scholarly journals Atrial pacing: An alternative treatment for protein-losing enteropathy after the Fontan operation

2001 ◽  
Vol 121 (3) ◽  
pp. 582-583 ◽  
Author(s):  
Mitchell I. Cohen ◽  
Larry A. Rhodes ◽  
Gil Wernovsky ◽  
J.William Gaynor ◽  
Thomas L. Spray ◽  
...  
Keyword(s):  
Alternative Treatment ◽  
Fontan Operation ◽  
Atrial Pacing ◽  
Protein Losing Enteropathy

scholarly journals Pathological and Immunological Approaches for a Patient Complicated with Protein-losing Enteropathy Following the Fontan Operation

2012 ◽  
Vol 28 (4) ◽  
pp. 224-229
Author(s):  
Kentaro Ueno ◽  
Hideaki Nakamura ◽  
Ryohei Gatayama ◽  
Sadamitsu Yanagi ◽  
Hideaki Ueda ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Protein Losing Enteropathy

scholarly journals Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation

2018 ◽  
Vol 38 (6) ◽  
pp. e5-e12 ◽  
Author(s):  
Christine Peyton
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Complex Disease ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Venous Hypertension ◽  
Plastic Bronchitis ◽  
Protein Losing Enteropathy ◽  
Fontan Physiology

Protein-losing enteropathy and plastic bronchitis remain challenging to treat despite recent treatment advances. Protein-losing enteropathy and plastic bronchitis have been diagnosed in patients with cardiomyopathy, constrictive pericarditis, and congestive heart failure. This article focuses on patients with protein-losing enteropathy or plastic bronchitis following the Fontan procedure. Patients with single-ventricle physiology who have undergone the Fontan procedure are at risk for these conditions. Fontan physiology predisposes patients to chronically low cardiac output, increased central venous pressure, and congestive heart failure. These altered hemodynamics lead to increased mesenteric vascular resistance, resulting in venous hypertension and congestion in protein-losing enteropathy. Plastic bronchitis is a complex disease in which chronic high lymphatic pressures from Fontan physiology cause acellular bronchial casts to develop. These entities may also occur in patients with normal Fontan hemodynamics. This article also covers medical and surgical interventions for protein-losing enteropathy and plastic bronchitis. (Critical Care Nurse. 2018;38[6]:e5–e12)

Current treatment options for the Failing Fontan Circulation

2022 ◽  
Vol 18 ◽  
Author(s):  
Bart. W. Driesen ◽  
Michiel Voskuil ◽  
Heynric B. Grotenhuis
Keyword(s):  
Myocardial Dysfunction ◽  
Fontan Procedure ◽  
Treatment Options ◽  
Fontan Operation ◽  
Current Treatment ◽  
Fontan Circulation ◽  
Protein Losing Enteropathy ◽  
Resistance Protein ◽  
Failing Fontan

Abstract: The Fontan operation was introduced in 1968. For congenital malformations where biventricular repair is not suitable, the Fontan procedure has provided a long-term palliation strategy with improved outcome compared to the initially developed procedures. Despite these improvements, several complications merely as a result of a failing Fontan circulation (including myocardial dysfunction, arrhythmias, increased pulmonary vascular resistance, protein losing enteropathy, hepatic dysfunction, plastic bronchitis and thrombo-embolism) will limit life-expectancy in this patient cohort. This review provides an overview of the most common complications of the Fontan circulation and the currently available treatment options.

scholarly journals Contractility–Afterload Mismatch in Patients With Protein-Losing Enteropathy After the Fontan Operation

