Protein-losing enteropathy after Fontan operation

Hess, John; K, Kruizinga; Bijleveld, Cma; Hardjowijono, R; Eygelaar, A

doi:10.1016/s0022-5223(19)38299-6

Cited by 88 publications

(20 citation statements)

References 13 publications

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“…The more contemporary association of protein-losing enteropathy with patients having undergone Fontan procedures for palliation of complex congenital cardiac disease did not become apparent until the 1980s. 7,8 From these and subsequent studies, the complexity of protein-losing enteropathy as a disease process is apparent, and so too are the consequences. Mortality associated with protein-losing enteropathy in this population is high, 1,2 the morbidity is incalculable, and attempts at therapy remain frustratingly ineffective.…”

Section: Discussionmentioning

confidence: 99%

Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment

Meadows

Jenkins

2011

Cardiol Young

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Protein-losing enteropathy is a relatively uncommon complication of Fontan procedures for palliation of complex congenital cardiac disease. However, the relative infrequency of protein-losing enteropathy belies the tremendous medical, psychosocial and financial burdens it places upon afflicted patients, their families and the healthcare system that supports them. Unfortunately, because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and attempts at treatment seldom yield long-term success. The most comprehensive analyses of protein-losing enteropathy in this patient population are now over a decade old, and re-evaluation of the prevalence and progress in treatment of this disease is needed. This report describes a single institution experience with the evaluation, management, and treatment of protein-losing enteropathy in patients with congenital cardiac disease in the current era, follows with a comprehensive review of protein-losing enteropathy, focused upon what is known and not known about the pathophysiology of protein-losing enteropathy in this patient population, and concludes with suggestions for prevention and treatment.

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Section: Discussionmentioning

confidence: 99%

Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment

Meadows

Jenkins

2011

Cardiol Young

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“…In an earlier study, increased right atrial diastolic pressure is shown to be associated with PLE. 6 The diagnosis of PLE is made on the basis of history and data for surgical correction of Complex Congenital Heart Diseases, clinical symptoms and laboratory confirmation. Alpha-1-antitrypsin is a protein normally found in the blood and in the stool.…”

Section: Discussionmentioning

confidence: 99%

An unusual cause of edema in a child with congenital heart disease post Fontan procedure

2019

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Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.

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“…Pressures in the hepatic veins are higher than normal, with abnormal liver haemodynamics predisposing to late liver complications [8]. Elevated thoracic duct pressures are thought to contribute to prolonged chylous effusions in the early post-Fontan period [9], and later to the development of plastic bronchitis [10,11] and protein-losing enteropathy (PLE) [12,13].…”

Section: Effects Of Systemic Venous Hypertensionmentioning

confidence: 99%

“…This complication of the Fontan circulation has challenged physicians for many years [12,19], diagnosed by a low serum albumin level and the presence of high faecal α 1 -antitrypsin levels [20]. It is a characteristic feature of Fontan failure, leading to peripheral oedema, ascites, pleural effusions, malabsorption and loss of immunoglobulins.…”

Section: Protein-losing Enteropathymentioning

confidence: 99%

Managing adult Fontan patients: where do we stand?

Clift

Celermajer

2016

Eur Respir Rev

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The Fontan operation is performed as a palliative procedure to improve survival in infants born with a functionally univentricular circulation. The success of the operation is demonstrated by a growing adult Fontan population that exists with this unique physiology. Late follow-up has demonstrated expected and unexpected sequelae, and has shown multisystem effects of this circulation. This review discusses the challenges of managing the late complications in terms of understanding this unique physiology and the innovative therapeutic interventions that are being investigated. The challenge remains to maintain quality of life for adult survivors, as well as extending life expectancy.

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Protein-losing enteropathy after Fontan operation

Cited by 88 publications

References 13 publications

Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment

Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment

An unusual cause of edema in a child with congenital heart disease post Fontan procedure

Managing adult Fontan patients: where do we stand?

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