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Published on: May 1, 2015
Lymphatic obstruction and protein-losing enteropathy in patients with congenital heart disease
Jeffery Meadows1, Kimberlee Gauvreau, Kathy Jenkins
1Department of Cardiology, Children's Hospital, Boston, MA, USA. jeffery.meadows@ucsf.edu
Insights
Protein-losing enteropathy (PLE) in congenital heart disease patients may stem from lymphatic obstruction. This study suggests physical lymphatic blockage plays a key role in PLE development, impacting patient outcomes.
Area of Science:
- Cardiology
- Gastroenterology
- Pediatric Surgery
Background:
- Protein-losing enteropathy (PLE) is a recognized complication following surgical correction of congenital heart disease (CHD).
- The exact causes and mechanisms of PLE are not fully understood, but lymphatic system dysfunction is suspected.
- Lymphatic insufficiency is believed to be a central factor in the development of PLE.
Purpose of the Study:
- To investigate the potential role of lymphatic obstruction in patients with CHD and PLE.
- To examine the association between central venous catheter-related thrombosis and PLE in this patient population.
Main Methods:
- A case-control study was conducted comparing patients with CHD and PLE to matched controls who underwent similar surgical procedures.
- Lymphatic return obstruction was defined by thoracic duct ligation or complete central venous obstruction at the thoracic duct's drainage site.
Main Results:
- Apparent lymphatic obstruction was observed in 25% of PLE cases versus 4% of controls (P = .06).
- No significant association was found between PLE and central venous catheter use, duration, or specific patient/operative characteristics.
- Mortality was higher in the PLE group (25%) compared to controls (9%), though not statistically significant. Long-term PLE resolution was achieved in 38% of patients.
Conclusions:
- A high incidence of lymphatic obstruction suggests it may be a significant, previously unrecognized factor in the pathogenesis of PLE in complex CHD.
- Further research is warranted to elucidate the precise mechanisms linking lymphatic compromise to PLE in this vulnerable patient group.
Objective:
Protein-losing enteropathy (PLE) is a known complication of surgical procedures for congenital heart disease. The pathogenesis and pathophysiology of PLE remain poorly understood. However, lymphatic insufficiency appears central to the disease process. We sought to investigate the role of lymphatic obstruction and central venous catheter-related central venous thrombosis in patients with congenital heart disease and PLE.
Design:
A case-control study design was constructed consisting of patients with congenital heart disease and PLE and 2:1 matched controls having undergone the same definitive surgical procedure. Obstruction to lymphatic return was considered present if the thoracic duct was ligated, or if there was complete central venous obstruction at the usual site of thoracic duct drainage.
Results:
Obstruction to lymphatic return was identified in 4 of 16 cases (25%) and 1 of 32 controls (4%), P = .06. There was no association between PLE and central venous catheter use or duration, and no discriminating characteristics between cases and controls with respect to anatomy, pre-Fontan hemodynamic variables, operative or perioperative factors, or hemodynamic variables at the time of PLE diagnosis. Mortality for patients with PLE was 25% compared with 9% in controls (P = not significant). Long-term resolution of PLE was obtained in six patients (38%).
Conclusion:
There is a high prevalence of apparent lymphatic obstruction in patients with congenital heart disease and PLE, suggesting that physical lymphatic obstruction may play an important, and previously unrecognized role in the development of PLE in patients with complex congenital heart disease.
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