Management of the aortic arch in patients with Loeys-Dietz syndrome

J Thorac Cardiovasc Surg. 2020 Nov;160(5):1166-1175. doi: 10.1016/j.jtcvs.2019.07.130. Epub 2019 Sep 9.


Objectives: We sought to develop strategies for management of the aortic arch in patients with Loeys-Dietz syndrome (LDS) through a review of our clinical experience with these patients and a comparison with our experience in patients with Marfan syndrome (MFS).

Methods: We reviewed hospital and follow-up records of 79 patients with LDS and compared them with 256 patients with MFS who served as reference controls.

Results: In the LDS group, 16% of patients presented initially with acute aortic dissection (AAD) (67% type A, 33% type B) or developed AAD during follow-up, compared with 10% of patients with MFS (95% type A, 5% type B). There was no difference between patients with LDS or MFS in need for subsequent arch interventions after aortic root surgery (46% vs 50%, P = 1.0). Among the patients who never had AAD, the need for arch repair at initial root surgery was greater in patients with LDS (5% vs 0.4%, P = .04), as was the need for any subsequent aortic surgery (12% vs 1.3%, P = .0004). Late mortality in patients with LDS after arch repair was greater than in those patients who had no arch intervention (33% vs 6%, P = .007).

Conclusions: In the absence of dissection, patients with LDS have a greater rate of arch intervention after root surgery than patients with MFS. After a dissection, arch reintervention rates are similar in the 2 groups. Arch intervention portends greater late mortality in LDS.

Keywords: Loeys–Dietz syndrome; Marfan syndrome; acute aortic dissection; aortic arch; aortic surgery; connective tissue disorders.

MeSH terms

  • Adolescent
  • Adult
  • Aorta, Thoracic / surgery*
  • Aortic Aneurysm / surgery*
  • Child
  • Child, Preschool
  • Humans
  • Loeys-Dietz Syndrome* / epidemiology
  • Loeys-Dietz Syndrome* / mortality
  • Loeys-Dietz Syndrome* / surgery
  • Marfan Syndrome / epidemiology
  • Marfan Syndrome / mortality
  • Marfan Syndrome / surgery
  • Middle Aged
  • Retrospective Studies
  • Young Adult