Failed fontan

We are developing a clinically practical percutaneous double lumen cannula (DLC)-based cavopulmonary assist (CPA) system to support failing Fontan patients. In this study, our CPA DLC was redesigned for even blood flow, minimal recirculation, and easy insertion/deployment. After bench testing, this new CPA system was evaluated for 4 hours (n = 10) and 96 hours (n = 5) in our clinically relevant lethal cavopulmonary failure (CPF) sheep model for ease of cannulation/deployment, reversal of CPF hemodynamics/end-organ hypoperfusion, and durability/biocompatibility...

A successful hepatic-to-azygos vein redirection was performed in a patient with absent inferior vena cava using a long vascular graft to address a pulmonary arterio-venous fistula after a failed Fontan conversion. No exacerbation was observed 5 years postoperatively.

Children with hypoplastic left heart syndrome share unique hemodynamic features that alter lymphatic integrity at all stages of palliation. Lymphatic congestion is almost universal in this patient group to some extent. It may lead to reversal of lymphatic flow, the development of abnormal lymphatic channels and ultimately decompression and loss of protein rich lymphatic fluid into extra lymphatic compartments in prone individuals. Some of the most devastating complications that are associated with single ventricle physiology, notably plastic bronchitis and protein losing enteropathy, have now been proven to be lymphatic in origin...

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 for a viscous impeller pump (VIP) we are developing for Fontan patients, and validate flow simulations against in-vitro data...

PURPOSE: The Fontan circulation carries a dismal prognosis in the long term due to its peculiar physiology and lack of a subpulmonic ventricle. Although it is multifactorial, elevated IVC pressure is accepted to be the primary cause of Fontan's high mortality and morbidity. This study presents a self-powered venous ejector pump (VEP) that can be used to lower the high IVC venous pressure in single-ventricle patients. METHODS: A self-powered venous assist device that exploits the high-energy aortic flow to lower IVC pressure is designed...

A child with early failure of a Fontan circulation was listed for cardiac transplantation and then developed a subhepatic abscess. Surgical drainage was deemed necessary after the failure of an attempted percutaneous procedure. Following a multidisciplinary discussion, a laparoscopic technique was chosen to optimise postoperative recovery. To our knowledge, the literature does not describe any case of laparoscopic surgery in a patient with a failing Fontan circulation. This case report highlights the physiological variations involved with this management strategy, discusses the implications and risks, and offers some recommendations...

Some patients with functionally univentricular circulation develop cardiac failure refractory to maximal management and are supported with a ventricular assist device (VAD). The purpose of this manuscript is to summarize our previous publications related to single ventricle-ventricular assist device (sVAD) support in patients with functionally univentricular circulation and to describe our current institutional approach at University of Florida to sVAD support in neonates, infants, and children prior to Fontan...

OBJECTIVE: This study aimed to prove if pulmonary artery size influences survival and if an additional aortopulmonary shunt promotes left pulmonary artery growth after bidirectional cavopulmonary shunt in patients with hypoplastic left heart syndrome. METHODS: The medical records of patients with hypoplastic left heart syndrome who underwent Norwood procedure and bidirectional cavopulmonary shunt between 2007 and 2020 were reviewed. Right, left, and total (right + left) pulmonary artery indices were calculated according to Nakata and colleagues...

Women with single ventricle physiology after the Fontan procedure, despite numerous possible complications, can reach adulthood and give birth. Pregnancy poses a hemodynamic burden for distorted physiology of Fontan circulation, but according to the literature, it is usually well tolerated unless the patient is a "failing" Fontan. Our study aimed to assess maternal and fetal outcomes in patients after the Fontan procedure followed up in two tertiary Polish medical centers. We retrospectively evaluated all pregnancies in women after the Fontan procedure who were followed up between 1995-2022...

INTRODUCTION: The Atrial Flow Regulator (AFR) is a new self-expandable percutaneous-delivered fenestrated device providing an interatrial shunt. Its use in pediatric population has been reported in failing Fontan, pulmonary hypertension, or in patients with cardiomyopathy and venoarterial extracorporeal membrane oxygenator (ECMO) support. Its use in small children under 10 kg has not been reported. METHODS: We report the initial single center experience of the AFR implantation in children below 10 kg...

