This is a case of a 4-cm left extrathoracic subclavian artery aneurysm (SCAA) in a 58-year-old man with an aortic root and abdominal aortic aneurysm. The patient had features suggestive of genetic arteriopathy, including vertebral artery tortuosity, pectus excavatum, tall stature, and scoliosis. The SCAA was successfully repaired with an inline prosthetic graft and anastomotic pledgets via a supraclavicular approach. Genetic testing revealed an FBN1 pathogenic variant consistent with Marfan syndrome. Repair is satisfactory 2 years later. Patients with SCAA should include consideration of genetic arteriopathy. Open repair of the extrathoracic SCAA in Marfan syndrome is recommended.
have established that rib resection can be beneficial. We sought to determine long-term, objective functional outcomes using the shortened Disabilities of the Arm, Shoulder, and Hand survey in patients with TOS who underwent rib resection.Methods: Clinical records for patients who underwent rib resection for TOS at a single institution were reviewed. All patients were contacted by telephone, and long-term functional outcome was assessed by the 11item version of the Disabilities of the Arm, Shoulder, and Hand questionnaire. Patients were also asked whether they returned to baseline activity and whether they were subjectively better postoperatively.Results: From 2000 to 2018, there were 261 patients who underwent rib resection surgery; 170 (65.1%) were reached by telephone for long-term follow-up. A total of 188 operations (102 neurogenic TOS, 82 venous TOS, 4 arterial TOS) were performed in these 170 patients. The Table summarizes our findings. The mean follow-up time for the cohort was 5.3 years (range, 1-18 years). Overall, 167 (88.9%) patients returned to baseline activity postoperatively.Conclusions: Rib resection for all forms of TOS is a durable surgical treatment that results in excellent long-term functional outcomes. This is the longest follow-up reported of any TOS series in the literature; our results confirm that the majority of patients have both an objectively measured benefit and subjectively reported return to baseline functional activity during a period as long as 18 years after rib resection.Background: This video describes a patient with clinical findings consistent with a genetically triggered arteriopathy with an enlarging left subclavian artery aneurysm. This video reviews the approach to open surgical repair of the extrathoracic distal subclavian artery.
Background Pregnant patients with Marfan syndrome (MFS) are at an increased risk for adverse aortic outcomes. While beta-blockers are used to slow aortic root dilatation in non-pregnant MFS patients, the benefit of such therapy in pregnant MFS patients remains controversial. The purpose of this study was to investigate the effect of beta-blockers on aortic root dilatation during pregnancy in MFS patients. Methods This was a longitudinal single-center retrospective cohort study of females with MFS who completed a pregnancy between 2004 and 2020. Clinical, fetal, and echocardiographic data were compared in patients on vs off beta-blockers during pregnancy. Results A total of 20 pregnancies completed by 19 patients were evaluated. Beta-blocker therapy was initiated or continued in 13 (65%) of the 20 pregnancies. Pregnancies on beta-blocker therapy experienced less aortic growth compared to those off beta-blockers (0.10cm [IQR: 0.10-0.20cm] vs 0.30cm [IQR: 0.25-0.35cm]; P=0.03). Using univariate linear regression, maximum systolic blood pressures (SBP), increase in SBP, and absence of beta-blocker use in pregnancy were found to be significantly associated with greater increase in aortic diameter during pregnancy. There were no differences in rates of fetal growth restriction between pregnancies on vs off beta-blockers. Conclusions This is the first study to evaluate changes in aortic dimensions in MFS pregnancies stratified by beta-blocker use. Beta-blocker therapy was found to be associated with less aortic root growth during pregnancy in MFS patients.
Conclusions: Injection of EDP induces immune sensitization as evidenced by a DTH in the ear. Furthermore, the degree of EDP sensitization as measured by DTH correlates with aneurysm volume. EDP-sensitized mice also demonstrated a proportional decrease in Tr1 regulatory cells and an increase in Th17 effector cells, replicating our findings in AAA patients.
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