Type A Aortic Dissection in Marfan Syndrome

Rylski, Bartosz; Bavaria, Joseph E.; Beyersdorf, Friedhelm; Branchetti, Emanuela; Desai, Nimesh D.; Milewski, Rita K.; Szeto, Wilson Y.; Vallabhajosyula, Prashanth; Siepe, Matthias; Kari, Fabian A.

doi:10.1161/circulationaha.113.005865

Cited by 92 publications

(24 citation statements)

References 24 publications

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“…The other remaining 54 VSARR (96.4%) were elective aneurysmal aortic root replacements, which could have also contributed favorably to our outcomes. Furthermore, aortic dissection is a known risk factor with regard to reinterventions of the downstream aorta or the aortic root 18–20 . In our cohort operations of the aortic arch and descending aorta – including abdominal aorta - were performed in 6 patients, of whom 4 initially presented with aortic dissections (type A or B), which is also in line with previous findings 19–21 .…”

Section: Discussionmentioning

confidence: 99%

The reimplantation valve-sparing aortic root replacement technique for patients with Marfan syndrome: A single-center experience

Kremer

Farag

Zaradzki

et al. 2019

Sci Rep

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Valve-sparing aortic root replacement (VSARR) through reimplantation technique is widely regarded as optimal surgical approach for Marfan syndrome (MFS) patients. Perioperative and long-term data from all MFS patients undergoing VSARR using David’s technique at our center from 2007–2018 were analyzed. We included 56 patients with a mean age of 32.3 ± 12.3 years. Logistic EuroSCORE was 7.96 ± 5.2. Among others concomitant surgical procedures included aortic arch surgery (8.9%), mitral valve repair (23.2%) and replacement (1.7%). There were no operative deaths, nor in-hospital-mortality. One patient underwent re-exploration for bleeding, dialysis and pacemaker implantation was required in one case each. There was no occurrence of low-output syndrome nor neurological complications. Significant gender differences were not found, except for intraoperative blood transfusion occurring significantly more often in the female gender (p = 0.009). Despite significantly longer procedural times, concomitant surgery did not negatively impact overall outcome. Freedom of reoperation of the aortic root was 100% at 1 year, 97.7% at 8 years. Until last follow-up (61 ± 38 month) all patients survived, with no evidence of endocarditis. We emphasize once more that VSARR using David’s procedure is a safe method for MFS patients with excellent long-term results even if concomitant procedures are performed.

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Section: Discussionmentioning

confidence: 99%

The reimplantation valve-sparing aortic root replacement technique for patients with Marfan syndrome: A single-center experience

Kremer

Farag

Zaradzki

et al. 2019

Sci Rep

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“…Dissecting the brachiocephalic arteries, especially the left subclavian is much more technically demanding. A recent study has found that at the time of initial repair for TAAD in MFS, a more aggressive surgical approach seems to be superior to limited repair (29). However, there is scarce data on the long-term outcomes of such extensive repair for TAAD in such patients (19,22,30).…”

Section: Discussionmentioning

confidence: 99%

Total arch replacement and frozen elephant trunk for type A aortic dissection after Bentall procedure in Marfan syndrome

