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In this study of abdominal aortic aneurysm repair, endovascular repair was shown to have an early survival advantage over open repair during the first three years. However, interventions related to aneurysm and ruptures were more common after endovascular repair. The use of endovascular repair of abdominal aortic aneurysms is increasing. By 2010, endovascular repair accounted for 78% of all intact repairs. 1 , 2 Randomized, controlled trials comparing endovascular repair with open repair generally have shown a perioperative benefit of endovascular repair over open repair. 3 – 5 Long-term survival, however, is similar with the two approaches. 6 – 9 As data on long-term outcomes accumulate, concerns have been raised about endovascular repair with respect to the increased rate of late failure leading to rupture and higher rates of reintervention. In our previous analyses performed with the use of Medicare data, which account for more . . .

Key Points Endovascular aneurysm repair (EVAR), rather than open repair, is currently the treatment of choice for most patients with an anatomically suitable infrarenal abdominal aortic aneurysm (AAA) Clinical evidence-based research shows a lower perioperative morbidity and mortality, and similar long-term survival, for EVAR compared with open repair of suitable infrarenal AAAs The indications for endovascular management of AAA are expanding to include increasingly complex and anatomically challenging aneurysms Challenging anatomy might require the use of fenestrated and branched stent grafts, chimney grafts, or the sandwich technique Future directions for stent grafts include fenestrated and branched off-the-shelf stent grafts, multilayer stents, endoanchor systems, and sac-anchoring endoprostheses Stent graft technology for infrarenal AAA continues to evolve, with profile downsizing, optimization of sealing and fixation, and the use of fabrics with reduced porosity Endovascular aneurysm repair has become the standard of care in many hospitals for patients with abdominal aortic aneurysms (AAAs) who have anatomy deemed suitable for the procedure. In this Review, Dominique Buck and colleagues discuss evidence-based practice and evaluate promising new strategies for endovascular repair of AAAs. The role of imaging in the management of AAAs is also highlighted. Patients with abdominal aortic aneurysms (AAAs) are usually treated with endovascular aneurysm repair (EVAR), which has become the standard of care in many hospitals for patients with suitable anatomy. Clinical evidence indicates that EVAR is associated with superior perioperative outcomes and similar long-term survival compared with open repair. Since the randomized, controlled trials that provided this evidence were conducted, however, the stent graft technology for infrarenal AAA has been further developed. Improvements include profile downsizing, optimization of sealing and fixation, and the use of low porosity fabrics. In addition, imaging techniques have improved, enabling better preoperative planning, stent graft placement, and postoperative surveillance. Also in the past few years, fenestrated and branched stent grafts have increasingly been used to manage anatomically challenging aneurysms, and experiments with off-label use of stent grafts have been performed to treat patients deemed unfit or unsuitable for other treatment strategies. Overall, the indications for endovascular management of AAA are expanding to include increasingly complex and anatomically challenging aneurysms. Ongoing studies and optimization of imaging, in addition to technological refinement of stent grafts, will hopefully continue to broaden the utilization of EVAR.

Nat. Rev. Cardiol. 11, 112–123 (2014); doi:10.1038/nrcardio.2013.196 In the version of this article originally published, the following statement was missing from the acknowledgements: This work was supported by the NIH T32 Harvard-Longwood Research Training in Vascular Surgery grant HL007734. This error has been corrected in the HTML and PDF versions of the article.

Background Arteriosclerosis and emphysema develop in individuals with Schimke immuno-osseous dysplasia (SIOD), a multisystem disorder caused by biallelic mutations in SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1). However, the mechanism by which the vascular and pulmonary disease arises in SIOD remains unknown. Methods We reviewed the records of 65 patients with SMARCAL1 mutations. Molecular and immunohistochemical analyses were conducted on autopsy tissue from 4 SIOD patients. Results Thirty-two of 63 patients had signs of arteriosclerosis and 3 of 51 had signs of emphysema. The arteriosclerosis was characterized by intimal and medial hyperplasia, smooth muscle cell hyperplasia and fragmented and disorganized elastin fibers, and the pulmonary disease was characterized by panlobular enlargement of air spaces. Consistent with a cell autonomous disorder, SMARCAL1 was expressed in arterial and lung tissue, and both the aorta and lung of SIOD patients had reduced expression of elastin and alterations in the expression of regulators of elastin gene expression. Conclusions This first comprehensive study of the vascular and pulmonary complications of SIOD shows that these commonly cause morbidity and mortality and might arise from impaired elastogenesis. Additionally, the effect of SMARCAL1 deficiency on elastin expression provides a model for understanding other features of SIOD.