Elastin and collagen in the aortic wall: Changes in the Marfan syndrome and annuloaortic ectasia

Halme, Tapio; Savunen, Timo; Aho, Heikki; Vihersaari, Timo; Penttinen, Risto

doi:10.1016/0014-4800(85)90050-4

Cited by 51 publications

(30 citation statements)

References 23 publications

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“…As shown in the presented results, degradation of the elastin fibers was observed in the MFS and TAA tissue, also consistent with previously published findings (32)(33)(34). In addition to elastin degradation, higher interfibrillar spaces, and decreased elastin fiber concentration have been documented (32)(33)(34), supporting our results.…”

Section: Structuresupporting

confidence: 93%

Biomechanical properties of the thoracic aorta in Marfan patients

Sulejmani¹,

Pokutta-Paskaleva²,

Ziganshin³

et al. 2017

Ann. Cardiothorac. Surg.

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Background: Marfan syndrome (MFS), a genetic disorder of the connective tissue, has been strongly linked to dilation of the thoracic aorta, among other cardiovascular complications. As a result, MFS patients frequently suffer from aortic dissection and rupture, contributing to the high rate of mortality and morbidity among MFS patients. Despite the significant effort devoted to the investigation of mechanical and structural properties of aneurysmal tissue, studies on Marfan aneurysmal biomechanics are scarce. Ex vivo mechanical characterization of MFS aneurysmal tissue can provide a better insight into tissue strength outside the physiologic loading range and serve as a basis for improved risk assessment and failure prediction. Methods:The mechanical and microstructural properties of MFS aneurysmal thoracic aorta (MFS, n=15, 39.5±3.91 years), non-MFS aneurysmal thoracic aorta (TAA, n=8, 52.8±4.9 years), healthy human thoracic aorta (HH, n=8, 75.4±6.1 years), and porcine thoracic aorta (n=10) are investigated. Planar biaxial tensile testing and uniaxial failure testing were utilized to characterize the mechanical and failure properties of the tissue, respectively. Verhoeff-Van Gieson (VVG) and PicroSirius Red stains were utilized to visualize the elastin and collagen fiber architecture, respectively.Results: MFS tissue was found to have age-dependent but diameter-independent mechanical, structural, and morphological properties, also showing extensive elastin fiber degradation. Non-MFS thoracic aneurysmal aorta was thicker and stiffer than age-matched MFS tissue. Moreover, non-MFS thoracic aneurysmal mechanics resembled closely the mechanics of older healthy human tissue. Younger MFS tissue (<40 years) exhibited similar mechanical and structural properties to aged porcine tissue.Conclusions: Both age and aneurysmal presence were found to be factors associated with increased stiffness in aortic tissue, and aortic diameter was not a significant determinant of mechanical property deterioration. Additionally, the presence of MFS was found to induce stiffening of the thoracic aorta, although not to the extent of the non-MFS aneurysm.

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Section: Structuresupporting

confidence: 93%

Biomechanical properties of the thoracic aorta in Marfan patients

Sulejmani¹,

Pokutta-Paskaleva²,

Ziganshin³

et al. 2017

Ann. Cardiothorac. Surg.

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“…The collagen content in aortas of MFS, fusiform aortic aneurysm and dissecting aortic aneurysm patients was similar to overall: fragmentation, accellularity [52] AAE þ MFS overall: varied drastically (total lack of to normal) [52] rsif.royalsocietypublishing.org J R Soc Interface 10: 20121004 rsif.royalsocietypublishing.org J R Soc Interface 10: 20121004 overall: / overall: microfibrillar, fragmented layer-specific: virtually absent and completely disrupted in media [68,72 -76] secondary bleb location-specific: progressively along the circumference of the bleb layer-specific: multiple layers of fragmented elastin lamellae in media w/o bleb, no identifiable elastin lamellae in media of bleb location-specific: scattered at bleb apex [77] aneurysm: AAA overall:…”

Section: Effects Of Diseasementioning

confidence: 62%

“…The latter is consistent with the results of a recent study on hypertensive ATAs using multiphoton microscopy [63], whereby it was reported that elastin content was increased in the inner wall layers, and slightly decreased in the middle and outer wall layers, compared with normotensive ATA. The elastin concentration was studied in the ATA wall from patients with annuloaortic ectasia (AAE), some of which also possessed MFS [52]. In some of the ATAs, the concentration of elastin was less than control, but in others there was less marked difference.…”

