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

https://doi.org/10.1016/j.jtcvs.2019.07.130Get rights and content
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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.

Graphical abstract

Findings of the study: The need for subsequent arch interventions after acute aortic dissection (AAD) does not seem to be greater in patients with Loeys–Dietz syndrome (LDS) compared with Marfan syndrome (MFS). In contrast, patients with LDS undergoing elective root repair have an increased risk for subsequent arch interventions, so consideration should be given to complete removal of the distal ascending aorta.

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Key Words

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

Abbreviations and Acronyms

AAD
acute aortic dissection
CI
confidence interval
HAR
hemiarch replacement
JHH
Johns Hopkins Hospital
LDS
Loeys–Dietz syndrome
MFS
Marfan syndrome
SHR
subhazard ratio
TAR
total aortic arch replacement

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Read at the 2016 American Association for Thoracic Surgery Aortic Symposium, New York, New York, and recipient of the Randall Griepp Award.