Results of surgery for aortic root aneurysm in patients with Marfan syndrome

Oliveira, Nilto C. De; David, Tirone E.; Ivanov, Joan; Armstrong, Susan; Eriksson, Maria; Rakowski, Harry; Webb, Gary D.

doi:10.1067/mtc.2003.57

Cited by 210 publications

(149 citation statements)

References 25 publications

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“…There were 2 studies reporting the outcomes of CVG only (11,19) and 8 studies reporting the outcomes of VSRR only (15,17,20,22,23,26,28,31). Thirteen studies reported outcomes for both CVG and VSRR (12)(13)(14)16,18,21,24,25,27,29,30,32,33). The overall study characteristics are detailed in Table 1.…”

Section: Resultsmentioning

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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Section: Resultsmentioning

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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“…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%

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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“…39 Alternative procedures include the Bentall composite graft repair, in which both the aortic root and the aortic valve are replaced, or a valve conserving technique such as re-implantation of the native aortic valve in a Dacron tube (described by David) or remodelling of the aortic root (described by Yacoub). 40,41 The Bentall procedure has a low mortality in experienced hands with long-term survival of around 80% at 5 years and 60% at 10 years, 42 but requires lifelong anti-coagulation post-operatively, whereas valve conserving techniques may avoid the need for anticoagulation. Use of a valve-conserving procedure has been controversial as it is suggested that further deterioration of the aortic valve leaflets will require later valve replacement surgery.…”

Section: Cardiovascular System In Marfan Syndromementioning

confidence: 99%

Marfan syndrome: clinical diagnosis and management

2007

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In association withMarfan syndrome: clinical diagnosis and management Marfan syndrome is a multisystem connective tissue disorder usually associated with mutation in fibrillin, and occasionally with mutation in TGFBR1 or 2. The clinical diagnosis is made using the Ghent nosology, which will unequivocally diagnose or exclude Marfan syndrome in 86% of cases. Use of a care pathway can help implementation of the nosology in the clinic. The penetrance of some features is age dependent, so the nosology must be used with caution in children. Molecular testing may be helpful in this context. The nosology cannot be used in families with isolated aortic dissection, or with related conditions such as Loeys -Dietz syndrome, although it may help identify families for further diagnostic evaluation because they do not fulfill the nosology, despite a history of aneurysm. Prophylactic medical (eg b-blockade) and surgical intervention is important in reducing the cardiovascular complications of Marfan syndrome. Musculoskeletal symptoms are common, although the pathophysiology is less clear -for example, the correlation between dural ectasia and back pain is uncertain. Symptoms in other systems require specialist review such as ophthalmology assessment of refractive errors and ectopia lentis. Pregnancy is a time of increased cardiovascular risk for women with Marfan syndrome, particularly if the aortic root exceeds 4 cm at the start of pregnancy. High-intensity static exercise should be discouraged although low-moderate intensity dynamic exercise may be beneficial. The diagnosis and management of Marfan syndrome requires a multidisciplinary team approach, in view of its multisystem effects and phenotypic variability.

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Results of surgery for aortic root aneurysm in patients with Marfan syndrome

Cited by 210 publications

References 25 publications

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

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

Surgery for Aneurysms of the Aortic Root

Marfan syndrome: clinical diagnosis and management

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