Cardiovascular Manifestations and Complications of Loeys-Dietz Syndrome: CT and MR Imaging Findings.

Loeys-Dietz syndrome (LDS) is a recently described genetic connective tissue disorder with a wide spectrum of multisystem involvement. LDS is characterized by rapidly progressive aortic and peripheral arterial aneurysmal disease. LDS and the other inherited aortopathies such as Marfan syndrome have overlapping phenotypic features. However, LDS is characterized by a more aggressive vascular course; patient morbidity and mortality occur at an early age, with complications developing at relatively smaller aortic dimensions. In addition, there is more diffuse arterial involvement in LDS, with a large proportion of patients developing aneurysms of the iliac, mesenteric, and intracranial arteries. Early diagnosis and careful follow-up are essential for ensuring timely intervention in patients with arterial disease. Cross-sectional angiography has an important role in the baseline assessment, follow-up, and evaluation of acute complications of LDS, the thresholds and considerations of which differ from those of other inherited aortopathies. In this article, LDS is compared with other genetic vascular connective tissue disorders. In addition, the genetic, histopathologic, and cardiovascular manifestations of this disease process are reviewed, with a focus on computed tomographic and magnetic resonance imaging findings. Online DICOM image stacks and supplemental material are available for this article. ©RSNA, 2018.

Defining the role of endovascular therapy in the treatment of abdominal aortic aneurysm: results of a prospective randomized trial.

Following publication of early registry data showing poor durability for first-generation endografts, EVAR was labeled by some as a failed experiment. The EVAR trial results prove such a pessimistic appraisal of EVAR wrong. In patients fit for open AAA repair EVAR w ith current devicesachieves a 3% benefit in operative and 4-year aneurysm-related mortality compared with open surgery. In patients unfit for open repair 30-day mortality is significantly greater and can no longer be described as safe. Nor does EVAR affect aneurysm-related or all-cause mortality in the 4-year follow-up. EVAR, at least for the first 4 years, is not safe or effective. Based on these results it seems appropriate in unfit patients to attend to concurrent medical problems before considering intervention for an asymptomatic aneurysm. Before the publication of this trial it was generally believed that EVAR would be of benefit in such patients; indeed, it was for the high-risk patient that EVAR was originally conceived. The focus changes from urgency to deploy EVAR to improvement of fitness, recognizing that such patients are very sick with multiple comorbidities. In both fit and unfit patients with large aneurysms most late deaths were cardiovascular related. The importance of risk factor management in both patient groups cannot be overstated. Despite the cost implications of EVAR and its failure to improve mid-term all-cause mortality over open AAA repair it is likely that the bias of both patients and surgeons toward this minimally invasive procedure means that it will continue to have a significant role. Experience and endograft developments have the potential to reduce postoperative complications; surveillance strategies could then be amended to reduce cost implications. Alternatively, over time the currently static rate of complications may increase as endografts reach the end of their working life. The long-term follow-up of patients in both the both EVAR Trials 1 and 2 has the potential for future surprises.