Haemodynamic characteristics before and after the onset of protein losing enteropathy in patients after the Fontan operation

Ohuchi, Hideo; Yasuda, Kenji; Miyazaki, Aya; Kitano, Masataka; Sakaguchi, Heima; Yazaki, Satoshi; Tsuda, Etsuko; Yamada, Osamu

doi:10.1093/ejcts/ezs714

Cited by 61 publications

(30 citation statements)

References 19 publications

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“…There is a correlation of PLE with the central venous pressure at the time of development of PLE, but this correlation disappears during the chronic phase 58. However, this correlation is weak, with some patients developing PLE even though they have a “good” surgical result (i.e., right atrial pressure <15 mmHg) 51.…”

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

105

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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

105

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“…The defect renders the Fontan circulation dependent critically on elevated central venous pressure to maintain cardiac output (CO). As a consequence, Fontan patients often have suboptimal hemodynamic conditions compared with normal biventricular subjects, such as higher venous pressure and lower CO (58,59). More importantly, the single-ventricle defect significantly impairs the regulatory capacity of the circulatory system, resulting in profound cardiovascular limitations under stress, typically during exercise (27,62,76).…”

mentioning

confidence: 99%

Hemodynamic performance of the Fontan circulation compared with a normal biventricular circulation: a computational model study

Liang

Senzaki

Kurishima

et al. 2014

American Journal of Physiology-Heart and Circulatory Physiology

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performance of the Fontan circulation compared with a normal biventricular circulation: a computational model study. Am J Physiol Heart Circ Physiol 307: H1056 -H1072, 2014. First published July 25, 2014 doi:10.1152/ajpheart.00245.2014The physiological limitations of the Fontan circulation have been extensively addressed in the literature. Many studies emphasized the importance of pulmonary vascular resistance in determining cardiac output (CO) but gave little attention to other cardiovascular properties that may play considerable roles as well. The present study was aimed to systemically investigate the effects of various cardiovascular properties on clinically relevant hemodynamic variables (e.g., CO and central venous pressure). To this aim, a computational modeling method was employed. The constructed models provided a useful tool for quantifying the hemodynamic effects of any cardiovascular property of interest by varying the corresponding model parameters in model-based simulations. Herein, the Fontan circulation was studied compared with a normal biventricular circulation so as to highlight the unique characteristics of the Fontan circulation. Based on a series of numerical experiments, it was found that 1) pulmonary vascular resistance, ventricular diastolic function, and systemic vascular compliance play a major role, while heart rate, ventricular contractility, and systemic vascular resistance play a secondary role in the regulation of CO in the Fontan circulation; 2) CO is nonlinearly related to any single cardiovascular property, with their relationship being simultaneously influenced by other cardiovascular properties; and 3) the stability of central venous pressure is significantly reduced in the Fontan circulation. The findings suggest that the hemodynamic performance of the Fontan circulation is codetermined by various cardiovascular properties and hence a full understanding of patientspecific cardiovascular conditions is necessary to optimize the treatment of Fontan patients.

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“…In Fontan patients, systemic venous hypertension is responsible for endorgan failure, like protein losing enteropathy or hepatic fibrosis, leading to death or heart transplantation[10, 11]. As heart transplantation is a limited option, several technologies, including mechanically assisted Fontan circulation and cardiomyoplasty procedures have been attempted[12–14].…”

Section: Discussionmentioning

confidence: 99%

Towards a Tissue-Engineered Contractile Fontan-Conduit: The Fate of Cardiac Myocytes in the Subpulmonary Circulation

et al. 2016

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The long-term outcome of patients with single ventricles improved over time, but remains poor compared to other congenital heart lesions with biventricular circulation. Main cause for this unfavourable outcome is the unphysiological hemodynamic of the Fontan circulation, such as subnormal systemic cardiac output and increased systemic-venous pressure. To overcome this limitation, we are developing the concept of a contractile extracardiac Fontan-tunnel. In this study, we evaluated the survival and structural development of a tissue-engineered conduit under in vivo conditions. Engineered heart tissue was generated from ventricular heart cells of neonatal Wistar rats, fibrinogen and thrombin. Engineered heart tissues started beating around day 8 in vitro and remained contractile in vivo throughout the experiment. After culture for 14 days constructs were implanted around the right superior vena cava of Wistar rats (n = 12). Animals were euthanized after 7, 14, 28 and 56 days postoperatively. Hematoxylin and eosin staining showed cardiomyocytes arranged in thick bundles within the engineered heart tissue-conduit. Immunostaining of sarcomeric actin, alpha-actin and connexin 43 revealed a well -developed cardiac myocyte structure. Magnetic resonance imaging (d14, n = 3) revealed no constriction or stenosis of the superior vena cava by the constructs. Engineered heart tissues survive and contract for extended periods after implantation around the superior vena cava of rats. Generation of larger constructs is warranted to evaluate functional benefits of a contractile Fontan-conduit.

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Haemodynamic characteristics before and after the onset of protein losing enteropathy in patients after the Fontan operation

Cited by 61 publications

References 19 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

Hemodynamic performance of the Fontan circulation compared with a normal biventricular circulation: a computational model study

Towards a Tissue-Engineered Contractile Fontan-Conduit: The Fate of Cardiac Myocytes in the Subpulmonary Circulation

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