Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation

Antonio, Marta de; Gordo, Andreia; Pereira, Conceicao; Pinto, Fátima; Fragata, Isabel; Fragata, José

doi:10.1016/j.athoracsur.2015.08.079

Cited by 41 publications

(26 citation statements)

References 8 publications

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“…However, the mucosal histology in PLE of these subjects is identical to that found in primary intestinal lymphangiectasia and constrictive pericarditis,52–57 and it is now generally accepted that the PLE is the result of the high systemic venous pressure that leads to lymphatic rupture in the gut. Direct evidence that PLE in Fontan subjects results from the increased lymphatic pressure is provided by the recent description of dramatic improvement of PLE following diversion of the innominate vein (which drains the thoracic duct) to the low-pressure atrium 55. The deleterious effect of the resultant shunting of blood past the lung with the concomitant arterial desaturation is considered to be of minor clinical importance relative to the benefit that is achieved with the alleviation of the PLE.…”

Section: Increased Lymphatic Pressurementioning

confidence: 99%

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states

Levitt¹,

Levitt²

2017

CEG

116

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Protein losing enteropathy (PLE) has been associated with more than 60 different conditions, including nearly all gastrointestinal diseases (Crohn’s disease, celiac, Whipple’s, intestinal infections, and so on) and a large number of non-gut conditions (cardiac and liver disease, lupus, sarcoidosis, and so on). This review presents the first attempt to quantitatively understand the magnitude of the PLE in relation to the associated pathology for three different disease categories: 1) increased lymphatic pressure (e.g., lymphangiectasis); 2) diseases with mucosal erosions (e.g., Crohn’s disease); and 3) diseases without mucosal erosions (e.g., celiac disease). The PLE with lymphangiectasis results from rupture of the mucosal lymphatics, with retrograde drainage of systemic lymph into the intestinal lumen with the resultant loss of CD4 T cells, which is diagnostic. Mucosal erosion PLE results from macroscopic breakdown of the mucosal barrier, with the epithelial capillaries becoming the rate-limiting factor in albumin loss. The equation derived to describe the relationship between the reduction in serum albumin (CP) and PLE indicates that gastrointestinal albumin clearance must increase by at least 17 times normal to reduce the CP by half. The strengths and limitations of the two quantitative measures of PLE (51Cr-albumin or α1-antitrypsin [αAT] clearance) are reviewed. αAT provides a simple quantitative diagnostic test that is probably underused clinically. The strong, unexplained correlation between minor decreases in CP and subsequent mortality in seemingly healthy individuals raises the question of whether subclinical PLE could account for the decreased CP and, if so, could the mechanism responsible for PLE play a role in the increased mortality? A large-scale study correlating αAT clearance with serum albumin concentrations will be required in order to determine the role of PLE in the regulation of the serum albumin concentration of seemingly healthy subjects.

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Section: Increased Lymphatic Pressurementioning

confidence: 99%

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states

Levitt¹,

Levitt²

2017

CEG

116

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“…These differences may be useful to monitor hemodynamic conditions of patients with Fontan palliation and guide interventions for prevention or relief of complications associated with Fontan single‐ventricle physiology. Symptomatic improvement of plastic bronchitis has been reported with lymphatic embolization with MRI guidance, and lymphatic drainage improved in protein losing enteropathy by diverting the thoracic duct to the common atrium . Echocardiography is a widely available, noninvasive technique that could be used as a first step in evaluating the thoracic duct.…”

Section: Introductionmentioning

confidence: 99%

The thoracic duct and the Fontan patient

et al. 2017

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“…Recent data suggest dysregulation of lymphatic circulation can contribute to the development of PLE in Fontan patients, and mechanical blockage of lymphatic drainage can be identified in significant numbers of Fontan patients with PLE . Surgical decompression of the thoracic duct has been shown to relieve symptoms in two Fontan patients with otherwise refractory PLE . Further evidence supporting a link between disrupted lymph circulation and PLE in Fontan patients is the observation that Fontan patients with PLE display immune abnormalities which are also found in patients with PLE secondary to intestinal lymphangiectasia, but not in patients with PLE related to ulcerative colitis or celiac disease …”

Section: Discussionmentioning

confidence: 99%

Dopamine as a potential rescue therapy for refractory protein‐losing enteropathy in Fontan‐palliated patients

Friedland‐Little

Gajarski

Schumacher

2017

Pediatric Transplantation

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PLE is an important cause of morbidity and mortality in patients who have undergone Fontan palliation. While multiple PLE therapies have been reported, none has proved consistently effective. Patients who do not respond to "standard" PLE therapies face poor long-term outcomes. We report here a significant response to dopamine infusion in three patients with chronic, refractory PLE. We hypothesize that this response may be at least partially due to a dopamine effect on lymphatic receptors rather than to an augmentation of cardiac output.

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Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation

Cited by 41 publications

References 8 publications

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states

The thoracic duct and the Fontan patient

Dopamine as a potential rescue therapy for refractory protein‐losing enteropathy in Fontan‐palliated patients

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