Protein-Losing Enteropathy in Patients With Congenital Heart Disease

Itkin, Maxim; Piccoli, David A.; Nadolski, Gregory J.; Rychik, Jack; DeWitt, Aaron; Pinto, Erin; Rome, Jonathan J.; Dori, Yoav

doi:10.1016/j.jacc.2017.04.023

Cited by 146 publications

(136 citation statements)

References 20 publications

Supporting

Mentioning

131

Contrasting

Order By: Relevance

“…It has been hypothesized that lymphatic over‐distention due to transmission of elevated central venous pressure in predisposed patients can lead to the rupture of intestinal lymphatic vessels and to ensuing leakage of protein‐rich lymph into the lumen of the small intestine 1. Despite that increased central venous pressure is considered to be the prerequisite for cardiac disease‐related PLE, our results illustrate that degree of PLE poorly correlates with haemodynamics, indicating that other factors are causally involved 2.…”

Section: Discussionmentioning

confidence: 63%

“…It is plausible to speculate that protracted and profound reduction of right atrial pressure after transplantation led to redirection of lymphatic flow from the gut into the liver and ductus thoracicus 1. Such unloading may even lead to natural ‘sealing’ of perforated lymphatic channels.…”

Section: Discussionmentioning

confidence: 99%

“…PLE may occur as a late complication of Fontan procedure1, 2, 3, 4 or in patients with constrictive pericarditis4, 5 or rheumatic tricuspid regurgitation,6 but it is probably very rare in other conditions that lead to biventricular heart failure (HF). The mechanisms and reasons responsible for PLE development are still poorly understood.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Protein‐losing enteropathy in an adult with non‐ischaemic cardiomyopathy: complete reversal by heart transplantation

2018

View full text Add to dashboard Cite

Protein‐losing enteropathy (PLE) due to leakage of lymph into the gut sometimes occurs in young patients after Fontan palliation but is very rarely reported with other aetiologies of chronic heart failure (HF). PLE leads to severe hypoalbuminemia and immunodeficiency and is associated with poor prognosis. The mechanisms and the predispositions to PLE are poorly understood. Here, we report an adult patient with advanced HF due to non‐ischaemic non‐dilated hypocontractile cardiomyopathy who developed severe PLE, probably owing to increased ventricular stiffness and constraint by atypically placed epicardial electrode encircling both ventricles. Importantly, both PLE and immunodeficiency completely resolved after heart transplantation.

show abstract

Section: Discussionmentioning

confidence: 63%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Protein‐losing enteropathy in an adult with non‐ischaemic cardiomyopathy: complete reversal by heart transplantation

2018

View full text Add to dashboard Cite

show abstract

“…The chronically elevated hepatic venous pressure leads to mechanical transduction of hepatic stellate cells and transformation into myofibroblasts, which produce collagen deposition in both centrilobular and portal venous patterns. In addition, the chronic venous pressure elevation on the gut may lead to protein‐losing enteropathy, characterized by the loss of serum protein into the intestine and hypoproteinemia, leading to peripheral edema, ascites, pleural effusions, and malnutrition that further complicate the liver disease …”

Section: Chd and The Livermentioning

confidence: 99%

Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation

et al. 2019

View full text Add to dashboard Cite

There is close interaction between the functions of the liver and heart affecting the presentation, diagnosis, and outcome of acute and chronic cardiac and liver disease. Conditions affecting both organ systems should be considered when proposing transplantation because the interaction between cardiac disease and liver disease has implications for diagnosis, management, selection for transplantation, and, ultimately, for longterm outcomes after liver transplantation (LT). The combination of cardiac and liver disease is well recognized in adults but is less appreciated in pediatric patients. The focus of this review is to describe conditions affecting both the liver and heart and how they affect selection and management of LT in the pediatric population.

show abstract

“…However, even though all Fontan patients are victims of an elevated CVP, not all develop PLE. This suggests local lymphatic problems, with possible additional causes such as diminished cardiac output, increased mesenteric vascular resistance, inflammation/infection, intestinal cell heparan sulfate depletion, or other factors …”

Section: Introductionmentioning

confidence: 99%

Percutaneous embolization of lymphatic fistulae as treatment for protein‐losing enteropathy and plastic bronchitis in patients with failing Fontan circulation

Maleux

Storme

Cools

et al. 2019

Cathet Cardio Intervent

View full text Add to dashboard Cite

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 vessels in periportal (PLE) or paratracheal (PB) position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepatoduodenal or tracheal fistulae. After flushing with 10% glucose solution, occlusion of hepatoduodenal or paratreacheal lymphatics was effected by injection of 1–4 cc mixture 4/1 of Lipiodol/n‐butyl cyanoacrylate (n‐BCA; Histoacryl). Results Seven patients with proven PLE were treated with periportal lymphatic embolization 10.7 (range: 6.6–13.5) years after the Fontan operation. The Fontan operation was performed at a median age of 3.7 (range: 2.9–5.7) years and PLE started a median of 3.1 (range: 0.9–4.7) years later. Five patients required a second procedure 2–8 months later. Complications were limited (spillage of glue in portal branch, transient cholangitis, and caustic duodenal bleeding). Six of seven patients reported significant improvement in quality of life and normalization of albumin levels after limited follow‐up (p < .01). One patient (Fontan at 2.9 years; age 16.4 years) had PB for 2 years. Selective transthoracic cone‐beam‐directed puncture of left and right paratracheal lymphatics with n‐BCA embolization of distal lymphatic fistulae resulted in lasting absence of tracheal casts (11 months). Conclusions Embolization of periportal/peritracheal lymphatics is a promising technique in Fontan patients with PLE/PB. Larger series are required to determine incidence and reasons of success/failure, with long‐term results and effects on liver function.

show abstract

Protein-Losing Enteropathy in Patients With Congenital Heart Disease

Cited by 146 publications

References 20 publications

Protein‐losing enteropathy in an adult with non‐ischaemic cardiomyopathy: complete reversal by heart transplantation

Protein‐losing enteropathy in an adult with non‐ischaemic cardiomyopathy: complete reversal by heart transplantation

Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation

Percutaneous embolization of lymphatic fistulae as treatment for protein‐losing enteropathy and plastic bronchitis in patients with failing Fontan circulation

Contact Info

Product

Resources

About