2014 ◽  
Vol 35 (7) ◽  
pp. 1225-1231 ◽  
Author(s):  
Hideto Ozawa ◽  
Takayoshi Ueno ◽  
Shigemitsu Iwai ◽  
Hiroaki Kawata ◽  
Kyouichi Nishigaki ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Protein Losing Enteropathy

scholarly journals Protein-losing enteropathy after the Fontan operation

1996 ◽  
Vol 112 (3) ◽  
pp. 672-680 ◽  
Author(s):  
Robert H. Feldt ◽  
David J. Driscoll ◽  
Kenneth P. Offord ◽  
Ruth H. Cha ◽  
Jean Perrault ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Protein Losing Enteropathy

Rare problems associated with the Fontan circulation

2010 ◽  
Vol 20 (S3) ◽  
pp. 113-119 ◽  
Author(s):  
David J. Goldberg ◽  
Kathryn Dodds ◽  
Jack Rychik
Keyword(s):  
Fontan Operation ◽  
Venous Pressure ◽  
Multiple Organ ◽  
Univentricular Heart ◽  
Protein Losing Enteropathy ◽  
Congenital Cardiac Disease ◽  
Organ Systems ◽  
Coagulation Protein ◽  
Life Threatening ◽  
Fontan Physiology

AbstractThe Fontan operation, originally described for the surgical management of tricuspid atresia, is now the final surgery in the strategy of staged palliation for a number of different forms of congenital cardiac disease with a functionally univentricular heart. Despite the improved technical outcomes of the Fontan operation, staged palliation does not recreate a normal physiology. Without a pumping chamber delivering blood to the lungs, the cardiovascular system is less efficient; cardiac output is generally diminished, and the systemic venous pressure is increased. As a result, patients with “Fontan physiology” may face a number of rare but potentially life-threatening complications including hepatic dysfunction, abnormalities of coagulation, protein-losing enteropathy, and plastic bronchitis. Despite the staged palliation resulting in remarkable survival, the possible complications for this group of patients are complex, involve multiple organ systems, and can be life threatening. Identifying the mechanisms associated with each of the rare complications, and developing strategies to treat them, requires the work of many people at many institutions. Continued collaboration between sub-specialists and between institutions will be required to optimise the care for this group of survivors with functionally univentricular hearts.

Gastrointestinal Bleeding and Protein-Losing Enteropathy After the Fontan Operation

2006 ◽  
Vol 98 (5) ◽  
pp. 666-667 ◽  
Author(s):  
Himeshkumar Vyas ◽  
David J. Driscoll ◽  
Frank Cetta ◽  
Conor G. Loftus ◽  
Heidi M. Connolly
Keyword(s):  
Gastrointestinal Bleeding ◽  
Fontan Operation ◽  
Protein Losing Enteropathy

New concepts: development of a survivorship programme for patients with a functionally univentricular heart

2011 ◽  
Vol 21 (S2) ◽  
pp. 77-79 ◽  
Author(s):  
David J. Goldberg ◽  
Kathryn Dodds ◽  
Jack Rychik
Keyword(s):  
Fontan Operation ◽  
Univentricular Heart ◽  
Plastic Bronchitis ◽  
Complete Understanding ◽  
Protein Losing Enteropathy ◽  
New Concepts ◽  
Fontan Physiology ◽  
The Impact ◽  
Valuable Service

AbstractChildren with functionally univentricular hearts are now surviving into their third and fourth decades of life. Although survival alone is a remarkable achievement, a lot must still be done to improve the quality and duration of life after the Fontan operation. Challenges that may be faced by these patients include the impact of the Fontan operation on the liver and the density of bone, protein-losing enteropathy, and plastic bronchitis. Paediatric cardiologists are familiar with the haemodynamic issues inherent in Fontan physiology; however, training in cardiology is often not sufficient to give us a complete understanding of the pathophysiology of the complications or of the options for treatment. Collaboration with other subspecialists including gastroenterologists, endocrinologists, and pulmonologists is essential in order to provide the rigorous and nuanced care that our patients need and deserve. A clinic in which a patient can see multiple subspecialists, and in which the subspecialists, as a group, can discuss each patient, can provide a unique and valuable service for patients with a functionally univentricular heart.

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