Characterization of the inflammatory cells in ascending thoracic aortic aneurysms in patients with Marfan syndrome, familial thoracic aortic aneurysms, and sporadic aneurysms

J Thorac Cardiovasc Surg. 2008 Oct;136(4):922-9, 929.e1. doi: 10.1016/j.jtcvs.2007.12.063. Epub 2008 Jun 12.


Objective: This study sought to characterize the inflammatory infiltrate in ascending thoracic aortic aneurysm in patients with Marfan syndrome, familial thoracic aortic aneurysm, or nonfamilial thoracic aortic aneurysm.

Background: Thoracic aortic aneurysms are associated with a pathologic lesion termed "medial degeneration," which is described as a noninflammatory lesion. Thoracic aortic aneurysms are a complication of Marfan syndrome and can be inherited in an autosomal dominant manner of familial thoracic aortic aneurysm.

Methods: Full aortic segments were collected from patients undergoing elective repair with Marfan syndrome (n = 5), familial thoracic aortic aneurysm (n = 6), and thoracic aortic aneurysms (n = 9), along with control aortas (n = 5). Immunohistochemistry staining was performed using antibodies directed against markers of lymphocytes and macrophages. Real-time polymerase chain reaction analysis was performed to quantify the expression level of the T-cell receptor beta-chain variable region gene.

Results: Immunohistochemistry of thoracic aortic aneurysm aortas demonstrated that the media and adventitia from Marfan syndrome, familial thoracic aortic aneurysm, and sporadic cases had increased numbers of T lymphocytes and macrophages when compared with control aortas. The number of T cells and macrophages in the aortic media of the aneurysm correlated inversely with the patient's age at the time of prophylactic surgical repair of the aorta. T-cell receptor profiling indicated a similar clonal nature of the T cells in the aortic wall in a majority of aneurysms, whether the patient had Marfan syndrome, familial thoracic aortic aneurysm, or sporadic disease.

Conclusion: These results indicate that the infiltration of inflammatory cells contributes to the pathogenesis of thoracic aortic aneurysms. Superantigen-driven stimulation of T lymphocytes in the aortic tissues of patients with thoracic aortic aneurysms may contribute to the initial immune response.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antigens, CD / immunology
  • Antigens, CD / metabolism
  • Antigens, Differentiation, T-Lymphocyte / genetics
  • Antigens, Differentiation, T-Lymphocyte / metabolism
  • Aortic Aneurysm, Abdominal / immunology
  • Aortic Aneurysm, Abdominal / pathology*
  • Aortic Aneurysm, Abdominal / surgery
  • Aortic Aneurysm, Thoracic / immunology
  • Aortic Aneurysm, Thoracic / pathology*
  • Aortic Aneurysm, Thoracic / surgery
  • Biomarkers / analysis
  • Biopsy, Needle
  • Cardiac Surgical Procedures / methods
  • Case-Control Studies
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Marfan Syndrome / genetics
  • Marfan Syndrome / pathology*
  • Marfan Syndrome / surgery
  • Middle Aged
  • Multivariate Analysis
  • RNA / analysis
  • Receptors, Antigen, T-Cell / immunology*
  • Receptors, Antigen, T-Cell / metabolism
  • Reference Values
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sensitivity and Specificity
  • Statistics, Nonparametric
  • Tissue Culture Techniques
  • Transforming Growth Factor beta2 / analysis
  • Transforming Growth Factor beta2 / immunology*
  • Vascular Cell Adhesion Molecule-1 / immunology
  • Vascular Cell Adhesion Molecule-1 / metabolism


  • Antigens, CD
  • Antigens, Differentiation, T-Lymphocyte
  • Biomarkers
  • Receptors, Antigen, T-Cell
  • Transforming Growth Factor beta2
  • Vascular Cell Adhesion Molecule-1
  • RNA