Immune Abnormalities in Patients With Single Ventricle Circulation Precede the Fontan Procedure

Kováčiková, Ľubica; Krasnanova, Veronika; Skrak, Peter; Zahorec, Martin; Kantorova, A; Šemberová, Jana; Bachárová, Ljuba

doi:10.1177/2150135117732529

Cited by 14 publications

(12 citation statements)

References 24 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…with lymphopenia and abnormal lymphocyte subpopulation dispersion. 9,[24][25][26] Postoperative lymphatic perfusion pattern abnormalities in the 6-month MRI might be partially explained by unfavorable postoperative hemodynamics and are associated with early Fontan complications in our study. In contrast, early Fontan complications in our study did not occur in absence of pathologic lymphatic perfusion pattern.…”

Section: Discussionmentioning

confidence: 55%

Association of Lymphatic Abnormalities with Early Complications after Fontan Operation

Dittrich

Weise

Cesnjevar

et al. 2020

Thorac Cardiovasc Surg

View full text Add to dashboard Cite

Background Increased central venous pressure is inherent in Fontan circulation but not strongly related to Fontan complication. Abnormalities of the lymphatic circulation may play a crucial role in early Fontan complications. Methods This was a retrospective, single-center study of patients undergoing Fontan operation from 2008 to 2015. The primary outcome was significant early Fontan complication defined as secondary in-hospital treatment due to peripheral edema, ascites, pleural effusions, protein-losing enteropathy, or plastic bronchitis. All patients received T2-weighted magnetic resonance images to assess abdominal and thoracic lymphatic perfusion pattern 6 months after Fontan completion with respect to localization, distribution, and extension of lymphatic perfusion pattern (type 1–4) and with application of an area score (0–12 points). Results Nine out of 42 patients developed early Fontan complication. Patients with complication had longer chest tube drainage (mean 28 [interquartile range [IQR]: 13–60] vs. 13 [IQR: 2–22] days, p = 0.01) and more often obstructions in the Fontan circuit 6 months after surgery (56 vs. 15%, p = 0.02). Twelve patients showed little or no abnormalities of lymphatic perfusion (lymphatic perfusion pattern type 1). Most frequently magnetic resonance imaging showed lymphatic congestion in the supraclavicular region (24/42 patients). Paramesenteric lymphatic congestion was observed in eight patients. Patients with early Fontan complications presented with higher lymphatic area score (6 [min–max: 2–10] vs. 2 [min–max: 0–8]), p = 0.001) and greater distribution and extension of thoracic lymphatic congestion (type 3–4: n = 5/9 vs. n = 1/33, p = 0.001). Conclusion Early Fontan complication is related to hemodynamic factors such as circuit obstruction and to the occurrence and extent of lymphatic congestion.

show abstract

Section: Discussionmentioning

confidence: 55%

Association of Lymphatic Abnormalities with Early Complications after Fontan Operation

Dittrich

Weise

Cesnjevar

et al. 2020

Thorac Cardiovasc Surg

View full text Add to dashboard Cite

show abstract

“…A decrease in lymphocyte and CD4þ count in Fontan patients with PLE has already been described. 5,17,18 We demonstrate that the decline of T cells in Fontan patients with PLE is due to both, a reduction in CD4þ T helper and CD8þ cytotoxic T cells. However, the depletion of T cells is mainly dependent on the reduction in CD4þ T helper cells.…”

Section: Discussionmentioning

confidence: 71%

“…PLE was diagnosed as a clinical syndrome, defined by at least three of the following six symptoms: (1) mild-to-profound diarrhea, (2) elevated fecal α-1 AT >400 µg/g, (3) serum total protein <50 g/L, (4) hypoalbuminemia <30 g/L, and (5) hyponatremia <135 mmol/L and (6) clinical third space volume retention (e.g., ascites, edema and/or pleural effusions). 11…”

Section: Patientsmentioning

confidence: 99%

“…Besides abnormal lymphatic perfusion pattern, which thanks to the latest imaging technique have received special attention in the scientific debate, immune abnormalities including lymphopenia and T cell deficiency have long been known to occur in Fontan patients with PLE. 5 The interaction of the lymphatic circulation and cellular lymphatic dysfunction in the progress of PLE needs further understanding.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Lymphocyte Immune Response and T Cell Differentiation in Fontan Patients with protein-losing enteropathy

Moosmann

Toka

Lukassen

et al. 2021

Thorac Cardiovasc Surg

View full text Add to dashboard Cite

Background Protein-losing enteropathy (PLE) is a severe complication of the Fontan circulation. There is increasing discussion about whether lymphatic dysregulation is involved as pathomechanism of PLE. This investigation focuses on the interplay between alteration of lymphatic cells and immunologic pathway alterations. Methods Micro-ribonucleic acid (miRNA) expression profiling was performed in 49 patients (n = 10 Fontan patients with PLE, n = 30 Fontan patients without PLE, and n = 9 patients with dextro-transposition of the great arteries (dTGA). miRNA pathway analysis was performed to identify significantly enriched pathways. To determine lymphocyte populations and subtypes multiparameter flow cytometry was used. Results miRNAs pathway analysis of Fontan patients with PLE revealed 20 significantly changed networks of which four of the ten largest were associated with immunologic processes. This finding is supported by significant T cell deficiency with decreased CD4+ count (p = 0.0002), altered CD4 +/CD8+ ratio, and significantly modified CD4+ (p < 0.0001) and CD8+ (p = 0.0002) T cell differentiation toward effector and terminal differentiated T cells in Fontan patients with PLE. Analyses of CD4+ T cell subsets demonstrated significantly increased frequencies of CD4+ CD25+ CD127– regulatory T cells (Treg) in Fontan patients with PLE (p = 0.0011). Conclusion PLE in Fontan patients is associated with severe lymphopenia, T cell deficiency, significant alterations of T cell differentiation, and increased Treg frequency reflecting an immune status of chronic inflammation and shortened protection against pathogens and autoimmunity. These cellular alterations seemed to be dysregulated by several miRNA controlled immunological pathways.

show abstract

“…The higher abdominal venous pressure that is inevitable with a Fontan circulation can lead to congestive hepatopathy, gastrointestinal derangements, and renal dysfunction. In this issue of the journal, Kovacikova et al 1 report abnormalities in the immune system that cannot be readily reconciled with known circulatory derangements in single ventricle physiology.…”

mentioning

confidence: 99%

Immune System in Single Ventricle Patients—A Complex Nexus

Kumar

2017

World J Pediatr Congenit Heart Surg

View full text Add to dashboard Cite

Immune Abnormalities in Patients With Single Ventricle Circulation Precede the Fontan Procedure

Cited by 14 publications

References 24 publications

Association of Lymphatic Abnormalities with Early Complications after Fontan Operation

Association of Lymphatic Abnormalities with Early Complications after Fontan Operation

Lymphocyte Immune Response and T Cell Differentiation in Fontan Patients with protein-losing enteropathy

Immune System in Single Ventricle Patients—A Complex Nexus

Contact Info

Product

Resources

About