Marfan Syndrome Is Associated With Recurrent Dissection of the Dissected Aorta

Ptaszek, Leon M.; Kim, Kibeom; Spooner, Amy E.; MacGillivray, Thomas E.; Cambria, Richard P.; Lindsay, Mark E.; Isselbacher, Eric M.

doi:10.1016/j.athoracsur.2014.12.066

Cited by 8 publications

(2 citation statements)

References 22 publications

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“…Recurrent dissections are extremely rare and only typically seen in patients with connective tissue disorders. [2][3][4] A recent study by Isselbacher et al showed that among 3624 patients with initial dissection, just 5% developed another dissection, and of those with recurrent dissections, more than 21% had Marfan syndrome. 4 While this patient does not have the clinical or molecular diagnosis of Marfan syndrome, she was found to have a disease-causing mutation in the ACTA2 gene.…”

Section: Discussionmentioning

confidence: 99%

Recurrent Aortic Dissection in a Patient With an Actin Mutation Treated With a Laser-Fenestrated Aortic Arch Stent-Graft

Cullen

Ailawadi

Angle

et al. 2018

Vasc Endovascular Surg

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A 58-year-old African American female, who had previously undergone a valve-sparing aortic root replacement for a type A dissection, represented 2 months later with a new focal dissection and aneurysm with intramural hematoma just distal to the origin of the left subclavian artery. Since no landing zone distal to the subclavian artery existed, the patient underwent thoracic endovascular aortic repair with laser fenestration-aided stent-graft placement in the left subclavian artery. The patient recovered without complication, and 52 months later, she is doing well with normal renal function and ambulatory status.

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Section: Discussionmentioning

confidence: 99%

Recurrent Aortic Dissection in a Patient With an Actin Mutation Treated With a Laser-Fenestrated Aortic Arch Stent-Graft

Cullen

Ailawadi

Angle

et al. 2018

Vasc Endovascular Surg

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“…Genetical: some syndromes are associated with aortic dissection, such as Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome, and Turner syndrome. Moreover, it is associated with non-syndromic conditions, such as familial TAAD or bicuspid aortic valve (Roberts & Roberts, 1991;Ptaszek et al, 2015;Isselbacher et al, 2016;Chen et al, 2020).…”

Section: Etiological Classificationmentioning

confidence: 99%

Review on Aortic Dissection Diagnostic & Management Approach in Emergency Department

Khan¹,

Alshehab²,

Baglagel³

et al. 2021

World Journal of Environmental Biosciences

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Aortic dissection is a rare occurrent condition involving the body's main artery, the aorta, and carrying with it high morbidity and mortality. It is one of the conditions that fall under the term acute aortic syndromes. Although it is uncommon, it is hard to describe the true number of cases as most cases are based on autopsy reports. Therefore, to encounter this catastrophic condition, early diagnosis and immediate management are vital for the survival of patients. The goal was to review the literature to enhance the awareness and understanding of mesenteric ischemia. We reviewed the literature for aortic dissection; pathophysiology, classification, diagnosis, and options for treatment. Articles were selected from the PubMed database, and the studies selected were subjected to a thorough review. Aortic dissection is an uncommon condition presenting as severe chest or back pain. Although it is uncommon, it remains a potentially fatal condition that may jeopardize a patient's life. It starts with a tear in the aortic intima, that can propagate on both sides leading eventually to end-organ ischemia and death. Early diagnosis and rapid management are the keys to a patient's survival.

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Invited Commentary

Moon

2015

The Annals of Thoracic Surgery

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Marfan Syndrome Is Associated With Recurrent Dissection of the Dissected Aorta

Cited by 8 publications

References 22 publications

Recurrent Aortic Dissection in a Patient With an Actin Mutation Treated With a Laser-Fenestrated Aortic Arch Stent-Graft

Recurrent Aortic Dissection in a Patient With an Actin Mutation Treated With a Laser-Fenestrated Aortic Arch Stent-Graft

Review on Aortic Dissection Diagnostic & Management Approach in Emergency Department

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