Lymphopenia in Patients with Single-ventricle Heart Disease after the Fontan Operation

Abstract

In a cohort of patients with Fontan circulation and no history of protein-losing enteropathy who underwent catheterization, lymphopenia was common and positively associated with low platelet count. Thrombocytopenia has been shown to correlate with the degree of hepatic fibrosis in those with Fontan and, thus, hepatic fibrosis may underlie lymphopenia in these patients.

Lymphopenia in adults after the Fontan operation: prevalence and associations

“…Prior reports suggest even higher frequency of lymphopenia, up to 47%, in patients with a Fontan circulation in a paediatric and adult Fontan population who underwent cardiac catheterisation for clinical haemodynamic assessment. 10 In line with results from prior analyses, we found an association between lymphopenia and lower white cell, lower monocyte, and platelet counts. 10,18 Figure 2.…”

“…10 In line with results from prior analyses, we found an association between lymphopenia and lower white cell, lower monocyte, and platelet counts. 10,18 Figure 2. Lymphopenia is associated with thrombocytopenia and lower BMI.…”

“…[7][8][9] However, recent reports suggest a high prevalence of lymphopenia even in Fontan patients in the absence of a manifest lymphatic leak syndrome. 10 Further, lymphopenia appears to be associated with lower platelet count, suggesting a relationship with splenomegaly and Fontan-associated liver disease commonly seen in Fontan patients secondary to chronically elevated central venous pressure. 11 The objective of this study was to evaluate the prevalence and associations of lymphopenia in a group of adults after the Fontan operation.…”

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

“…This is in contrast with lymphopenia observed by others in significant portion of Fontan patients several years after procedure. [3][4][5][6][7][8] It has been postulated that this is due to chronic systemic venous hypertension leading to increased tissue fluid generation, impediment of lymph drainage, and loss of lymphocyte-rich fluid into gut. 6,12,14 Lymphocyte Subpopulations…”

“…7 Recently, lymphopenia was documented even in patients without PLE several years after Fontan completion. [6][7][8][9] Since surgical thymectomy, insufficient hemodynamics, chylothorax, growth retardation, and inadequate nutrition play a role in an immune dysbalance, [9][10][11] we assume that immune abnormalities are present in early stages of single ventricle palliation. It can be also suggested that identification of patients with the specific immune derangements will allow immune-modulation therapies to optimize multimodal care for patients with single ventricle circulation.…”