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 language | English (US) |
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Pages (from-to) | 1166-1175 |
Number of pages | 10 |
Journal | Journal of Thoracic and Cardiovascular Surgery |
Volume | 160 |
Issue number | 5 |
DOIs | |
State | Published - 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