• 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, and Duke E Cameron.
• Division of Cardiac Surgery, Johns Hopkins Medical Institutions, Baltimore, Md; Department of Cardiovascular Surgery, University Hospital Bern, Bern, Switzerland.
• J. Thorac. Cardiovasc. Surg. 2020 Nov 1; 160 (5): 1166-1175.

ObjectivesWe 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).MethodsWe reviewed hospital and follow-up records of 79 patients with LDS and compared them with 256 patients with MFS who served as reference controls.ResultsIn 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).ConclusionsIn 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.Copyright © 2019. Published by Elsevier Inc.

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