Fontan procedure ≈ Fontan procedure
View article: The Fontan circulation after 45 years: update in physiology
The Fontan circulation after 45 years: update in physiology Open
The Fontan operation was first performed in 1968. Since then, this operation has been performed on thousands of patients worldwide. Results vary from very good for many decades to very bad with a pleiad of complications and early death. A …
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Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study Open
Background Congestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking. Methods and Results Liver biopsies and cardiac catherizations were performed as …
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Fontan-Associated Liver Disease Open
Surgical innovation and multidisciplinary management have allowed children born with univentricular physiology congenital heart disease to survive into adulthood. An estimated global population of 70 000 patients have undergone the Fontan …
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Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions Open
Universally fatal only 4 decades ago, the progress in the 3-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for these patients. Although the stage II ope…
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Results of the FUEL Trial Open
Background: The Fontan operation creates a total cavopulmonary connection, a circulation in which the importance of pulmonary vascular resistance is magnified. Over time, this circulation leads to deterioration of cardiovascular efficiency…
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MRI Evaluation of Lymphatic Abnormalities in the Neck and Thorax after Fontan Surgery: Relationship with Outcome Open
Background The Fontan operation is performed for surgical palliation of single ventricle physiology. This operation is usually preceded by a superior cavopulmonary connection (SCPC); lymphatic abnormalities after SCPC may be demonstrated a…
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Hemodynamics of Fontan Failure Open
Background Nonpulsatile pulmonary blood flow in Fontan circulation results in pulmonary vascular disease, but the potential relationships between pulmonary vascular resistance index (PVRI) and Fontan failure have not been studied. The obje…
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Pregnancy in Women With a Fontan Circulation Open
Background: The Fontan operation has provided life-saving palliation and adult survival for individuals born with single ventricle physiology. Many now seek advice about safe pregnancy. Little data are, however, available, consisting mainl…
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Clinical outcome following total cavopulmonary connection: a 20-year single-centre experience Open
Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. H…
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Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK Open
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a complex disease that presents with a broad spectrum of morphological and haemodynamic findings of varying severity. Recently, the aspect of paediatric …
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Reaching consensus for unified medical language in Fontan care Open
Aims The Fontan operation has resulted in improved survival in patients with single‐ventricle congenital heart disease. As a result, there is a growing population of teenagers and adults with a Fontan circulation. Many co‐morbidities have …
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Physical exercise training in patients with a Fontan circulation: A systematic review Open
Background Patients with a Fontan circulation have a reduced exercise capacity, which is an important prognostic predictor of morbidity and mortality. A way to increase exercise capacity in Fontan patients might be exercise training. This …
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Prognostic power of cardiopulmonary exercise testing in Fontan patients: a systematic review Open
Objective Exercise impairment is common in Fontan patients. Our aim is to systematically review previous literature to determine the prognostic value of exercise capacity in older adolescent and adult Fontan patients with respect to late o…
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Energetics of Blood Flow in Cardiovascular Disease Open
Visualization and quantification of the adverse effects of distorted blood flow are important emerging fields in cardiology. Abnormal blood flow patterns can be seen in various cardiovascular diseases and are associated with increased ener…
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Where Is the “Optimal” Fontan Hemodynamics? Open
Fontan circulation is generally characterized by high central venous pressure, low cardiac output, and slightly low arterial oxygen saturation, and it is quite different from normal biventricular physiology. Therefore, when a patient with …
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Body Composition in Young Adults Living With a Fontan Circulation: The Myopenic Profile Open
Background We sought to characterize body composition abnormalities in young patients living with a Fontan circulation and explore potential pathophysiologic associations. Methods and Results Twenty‐eight patients with a Fontan circulation…
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Estimated glomerular filtration rate and urine biomarkers in patients with single-ventricle Fontan circulation Open
Objectives To define whether adults with a Fontan circulation, who have lifelong venous congestion and limited cardiac output, have impaired glomerular filtration rate (GFR) or elevated urinary biomarkers of kidney injury. Methods We measu…
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Providing a framework of principles for conceptualising the Fontan circulation Open
The Fontan operation remains the final palliation for thousands of patients with complex congenital heart disease. By creating a Fontan circuit, control of cardiac output and congestion is wrested away from the ventricle and new haemodynam…
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Heart and heart–liver transplantation in adults with failing Fontan physiology Open
Background As the population of patients with a Fontan palliation grows so does, the number of patients with cardiac failure necessitating orthotopic heart transplant ( OHT ) and combined heart–liver transplant ( CHLT ). There is recent ev…
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Liver Cirrhosis and/or Hepatocellular Carcinoma Occurring Late After the Fontan Procedure ― A Nationwide Survey in Japan ― Open
LC and/or HCC in patients undergoing the Fontan procedure were not rare late complications and were associated with high mortality rates.
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Health‐Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta‐Analysis Open
Background People with a Fontan circulation experience a range of physical, psychosocial and neurodevelopmental challenges alongside, or caused by, their cardiac condition, with significant consequences for health‐related quality of life (…
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State of the art of the Fontan strategy for treatment of univentricular heart disease Open
In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have si…
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Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role? Open
Background Recent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein‐losing enteropathy. However, the role of the lymphatic circulation in early post‐Fontan outcomes is not w…
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Fate of the Fontan connection: Mechanisms of stenosis and management Open
We demonstrated the hemodynamics and angiographic subtypes of conduit stenosis in patients after Fontan, We showed that calcified homografts, stenotic Gore-Tex conduits and lateral tunnels pathways can be safely and effectively stented to …
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Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis Open
Aims The Fontan operation is associated with significant long‐term morbidity and mortality, posing challenges in management. No haemodynamic classification has been established to aid management and prognostication of patients with Fontan …
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Longitudinal Assessment of Outcome From Prenatal Diagnosis Through Fontan Operation for Over 500 Fetuses With Single Ventricle‐Type Congenital Heart Disease: The Philadelphia Fetus‐to‐Fontan Cohort Study Open
Background Prenatal diagnosis of single ventricle‐type congenital heart disease is associated with improved clinical courses. Prenatal counseling allows for optimal delivery preparations and opportunity for prenatal intervention. Expectant…
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The Epidemiology of Persons Living with Fontan in 2020 and Projections for 2030: Development of an Epidemiology Model Providing Multinational Estimates Open
According to this epidemiology model, the Fontan population is growing, partly driven by increased survival rates with the more recent LT-TCPC and EC-TCPC procedures (compared with AP). The 2020/2030 prevalence of persons living with Fonta…
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Percutaneous embolization of lymphatic fistulae as treatment for protein‐losing enteropathy and plastic bronchitis in patients with failing Fontan circulation Open
Background To determine the feasibility and clinical result of selective embolization of hepatoduodenal or paratracheal lymphatics in Fontan patients with protein‐losing enteropathy (PLE) or plastic bronchitis (PB). Methods Dilated lymph v…
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Assessment of Liver Stiffness in Pediatric Fontan Patients Using Transient Elastography Open
Background . Hepatic fibrosis is a potential complication following Fontan surgery and heralds long-term risk for cirrhosis. Transient elastography (TE) is a rapid, noninvasive method to assess liver fibrosis by measuring liver stiffness. …
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Hypoplastic left heart syndrome: a review Open
Background. Hypoplastic left heart syndrome (HLHS) is an etiologically multifactorial congenital heart disease affecting one in 5,000 newborns. Thirty years ago there were no treatment options for this pathology and the natural course of t…