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Journal of Cardiology Sep 2019The Fontan procedure has led to increased long-term survival of patients with single ventricle congenital heart disease. Hemodynamic changes associated with the Fontan... (Review)
Review
The Fontan procedure has led to increased long-term survival of patients with single ventricle congenital heart disease. Hemodynamic changes associated with the Fontan circulation, including elevated central venous pressure and diminished cardiac output are responsible for the development of Fontan-associated liver disease (FALD). Liver fibrosis is a universal feature following the Fontan operation. The incidence of both liver cirrhosis and hepatocellular carcinoma (HCC) increases with the duration of the Fontan circulation. The staging of liver fibrosis in FALD requires a multi-modality approach involving clinical assessment, biochemical/hematological parameters, non-invasive fibrosis scores, radiological imaging, elastography, and liver histology. Patients with a failing Fontan circulation who have evidence of significant hepatic congestion require careful hemodynamic assessment to optimize the Fontan pathway and physiology. This may necessitate percutaneous or surgical intervention, or heart transplantation. Combined heart-liver transplantation may be required in patients with clinical, imaging, or biopsy evidence of advanced liver cirrhosis, particularly if there is evidence of hepatic decompensation or localized HCC. Patients with suspected liver cirrhosis should be enrolled into HCC surveillance and require endoscopic variceal assessment. There is a clear need to establish local/national registries for Fontan patients with standardized guidelines for the management of FALD, bringing together the expertise of professional bodies representing both cardiologists and hepatologists.
Topics: Carcinoma, Hepatocellular; Cardiac Output, Low; Central Venous Pressure; Female; Fontan Procedure; Heart Transplantation; Humans; Incidence; Liver Cirrhosis; Liver Function Tests; Liver Neoplasms; Liver Transplantation; Male; Postoperative Complications; Registries; Time Factors; Univentricular Heart
PubMed: 30928109
DOI: 10.1016/j.jjcc.2019.02.016 -
Indian Heart Journal 2018This article reviews important features to improve the diagnosis of congenital heart disease (CHD) by applying ultrasound in prenatal cardiac screening. As low and... (Review)
Review
This article reviews important features to improve the diagnosis of congenital heart disease (CHD) by applying ultrasound in prenatal cardiac screening. As low and high-risk pregnancies for CHD are subject to routine obstetric ultrasound, the diagnosis of structural heart defects represents a challenge that involves a team of specialists and subspecialists on fetal ultrasonography. In this review, the images highlight normal anatomy of the heart as well as pathologic cases consistent with cardiac malposition and isomerism, septal defects, pulmonary stenosis/atresia, aortic malformations, hypoplastic left ventricle, conotruncal anomalies, tricuspid dysplasia, and Ebstein's anomaly, and univentricular heart, among other congenital cardiovascular defects. Anatomical details of most CHD in fetuses were provided by two-dimensional (2D) ultrasound with higher quality imaging, enhancing diagnostic accuracy in a variety of CHD. Moreover, the accuracy of the cardiac defects in obstetrics ultrasound improves the outcome of most CHD, providing planned delivery, aided genetic counseling, and perinatal management.
Topics: Female; Health Knowledge, Attitudes, Practice; Heart Defects, Congenital; Humans; Pregnancy; Prenatal Diagnosis
PubMed: 29455772
DOI: 10.1016/j.ihj.2017.12.005 -
Methodist DeBakey Cardiovascular Journal 2019Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive...
Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. This operation converts the atriopulmonary Fontan to an extracardiac Fontan that improves hemodynamics and controls the atrial arrhythmias. Operative mortality during that time was 2%, and freedom from death or heart transplant at 10 years is 84%. For properly selected patients, Fontan conversion improves both survival and quality of life. Patients with an atriopulmonary Fontan constitute an eroding population, as they face many comorbidities and have a decreased life expectancy without treatment; therefore, all patients with an atriopulmonary Fontan should be evaluated for this procedure.
Topics: Adolescent; Adult; Arrhythmias, Cardiac; Cardiomegaly; Child; Child, Preschool; Clinical Decision-Making; Female; Fontan Procedure; Heart Defects, Congenital; Humans; Male; Middle Aged; Patient Selection; Progression-Free Survival; Reoperation; Risk Assessment; Risk Factors; Time Factors; Young Adult
PubMed: 31384373
DOI: 10.14797/mdcj-15-2-105 -
Frontiers in Pediatrics 2019The conventional Fontan circulation deviates the superior vena cava (SVC = 1/3 of the systemic venous return) toward the right lung (3/5 of total lung volume) and the...
The conventional Fontan circulation deviates the superior vena cava (SVC = 1/3 of the systemic venous return) toward the right lung (3/5 of total lung volume) and the inferior vena cava (IVC = 2/3 of the systemic venous return) toward the left lung (2/5 of total lung volume). A "physiological" Fontan deviating the SVC toward the left lung and the IVC toward the right lung was compared with the conventional setting by computational fluid dynamics, studying whether this setting achieves a more favorable hemodynamics than the conventional Fontan circulation. An 3D parametric model of the Fontan procedure was developed using idealized vascular geometries with invariant sizes of SVC, IVC, right pulmonary artery (RPA), and left pulmonary artery (LPA), steady inflow velocities at IVC and SVC, and constant equal outflow pressures at RPA and LPA. These parameters were set to perform finite-volume incompressible steady flow simulations, assuming a single-phase, Newtonian, isothermal, laminar blood flow. Numerically converged finite-volume mass and momentum flow balances determined the inlet pressures and the outflow rates. Numerical closed-path integration of energy fluxes across domain boundaries determined the flow energy loss rate through the Fontan circulation. The comparison evaluated: (1) mean IVC pressure; (2) energy loss rate; (3) kinetic energy maximum value throughout the domain volume. The comparison of the physiological vs. conventional Fontan provided these results: (1) mean IVC pressure 13.9 vs. 14.1 mmHg (= 0.2 mmHg reduction); (2) energy loss rate 5.55 vs. 6.61 mW (= 16% reduction); (3) maximum kinetic energy 283 vs. 396 J/m (= 29% reduction). A more physiological flow distribution is accompanied by a reduction of mean IVC pressure and by substantial reductions of energy loss rate and of peak kinetic energy. The potential clinical impact of these hemodynamic changes in reducing the incidence and severity of the adverse long-term effects of the Fontan circulation, in particular liver failure and protein-losing enteropathy, still remains to be assessed and will be the subject of future work.
