Immunohistochemical Abnormalities of Fibrillin in Cardiovascular Tissues in Marfan’s Syndrome

Fleischer, Kirk J.; Nousari, Hossein C.; Anhalt, Grant J.; Stone, Christopher D.; Laschinger, John C.

doi:10.1016/s0003-4975(97)00061-1

Cited by 91 publications

(51 citation statements)

References 22 publications

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“…It has been shown that abnormal fibrillin metabolism affects valve tissue in patients with bona fide connective-tissue disorders. Fleischer et al 13 showed that the aortic valve cusps and mitral valve leaflets in Marfan syndrome patients were equally effected by fibrillin-1 fragmentation compared with the aortic wall. These changes were most severe in patients Ͼ20 years of age.…”

Section: Discussionmentioning

confidence: 99%

“…[5][6][7][8] In an attempt to avoid anticoagulation in this relatively young patient population, Sarsam and Yacoub 9 and David and Feindel 10 have described valve-preserving aortic root reconstruction techniques. Despite the fact that the valve cusps often appear functionally normal, there is evidence that they have abnormalities similar to those in the aortic wall tissue, [11][12][13] potentially limiting their durability. One of the objectives of this study was to evaluate whether or not the benefit of avoiding anticoagulation is offset by the need for reoperation.…”

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confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Surgery for Aneurysms of the Aortic Root

et al. 2004

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“…These methods obviate the need for anticoagulation and avoid the risk of valverelated thromboembolism. However, a major concern of preserving aortic valve leaflets known to contain defective fibrillin-1 in MFS patients is the risk of early repair failure and need for re-operation (7). The concerns regarding the durability of valve sparing root replacement (VSRR) are being addressed with increased experience in the techniques and longer-term follow up data.…”

Section: Introductionmentioning

confidence: 99%

Systematic review and meta-analysis of surgical outcomes in Marfan patients undergoing aortic root surgery by composite-valve graft or valve sparing root replacement

Flynn

Tian

Wilson-Smith

et al. 2017

Ann. Cardiothorac. Surg.

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Background: A major, life-limiting feature of Marfan syndrome (MFS) is the presence of aneurysmal disease. Cardiovascular intervention has dramatically improved the life expectancy of Marfan patients.Traditionally, the management of aortic root disease has been undertaken with composite-valve graft replacing the aortic valve and proximal aorta; more recently, valve sparing procedures have been developed to avoid the need for anticoagulation. This meta-analysis assesses the important surgical outcomes of the two surgical techniques.Methods: A systematic review and meta-analysis of 23 studies reporting the outcomes of aortic root surgery in Marfan patients with data extracted for outcomes of early and late mortality, thromboembolic events, late bleeding complications and surgical reintervention rates.Results: The outcomes of 2,976 Marfan patients undergoing aortic root surgery were analysed, 1,624 patients were treated with composite valve graft (CVG) and 1,352 patients were treated with valve sparing root replacement (VSRR). When compared against CVG, VSRR was associated with reduced risk of thromboembolism (OR =0.32; 95% CI, 0.16-0.62, P=0.0008), late hemorrhagic complications (OR =0.18; 95% CI, 0.07-0.45; P=0.0003) and endocarditis (OR =0.27; 95% CI, 0.10-0.68; P=0.006). Importantly there was no significant difference in reintervention rates between VSRR and CVG (OR =0.89; 95% CI, 0.35-2.24; P=0.80).Conclusions: There is an increasing body of evidence that VSRR can be reliably performed in Marfan patients, resulting in a durable repair with no increased risk of re-operation compared to CVG, thus avoiding the need for systemic anticoagulation in selected patients.

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“…Sarsam & Yacoub [6] initially, and later, David & Feindel [7] have proposed techniques for aortic root reconstruction with the preservation of native aortic valve. In these patients, the valve leaflets do not show significant primary changes, but they may present secondary changes to the dilation of the aortic ring, in addition to abnormalities similar to those in the related aortic wall, which could limit the longevity of this procedure [8][9][10]. The native valve preservation, the main objective of any operation involving the heart valves, makes the procedure more laborious and time consuming, and does not ensure an early or late adequate result of the procedure (which usually occurs when using a valved conduit).…”

Section: Introductionmentioning

confidence: 99%

Análise do tratamento cirúrgico da raiz da aorta com o tubo valvulado e com a preservação da valva aórtica

Dias¹,

Mejía²,

Fiorelli³

et al. 2010

Rev Bras Cir Cardiovasc

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Análise do tratamento cirúrgico da raiz da aorta com o tubo valvulado e com a preservação da valva aórticaAnalysis of aortic root surgery with composite mechanical aortic valve conduit and valve-sparing reconstruction 7. Faculty Member of Cardiovascular Surgery Discipline at InCor-FMUSP; CEO of InCor-FMUSP Hospital.Work performed at the Heart Institute, School of Medicine, University of Sao Paulo (InCor-FMUSP), Sao Paulo, SP, Brazil. Sixteen percent of patients had Marfan syndrome and 4.3% had bicuspid aortic valve. One hundred and forty-four patients (88%) were followed for a mean period of 41.1 ± 20.8 months.Results: The hospital mortality was 4.9%, 5.6% in operations with valved conduits and 2.6% in the valve sparing procedures (P <0.05). There was no difference neither in survival (95% CI = 86% -96%, P= 0.1) nor in reoperationfree survival (95% CI = 85% -90%, P = 0.29). The survival free of complications such as bleeding, thromboembolism and endocarditis were favorable to the valve sparing operations, respectively (95% CI = 70% -95%, P = 0.001), (95% CI = 82% -95% P = 0.03) and (95% CI = 81% -95%, P = 0.03). Multivariate analysis showed that creatinine greater than 1.4 mg/dl, Cabrol operation and renal dialysis were predictors of mortality, respectively, with occurrence chance of 6 (95% CI = 1.8 -19.5, P = 0.003), 12 (95% CI = 3 -49.7, P = 0.0004) and 16 (95% CI = 3.6 -71.3, P = 0.0002).Conclusions: The aortic root reconstruction has a low early and late mortality, high survival free of complications and low need for reoperation. During the late follow-up, valve sparing aortic root reconstructions presented fewer incidences of bleeding, thromboembolic events and endocarditis.Descriptors: Aorta. Aortic aneurysm, thoracic. Aortic valve insufficiency. Aortic valve. DIAS, RR ET AL -Analysis of aortic root surgery with composite mechanical aortic valve conduit and valve-sparing reconstruction Rev Bras Cir Cardiovasc 2010; 25(4): 491-499

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Immunohistochemical Abnormalities of Fibrillin in Cardiovascular Tissues in Marfan’s Syndrome

Cited by 91 publications

References 22 publications

Surgery for Aneurysms of the Aortic Root

Surgery for Aneurysms of the Aortic Root

Systematic review and meta-analysis of surgical outcomes in Marfan patients undergoing aortic root surgery by composite-valve graft or valve sparing root replacement

Análise do tratamento cirúrgico da raiz da aorta com o tubo valvulado e com a preservação da valva aórtica

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