Section: Effects Of Diseasementioning

confidence: 99%

Elastin and collagen fibre microstructure of the human aorta in ageing and disease: a review

Tsamis

Krawiec

Vorp

2013

J. R. Soc. Interface.

405

300

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Aortic disease is a significant cause of death in developed countries. The most common forms of aortic disease are aneurysm, dissection, atherosclerotic occlusion and ageing-induced stiffening. The microstructure of the aortic tissue has been studied with great interest, because alteration of the quantity and/or architecture of the connective fibres (elastin and collagen) within the aortic wall, which directly imparts elasticity and strength, can lead to the mechanical and functional changes associated with these conditions. This review article summarizes the state of the art with respect to characterization of connective fibre microstructure in the wall of the human aorta in ageing and disease, with emphasis on the ascending thoracic aorta and abdominal aorta where the most common forms of aortic disease tend to occur.

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“…There are also histologic and biochemical data that suggest that the medial abnormalities of the aortic wall and elastin abnormalities of the aortic wall are no different between those with an aortic root aneurysm without phenotypic characteristics of a connective-tissue disorder compared with those with a connective tissue disorder. 2,3 De Oliveira et al 24 reported on 61 Marfan syndrome patients undergoing a valve-preserving reconstruction compared with 44 patient having a composite reconstruction. Freedom from reoperation at 10 years was 100% in the valve-preserved group.…”

Section: Discussionmentioning

confidence: 99%

“…Cystic medial degeneration is the pathologic feature for either etiology. 2,3 Therefore, for this analysis, we chose to evaluate patients with the anatomic abnormality of an aortic root aneurysm.…”

mentioning

confidence: 99%

Surgery for Aneurysms of the Aortic Root

et al. 2004

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Background-This study evaluated long-term results of aortic root replacement and valve-preserving aortic root reconstruction for patients with aneurysms involving the aortic root. Methods and Results-Two-hundred three patients aged 53Ϯ16 years (meanϮSD; 153 male, 50 female) underwent elective or urgent aortic root surgery from 1971 to 2000 for an aortic root aneurysm: 149 patients underwent a composite valve conduit reconstruction, and 54 patients underwent valve-preserving aortic root reconstruction. Fifty patients had Marfan syndrome. In-hospital and 30-day mortality was 4.0% (8/203) overall: for a composite valve conduit procedure, the corresponding value was 4.0% (6/149) and for valve-preserving procedure, 3.7% (2/54) (PϭNS). Morbidity included 3 strokes (1%), 10 perioperative myocardial infarctions (5%), and 8 reoperations for bleeding (4%). Actuarial survival at 5, 10, 15, and 20 years was 93% (95% confidence interval [CI] ϭ 88% to 97%), 79% (95% CI ϭ 71% to 87%), 67% (95% CI ϭ 57% to 79%), and 52% (95% CI ϭ 36% to 69%), respectively. Freedom from reoperation was 72% (95% CI ϭ 54% to 86%) at 20 years. Complications with anticoagulation occurred in 29 patients; with valve thrombosis, in 2; and with hemorrhage, in 27 (4 life threatening and 23 minor). Freedom from thromboembolism was 91% (95% CI ϭ 77% to 98%) at 20 years. Freedom from endocarditis was 99% (95% CI ϭ 92% to 100%) at 20 years. Multivariate analysis revealed preoperative mitral valve regurgitation (ϩ3 to 4) and older age to be significant predictors of late death (PՅ0.005), and Marfan syndrome, initial valve-preserving aortic root reconstruction, and need for a concomitant procedure at initial operation to be significant predictors of the need for reoperation (PՅ0.01). Conclusions-Aortic root replacement for aortic root aneurysms can be done with low morbidity and mortality. Composite valve conduit reconstruction resulted in a durable result. There were few serious complications related to the need for long-term anticoagulation or a prosthetic valve. Reoperation was most commonly required because of failure of the aortic valve when a valve-preserving aortic root reconstruction was performed or for other cardiac or aortic disease elsewhere.

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Elastin and collagen in the aortic wall: Changes in the Marfan syndrome and annuloaortic ectasia

Cited by 51 publications

References 23 publications

Biomechanical properties of the thoracic aorta in Marfan patients

Biomechanical properties of the thoracic aorta in Marfan patients

Elastin and collagen fibre microstructure of the human aorta in ageing and disease: a review

Surgery for Aneurysms of the Aortic Root

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