External Aortic Root Support to Prevent Aortic Dilatation in Patients With Marfan Syndrome

İzgi, Cemil; Alpendurada, Francisco; Nyktari, Eva; Boutsikou, Maria; Pepper, John; Treasure, Tom; Mohiaddin, Raad H.

doi:10.1016/j.jacc.2018.06.053

Cited by 38 publications

(32 citation statements)

References 26 publications

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“…Reinforcing the pulmonary autograft allows a further increase in diameter of the autograft until two months. In accordance with Izgi et al, adding this type of macroporous mesh around the dilated aortic root of Marfan patients prevents further root dilatation 24 . It also decreases the change in diameter occurring throughout the cardiac cycle.…”

Section: Effect Of Systemic Conditions On Autograft Dilatationsupporting

confidence: 66%

Mechano-biological adaptation of the pulmonary artery exposed to systemic conditions

Vanderveken

Vastmans

Claus

et al. 2020

Sci Rep

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Cardiac surgeries may expose pulmonary arterial tissue to systemic conditions, potentially resulting in failure of that tissue. our goal was to quantitatively assess pulmonary artery adaptation due to changes in mechanical environment. In 17 sheep, we placed a pulmonary autograft in aortic position, with or without macroporous mesh reinforcement. It was exposed to systemic conditions for 6 months. All sheep underwent 3 ECG-gated MRI's. Explanted tissue was subjected to mechanical and histological analysis. Results showed progressive dilatation of the unreinforced autograft, while reinforced autografts stabilized after two months. Some unreinforced pulmonary autograft samples displayed more aorta-like mechanical behavior with increased collagen deposition. the mechanical behavior of reinforced autografts was dominated by the mesh. the decrease in media thickness and loss of vascular smooth muscle cells was more pronounced in reinforced than in unreinforced autografts. In conclusion, altering the mechanical environment of a pulmonary artery causes changes in its mechano-biological properties.

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Section: Effect Of Systemic Conditions On Autograft Dilatationsupporting

confidence: 66%

Mechano-biological adaptation of the pulmonary artery exposed to systemic conditions

Vanderveken

Vastmans

Claus

et al. 2020

Sci Rep

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“…Major developments have been undertaken in utilizing patient specific medical devices fabricated through 3D printing for different applications in the medical sector ( Limmahakhun et al, 2017 ; Mohseni et al, 2019 ). Recently an exciting technology called personalized external aortic root support (PEARS) has emerged as a powerful pre-emptive operation to treat patients suffering from aortic root aneurysm ( Treasure et al, 2014 , 2016 ; Izgi et al, 2018 ). This technique takes advantage from the advancements in medical imaging and 3D printing to fabricate a personalized model of the patient’s aorta, which is then used to fabricated a mesh sleeve that is purposed to restrict aortic root expansion.…”

Section: Resultsmentioning

confidence: 99%

Melt Electrowriting of Complex 3D Anatomically Relevant Scaffolds

Saidy

Shabab

Bas

et al. 2020

Front. Bioeng. Biotechnol.

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“…As an alternative procedure to the existing ascending aorta surgery practice, there is increasing evidence that PEARS is effective in stabilizing the aortic root and preventing its dilatation, secondary aortic regurgitation and aortic dissection in patients with connective tissue disorder [ 8 ]. However, there is further evidence that it is also applicable to aortic root aneurysms of other etiologies.…”

Section: Discussionmentioning

confidence: 99%

A contemporary approach to a young female patient with Loeys-Dietz syndrome and an uncomplicated type B aortic dissection: a case report

Prodanov

Línková

Robert

et al. 2020

J Cardiothorac Surg

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Background Aortic dissection is a relatively uncommon, but often catastrophic disease that requires early and accurate diagnosis. It often presents in patients with congenital connective tissue disorders. The current aortic surgical techniques are related with serious early and late complications. This case report emphasizes the importance of early diagnosis of aortic root dilatation and the risk of dissection, especially in patients with congenital connective tissue disorders. We present an alternative, contemporary and multidisciplinary approach based on the present state of knowledge. Case presentation We present a rare case of a young female patient with Loeys-Dietz syndrome who was admitted with an uncomplicated aortic dissection (Stanford type B / DeBakey type III) and a dilated aortic root. After a period of close surveillance and extensive vascular imaging, thoracic endovascular aortic repair was deemed to be technically not possible. Medical treatment was optimized and our patient successfully underwent a personalised external aortic root support procedure (PEARS) as a contemporary alternative to existing aortic root surgical techniques. Conclusions This case highlights the importance of interdisciplinary approach, close follow-up and multimodality imaging. The decision to intervene in a chronic type B aortic dissection is still challenging and should be made in experienced centers by an interdisciplinary team. However, if an acute complication occurs, thoracic endovascular aortic repair TEVAR is the method of choice. In all cases optimal medical treatment is important. There is increasing evidence that personalized external aortic root support procedure PEARS is effective in stabilizing the aortic root and preventing its dilatation and dissection not only in patients with Marfan syndrome, but also in other cases of aortic root dilation of other etiologies. Moreover, many publications have reported the additional benefit of reduction or even eradication of aortic regurgitation by improving coaptation of the aortic valve leaflets in dilated aortas.

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External Aortic Root Support to Prevent Aortic Dilatation in Patients With Marfan Syndrome

Abstract: PEARS is effective in stabilizing the aortic root and preventing its dilatation. It is a viable alternative for prevention of aortic root dissection in Marfan patients.

Cited by 38 publications

References 26 publications

Mechano-biological adaptation of the pulmonary artery exposed to systemic conditions

Mechano-biological adaptation of the pulmonary artery exposed to systemic conditions

Melt Electrowriting of Complex 3D Anatomically Relevant Scaffolds

A contemporary approach to a young female patient with Loeys-Dietz syndrome and an uncomplicated type B aortic dissection: a case report

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