Pseudoaneurysm formation after valve sparing root replacement in children with Loeys-Dietz syndrome

Liu, Rui H.; Fraser, Charles D.; Zhou, Xun; Cameron, Duke E.; Vricella, Luca A.; Hibino, Narutoshi

doi:10.1111/jocs.13709

Cited by 22 publications

(25 citation statements)

References 18 publications

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“…A retrospective study by Liu et al found that 4 of 31 patients that developed postoperative pseudoaneurysms requiring reoperation. They concluded that pseudoaneurysms following aortic root replacement can occur in patients with severe subtypes of LDS as all of their patients had a severe subtype with a TGFBR2 mutation [10]. This was also the case with our patient.…”

Section: Discussionsupporting

confidence: 75%

Loeys-Dietz Syndrome Complicated by Right Coronary Artery Pseudoaneurysm

Jawaid

Aqtash

Mansoor

et al. 2018

Case Reports in Cardiology

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Loeys-Dietz syndrome is a rare autosomal dominant connective tissue disorder notable for rapidly progressive vascular aneurysmal disease and craniofacial defects. Patients are at an increased risk for aneurysm rupture and dissection at younger ages compared to other aneurysmal syndromes. Early surgical intervention is important for prevention of ruptures and/or dissection. The coronary arterial tree is mostly involved as a result of postoperative complications of an aortic root repair. This fact has been sparsely reported. We report a unique case of LDS2 presenting with chest pain that was later diagnosed as a pseudoaneurysm as a result of a right coronary artery graft dehiscence.

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Section: Discussionsupporting

confidence: 75%

Loeys-Dietz Syndrome Complicated by Right Coronary Artery Pseudoaneurysm

Jawaid

Aqtash

Mansoor

et al. 2018

Case Reports in Cardiology

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“…Risks associated with the prostheses have led to the development of valve-sparing aortic root replacement (VSRR). VSRR eliminates the need for long-term anticoagulation therapy and subsequent surgeries to replace worn out prosthetic valves [83]. Recent studies on short-and mid-term outcomes of VSRR have shown superiority over traditional valve replacements in terms of survival and need for reoperation [84].…”

Section: Loeys-dietz Syndromementioning

confidence: 99%

“…Unfortunately, there is significantly increased likelihood of pseudoaneurysms in young children following VSRR; thus, further long-term studies are needed to define the optimal surgical management strategy. In addition, ongoing surveillance imaging with CTA or MRA is warranted post operatively as well [83].…”

Section: Loeys-dietz Syndromementioning

confidence: 99%

Disorders of the Aorta and Aortic Valve in Connective Tissue Diseases

et al. 2020

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Purpose of Review The incidence of aortic valve disease in inherited connective tissue disorders is well documented; however, recent studies have only begun to unravel the pathology behind this association. In this review, we aim to describe the etiology, clinical manifestations, management, and prognosis of aortic and aortic valvular disorders that co-exist in a variety of connective tissue diseases. An extensive literature review was performed in PubMed. Articles from 2008 to 2018 were included for review. Predetermined search terms used in PubMed include "aortic manifestation of connective tissue diseases" and "aortic valve disorders in rheumatologic disease." Recent Findings Manifestations of aortic valve disease in the context of connective tissue disorders include valvular stenosis, regurgitation, and/or thoracic aortic aneurysms. Both inherited and inflammatory connective tissue disorders contribute to aortic valve damage with increased susceptibility associated with specific gene variants. Summary Anti-inflammatory and immunosuppressive therapies have demonstrated beneficial results in Marfan's syndrome, Behcet disease, rheumatoid arthritis, ankylosing spondylitis, and systemic sclerosis, often leading to remission. Yet, such therapy is less effective in other disorders compared to alternative treatments such as surgical intervention. Additionally, regular echocardiographic studies should be recommended to those suffering from these disorders, especially those at higher risk for cardiovascular involvement. Given the rates of relapse with immunosuppressants, even following aortic valve replacement, further studies are needed to determine if certain dosing and/or combinations of immunosuppressants could be given to those diagnosed with connective tissue diseases to prevent progression of aortic valve involvement.

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“…Aortic pseudoaneurysms may occur following the replacement of the ascending aorta and arch in patients with connective tissue, congenital, or atherosclerotic disease . Patients with Takayasu's arteritis may also develop pseudoaneurysms due to calcification of aortic tissue .…”

mentioning

confidence: 99%

“…

Aortic pseudoaneurysms may occur following the replacement of the ascending aorta and arch in patients with connective tissue, congenital, or atherosclerotic disease. [1][2][3] Patients with Takayasu's arteritis may also develop pseudoaneurysms due to calcification of aortic tissue. 4 We present images of a patient with Takayasu's arteritis who developed a giant pseudoaneurysm compressing the right coronary artery (RCA) following aortic arch replacement.

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mentioning

confidence: 99%