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

Florian S. Schoenhoff, Diane E. Alejo, James H. Black, Todd C. Crawford, Harry C. Dietz, Joshua C. Grimm, Jonathan T. Magruder, Nishant D. Patel, Luca A. Vricella, Allen Young, Thierry P. Carrel, Duke E. Cameron

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1166-1175
Number of pages10
JournalJournal of Thoracic and Cardiovascular Surgery
Volume160
Issue number5
DOIs
StatePublished - Nov 2020

Keywords

  • Loeys–Dietz syndrome
  • Marfan syndrome
  • acute aortic dissection
  • aortic arch
  • aortic surgery
  • connective tissue disorders

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

Fingerprint Dive into the research topics of 'Management of the aortic arch in patients with Loeys–Dietz syndrome'. Together they form a unique fingerprint.

Cite this