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Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study
H Sonali Magdo1, Terri L Stillwell2, Matthew J Greenhawt3
1University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
Insights
Patients with protein-losing enteropathy (PLE) after Fontan surgery exhibit significant immune deficiencies, especially low CD4 T cell counts. These findings highlight the immunologic impact of PLE in this patient population.
Area of Science:
- Immunology
- Cardiology
- Gastroenterology
Background:
- Protein-losing enteropathy (PLE) is a known complication following Fontan surgery.
- The immunologic consequences of PLE post-Fontan are not fully understood.
- Understanding immune function in these patients is crucial for managing potential complications.
Purpose of the Study:
- To characterize the immune profiles of patients with PLE post-Fontan.
- To compare immune status between patients with and without PLE post-Fontan.
- To identify specific immune abnormalities associated with PLE post-Fontan.
Main Methods:
- Prospective study of 16 patients (8 with PLE, 8 controls) post-Fontan.
- Laboratory assessment of immune function markers.
- Comparison of demographics, cardiac history, immune characteristics, and infection history between groups.
Main Results:
- All patients with PLE had lymphopenia and markedly depressed CD4 T cell counts (median 58 cells/μL) compared to controls (median 450 cells/μL).
- CD4% was significantly lower in the PLE group (12.3%) versus controls (36.9%).
- A majority of PLE patients had negative vaccine titers (measles, mumps, rubella), unlike controls, despite no difference in infection frequency.
Conclusions:
- Patients with Fontan-associated PLE demonstrate significant quantitative immune abnormalities, notably CD4 deficiency.
- These immune deficits resemble those seen in non-Fontan patients with PLE due to intestinal lymphangiectasia.
- The findings underscore the profound immunologic impact of PLE in the context of Fontan circulation.
Objective:
To comprehensively characterize the immunologic characteristics of patients with protein-losing enteropathy (PLE) post-Fontan and compare them with patients without PLE post-Fontan.
Study Design:
Patients with PLE post-Fontan and age-matched controls post-Fontan were prospectively studied with laboratory markers of immune function. Infectious history was obtained by interview and chart review. The groups' demographics, cardiac history, immune characteristics, and infection history were compared using appropriate 2-group statistics.
Results:
A total of 16 patients enrolled (8 patients with PLE and 8 controls). All patients with PLE had lymphopenia compared with 25% of controls (P = .01). All patients with PLE had markedly depressed CD4 T cell counts (median 58 cells/μL) compared with controls (median 450 cells/μL, P = .0002); CD4% was also low in the PLE group (12.3%) and normal in control (36.9%, P = .004). Both groups had mildly depressed CD8 T cells and normal to slightly elevated natural killer and B-cell subsets. A majority of patients with PLE (62.5%) had negative titers to measles, mumps, and rubella vaccination, compared with no control Fontan with a negative titer (P = .03). Despite profoundly low CD4 counts, the frequency of infection was not different between groups with no reported opportunistic infections.
Conclusions:
Patients with Fontan-associated PLE have extensive quantitative immune abnormalities, particularly CD4 deficiency. These immune abnormalities are similar to those found in non-Fontan patients with PLE caused by intestinal lymphangiectasia.