PubMed: 31179252
DOI: 10.3389/fped.2019.00196 -
Translational Pediatrics Feb 2023Key medical and surgical advances have been made in the longitudinal management of patients with "functionally" single ventricle physiology, with the principles of... (Review)
Review
BACKGROUND AND OBJECTIVE
Key medical and surgical advances have been made in the longitudinal management of patients with "functionally" single ventricle physiology, with the principles of Fontan circulation applied to other complex congenital heart defects. The purpose of this article is to review all of the innovations, starting from fetal life, that led to a change of strategy for single ventricle.
METHODS
Our literature review included all full articles published in English language on the Cochrane, MedLine, and Embase with references to "single ventricle" and "univentricular hearts", including the initial history of the treatments for this congenital heart defects as well as the innovations reported within the last decades.
KEY CONTENT AND FINDINGS
All innovations introduced have been analyzed, including: (I) fetal diagnosis and interventions, in particular to prevent or reduce brain damages; (II) neonatal care; (III) post-natal diagnosis; (IV) interventional cardiology procedures; (V) surgical procedures, including neonatal palliations, hybrid procedures, bidirectional Glenn and variations, Fontan completion, biventricular repair; (VI) peri-operative management; (VII) Fontan failure, with Fontan take-down and conversion, and mechanical circulatory support; (VIII) transplantation, including heart, heart and lung, heart and liver; (IX) exercise; (X) pregnancy; (XI) adolescents and adults without Fontan completion; (XII) future studies, including experimental studies on animals, computational studies, genetics, stem cells and bioengineering.
CONCLUSIONS
These last 40 years have certainly changed the course of natural history for children born with any form of "functionally" single ventricle, thanks to the improvement in diagnostic and treatment techniques, and particularly to the increased knowledge of the morphology and function of these complex hearts, from fetal to adult life. There is still much left unexplored and room for improvement, and all efforts should be concentrated in collaborations among different institutions and specialties, focused on the same matter.
PubMed: 36891374
DOI: 10.21037/tp-22-573 -
Journal of the American College of... May 2022
Topics: Fontan Procedure; Heart Ventricles; Humans; Treatment Outcome; Tricuspid Valve; Tricuspid Valve Insufficiency
PubMed: 35331852
DOI: 10.1016/j.jacc.2022.03.003 -
Journal of Cardiovascular Imaging Apr 2021The Fontan procedure is a well-established surgical technique to improve survival in patients with univentricular heart disease. The procedure reroutes the systemic... (Review)
Review
The Fontan procedure is a well-established surgical technique to improve survival in patients with univentricular heart disease. The procedure reroutes the systemic venous flow to the lungs, bypassing the right ventricle. The originally proposed method involved direct anastomosis of the right atrium to the pulmonary artery. Since then, several modifications have been made in the original technique leading to the modern Fontan, or total cavopulmonary connection. The modern Fontan technique has shown improved surgical outcomes and increased life expectancy in patients with univentricular disease. Due to the increased survival of these patients, long-term complications are becoming more prevalent. Common complications of Fontan procedure include right atrial dilatation and thrombosis; conduit stenosis and thrombosis; right-to-left and left-to-right shunts; hepatic congestion and cirrhosis; and lymphovascular. Computed tomography (CT) can reliably depict the normal Fontan anatomy and various postoperative complications. A fundamental understanding of the techniques of CT, including imaging protocols and common interpretive pitfalls, allows targeted imaging and precise reporting of clinically significant findings. Radiologists should be familiar with the multiple stages of single-ventricle palliation, normal Fontan anatomy, pathophysiology, and imaging features of common Fontan-related complications.
PubMed: 33605094
DOI: 10.4250/jcvi.2020.0119 -
Diagnostics (Basel, Switzerland) Apr 2024A heart with a borderline ventricle refers to a situation where there is uncertainty about whether the left or right underdeveloped ventricle can effectively support the... (Review)
Review
A heart with a borderline ventricle refers to a situation where there is uncertainty about whether the left or right underdeveloped ventricle can effectively support the systemic or pulmonary circulation with appropriate filling pressures and sufficient physiological reserve. Pediatric cardiologists often deal with congenital heart diseases (CHDs) associated with various degrees of hypoplasia of the left or right ventricles. To date, no specific guidelines exist, and surgical management may be extremely variable in different centers and sometimes even in the same center at different times. Thus, the choice between the single-ventricle or biventricular approach is always controversial. The aim of this review is to better define when "small is too small and large is large enough" in order to help clinicians make the decision that could potentially affect the patient's entire life.
PubMed: 38667469
DOI: 10.3390/diagnostics14080823 -
The Journal of Thoracic and... Oct 2021
Topics: Fontan Procedure; Hemodynamics; Homeostasis; Humans; Models, Cardiovascular; Phosphodiesterase 5 Inhibitors; Postoperative Complications; Pulmonary Circulation; Treatment Outcome; Univentricular Heart; Vascular Resistance
PubMed: 33618865
DOI: 10.1016/j.jtcvs.2020.08.114