Chen

Zheng

et al. 2018

J. Thorac. Dis.

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Background: We seek to report the long-term outcomes of the total arch replacement and frozen elephant trunk (TAR + FET) technique for type A aortic dissection (TAAD) following prior Bentall procedure in patients with Marfan syndrome (MFS). Methods: Between 2003 and 2015, we performed TAR + FET for 26 patients with MFS who developed TAAD following a prior Bentall procedure. Mean age at FET 36.9±9.7 years and 24 were males. TAAD was acute in 8 (30.8%, all new dissections from precious root aneurysm) and chronic in 18 (69.2%, 15 residual and 3 new). The interval from Bentall procedure to FET averaged 6.4±5.8 years, which was significantly longer in the acute group (10.3±6.3 vs. 4.6±4.9, P=0.021). The early and long-term outcomes were compared between two groups and risk factors identified for late adverse events.Results: Operative mortality was 11.5% (3/26). Stroke, lower limb ischemia and reexploration for bleeding occurred in 1 patient each (3.8%). Follow-up was complete in 100% (23/23) at mean 5.1±2.3 years (range, 0.9-11.2 years). The maximal diameter (DMax) of distal aorta in the chronic group was significantly greater at the unstented descending aorta [DA, (56.4±15.5 vs. 35.6±12.2 mm, P=0.006)] compared to acute patients. The false lumen was obliterated in 95.7% across the FET and 56.5% in the unstented DA. Distal aortic dilation occurred in 13 patients (11 chronic, 68.8%). Of those 11 patients, 4 underwent an open thoracoabdominal aortic repair and 3 died of distal aortic rupture. Late death occurred in 7 patients at mean 3.9±2.5 years. At 6 years, the incidence was 18% for death, 11% for distal aortic reoperation, and 71% for reoperationfree survival. Survival did not differ between two groups (75.0% vs. 71.3%, P=0.851), while acute patients had significantly higher freedom from late rupture and reoperation at 6 years (100% vs. 61.9%, P=0.046). Bentall procedure in patients with MFS. Early and late survival did not differ with acute and chronic dissections, while freedom from late rupture and reoperation is significantly higher in patients with acute TAAD. Patients with hypertension and aged <35 years are at higher risk for late distal aortic dilation, reoperation and death.

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“…[1–3] Stanford type A AD originates primarily in the ascending aorta just above the aortic valve. [4] In spite of the substantial advances made in diagnostic imaging methods and surgical techniques for treatment, the mortality of type A AD still averages 25%. [5] Further difficulties in prevention and therapeutic management arise from the fact that the responsible molecular and genetic determinants of type A AD remain largely unidentified.…”

Section: Introductionmentioning

confidence: 99%

MMP-2 gene polymorphisms are associated with type A aortic dissection and aortic diameters in patients

et al. 2016

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Matrix metalloproteinases-2 (MMP-2) plays an important role in the pathogenesis of type A aortic dissection (AD). The aim of this study was to evaluate the association of 3 single nucleotide polymorphisms (SNPs) in the MMP-2 gene with type A AD risk and aortic diameters in patients. We performed a case–control study with 172 unrelated type A AD patients and 439 controls. Three SNPs rs11644561, rs11643630, and rs243865 were genotyped through the MassARRAY platform. Allelic associations of SNPs and SNP haplotypes with type A AD and aortic diameters in patients were evaluated. The frequency of the G allele of the rs11643630 polymorphism was significantly lower in type A AD patients than in control subjects (odds ratio 0.705, 95% confidence interval 0.545–0.912, P = 0.008). The association remained significant after adjusting for clinical covariates (P = 0.008). Carriers of the GG genotype of the rs11643630 polymorphism had significantly smaller aortic diameters than those with GT genotype or TT genotype (P = 0.02). Further haplotype analysis identified 1 protective haplotype (GC; P = 0.008) for development of type A AD. Again, a significant correlation was observed between haplotype GC and AD size (P = 0.020). Our results suggest that MMP-2 gene polymorphisms contribute to type A AD susceptibility. In addition, MMP-2 gene SNPs are associated with AD size, which could be used as a target for the development of new drug therapy.

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Type A Aortic Dissection in Marfan Syndrome

Cited by 92 publications

References 24 publications

The reimplantation valve-sparing aortic root replacement technique for patients with Marfan syndrome: A single-center experience

The reimplantation valve-sparing aortic root replacement technique for patients with Marfan syndrome: A single-center experience

Total arch replacement and frozen elephant trunk for type A aortic dissection after Bentall procedure in Marfan syndrome

MMP-2 gene polymorphisms are associated with type A aortic dissection and aortic diameters in patients

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