Key result
Surgical management of the aortic arch in inherited aortopathies generally involves a more aggressive approach for Loeys-Dietz and Ehlers-Danlos syndromes, while prophylactic total arch replacement is not routinely indicated for Marfan syndrome.
Why the study?
There are generally no clear guideline indications for when to intervene surgically on the aortic arch in patients with inherited aortopathies.
This review highlights that while prophylactic aortic arch replacement is generally not indicated during proximal aortic surgery for Marfan syndrome, a more aggressive surgical approach is warranted for patients with Loeys-Dietz syndrome.
Surgical management of the aortic root and ascending aorta has seen an evolution over the past 50 years. Despite the widely available guidelines for management of the aortic root and ascending aorta, including in those with connective tissue disease and inherited aortopathies, there are generally no clear guideline indications for when to intervene on the aortic arch in these patients. This perhaps may be related to the fact that whilst the majority of acquired aortopathies, and also in non-syndromic aortopathies such as in bicuspid aortic valve, size criteria are utilized to decide on when to intervene, the use of size criteria may not be appropriate in those with syndromic inherited aortopathies. The aim of the present mini review is to provide a general overview and guidance for the surgical management of patients with inherited aortopathies.
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Lucchese et al. (2022) conducted a review in Inherited aortopathy (Marfan syndrome, Loeys-Dietz syndrome, Ehlers-Danlos syndrome, Bicuspid aortic valve). Surgical management of the aortic arch was evaluated. Surgical management of the aortic arch in inherited aortopathies generally involves a more aggressive approach for Loeys-Dietz and Ehlers-Danlos syndromes, while prophylactic total arch replacement is not routinely indicated for Marfan syndrome.
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