BACKGROUND: Ventricular assist device (VAD) support for failing Glenn circulation represents a unique challenge. METHODS: We conducted a retrospective review of clinical outcomes in patients with VAD support for failing Glenn circulation between 2010 and 2020 at a tertiary pediatric institution. RESULTS: Ten patients were included: INTERMACS profiles were 1 in 3 patients and 2 in 7 patients. The median age, weight, and body surface area were 3...

OBJECTIVES: Fontan-associated liver disease (FALD) is universal post-Fontan palliation; however, its impact on survival remains controversial and current diagnostic tools have limitations. We aimed to assess the prognostic role of liver fibrosis scores (aminotransferase to platelet ratio [APRI] and fibrosis-4 [FIB-4]) and their association with haemodynamics and other markers of liver disease. METHODS: 159 adults (age ≥18 years) post-Fontan undergoing catheterisation at Mayo Clinic, Minnesota, between 1999 and 2017 were included...

Background Peak oxygen consumption (peak <mml:math xmlns:mml="https://www.w3.org/1998/Math/MathML"><mml:semantics><mml:mrow><mml:mover><mml:mi>V</mml:mi><mml:mo>̇</mml:mo></mml:mover><mml:msub><mml:mi>O</mml:mi><mml:mn>2</mml:mn></mml:msub></mml:mrow><mml:annotation>$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$</mml:annotation></mml:semantics></mml:math>) is traditionally divided ("ratio-scaled") by body mass (BM) for clinical interpretation...

PURPOSE OF REVIEW: Combined heart and liver transplantation (CHLT) is an uncommon but increasingly performed procedure with rising need as the population who has undergone Fontan palliation for single ventricle physiology grows. This article reviews the current literature to summarize what is known about patient selection and outcomes and highlights the questions that remain. RECENT FINDINGS: Congenital heart disease (CHD) with Fontan-associated liver disease (FALD) has surpassed noncongenital heart disease as the most common indication for CHLT...

BACKGROUND: The number of single ventricle patients undergoing Fontan palliation and surviving to adulthood worldwide has steadily increased in recent years. Nevertheless, the Fontan circulation is destined to fail. Ultimately, heart transplantation (HTx) remains the definitive treatment option. Due a shortage of organs, mechanical circulatory support in the form of ventricular assist devices (VADs) is widely used to bridge heart failure patients to HTx, but these devices have been mainly developed to address the needs of normal anatomies...

No abstract text is available yet for this article.

BACKGROUND: Although all congenital heart defects (CHD) present unique challenges, univentricular CHD are especially challenging given the difficulty of passively perfusing pulmonary blood flow. Three surgical procedures are required within the first years of life, with the final completing a Fontan circulation in which the inferior vena cava is connected to the pulmonary artery and previously connected superior vena cava. This allows passive venous return to the pulmonary circulation then flow into the single ventricle for systemic circulation...

Lymphatic congestion is known to play an important role in the development of late Fontan complications. This study aimed to (1) develop a gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA) contrast three-dimensional heavily T2-weighed MR technique that can detect abnormal lymphatic pathway in the abdomen while simultaneously evaluating hepatocellular carcinoma (HCC) and to (2) propose a new classification of abnormal abdominal lymphatic pathway using a non-invasive method in adults with Fontan circulation...

In [1], there are errors in Table I and equation (3) which are corrected here. This does not affect the study results. We sincerely apologize for the errors and any confusion they may have caused. \begin{equation*} \Delta {\mathrm {P}} = {\mathrm {A}}{\mathrm {Q}}^3 + \mathrm {B}{\mathrm {Q}}^2 + \text{CQ} + \mathrm {D} \tag{3} \end{equation*}.

Fontan circulation (FC) is a surgically achieved palliation state offered to patients affected by a wide variety of congenital heart defects (CHDs) that are grouped under the name of univentricular heart. The procedure includes three different surgical stages. Malnutrition is a matter of concern in any phase of life for these children, often leading to longer hospital stays, higher mortality rates, and a higher risk of adverse neurodevelopmental and growth outcomes. Notwithstanding the relevance of proper nutrition for this subset of patients, specific guidelines on the matter are lacking...