations [FORCE]). Materials and Methods This retrospective study used 250 cardiac MRI examinations (November 2007-December 2022) from 13 institutions for training, validation, and testing. The pipeline contained three DL models: a classifier to identify short-axis cine stacks and two U-Net 3+ models for image cropping and segmentation...

Patients with single-ventricle heart disease and failing Fontan circulation represent the largest and most rapidly growing subgroup of adults with congenital heart disease referred for transplant assessment. Few clinical therapies are available for improving Fontan hemodynamics. Mechanical circulatory support devices have been used successfully in the clinical setting to assist the single ventricle, but no device is currently available to support the subpulmonary circulation. A subpulmonary pump could be used to support patients with failing Fontan circulation by mitigating chronic venous hypertension and restoring normal physiology...

Several device designs for cavopulmonary mechanical circulatory support (MCS) are under investigation, however, challenged by the Fontan population's heterogeneity in size, cardiovascular and thoracic anatomy. This study aimed to preclinically assess the anatomical compliance of proposed device designs in silico. Representative double- and single-outlet cavopulmonary assist device (CPAD) designs were virtually implanted into CT imaging data of 10 patients previously palliated with total cavopulmonary connection (TCPC) for functionally univentricular hearts...

INTRODUCTION: Fontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). Currently, there is no consensus on how to stage FALD. Transient elastography (TE) is a rapid, non-invasive method to assess FALD and liver fibrosis. METHOD: To assess the availability and conditions of using TE to monitor liver disease in Fontan patients in german centers for pediatric cardiology and to propose the introduction of a standardized national protocol for the monitoring of liver disease, we developed a questionnaire...

End-stage Fontan patients with single-ventricle (SV) circulation are often bridged-to-heart transplantation via mechanical circulatory support (MCS). Donor shortage and complexity of the SV physiology demand innovative MCS. In this paper, an out-of-the-box circulation concept, in which the left and right ventricles are switched with each other is introduced as a novel bi-ventricle MCS configuration for the "failing" Fontan patients. In the proposed configuration, the systemic circulation is maintained through a conventional mechanical ventricle assist device (VAD) while the venous circulation is delegated to the native SV...

Most studies on single ventricle (SV) circulation take a physiological or anatomical approach. Although there is a tight coupling between cardiac contractility and metabolism, the metabolic perspective on this patient population is very recent. Early findings point to major metabolic disturbances, with both impaired glucose and fatty acid oxidation in the cardiomyocytes. Additionally, Fontan patients have systemic metabolic derangements such as abnormal glucose metabolism and hypocholesterolemia. Our literature review compares the metabolism of patients with a SV circulation after Fontan palliation with that of patients with a healthy biventricular (BV) heart, or different subtypes of a failing BV heart, by Pubmed review of the literature on cardiac metabolism, Fontan failure, heart failure (HF), ketosis, metabolism published in English from 1939 to 2023...

Patients with single ventricle defects undergoing the Fontan procedure eventually face Fontan failure. Long-term cavopulmonary assist devices using rotary pump technologies are currently being developed as a subpulmonary power source to prevent and treat Fontan failure. Low hydraulic resistance is a critical safety requirement in the event of pump failure (0 RPM) as a modest 2 mmHg cavopulmonary pressure drop can compromise patient hemodynamics. The goal of this study is therefore to assess the passive performance of a viscous impeller pump (VIP) we are developing for Fontan patients, and validate flow simulations against in-vitro data...

We describe serial implantation of atrial flow regulator (AFR) devices in the Fontan fenestration of a 4-year-old patient. Initially, the fenestration size was decreased using a 6/5 AFR, resulting in improved saturations and hemodynamics. One year later, further improvement was achieved by placing a 4/10 AFR inside the original device. ( Level of Difficulty: Advanced. ).

Congenital and acquired heart diseases can cause pulmonary hypertension (PH) in children, either by increasing pulmonary blood flow (PBF), left atrial pressure (LAp), and/or pulmonary vascular resistance (PVR). Pathophysiological process of pulmonary vascular disease (PVD) in different types of congenital heart diseases (CHDs) are reviewed hereafter. As with other types of PH, a rigorous diagnostic evaluation is mandatory to characterize the etiology of the PH, rule out other or additional causes of PH, and establish a risk profile...

BACKGROUND: An increasing number of adult Fontan patients require heart transplantation (HT) or combined heart-liver transplant (CHLT); however, data regarding outcomes and optimal referral time remain limited. OBJECTIVES: The purpose of this study was to define survivorship post-HT/CHLT and predictors of post-transplant mortality, including timing of referral, in the adult Fontan population. METHODS: A retrospective cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers in the United States and Canada was performed...