Loeys-Dietz Syndrome

Velchev JD, Van Laer L, Luyckx I, Dietz H, Loeys B. Loeys-Dietz Syndrome. Adv Exp Med Biol. 2021;1348:251-264. Full-text for Emory users.

From: Table 11.1. Clinical features at initial diagnosis of LDS. (Velchev JD, et al., p. 253.)

  • Vascular findings
    • Arterial tortuosity 92%
      • Most common in head and neck vessels
        • Carotids (55%)
        • Vertebral (56%)
        • Intracranial (37%)
        • Ascending aorta (5%), aortic arch (10%)
        • Descending thoracic (4%) or abdominal
        • (7%) Ao, also other vessels (e.g. iliacs)
  • Aneurysms
    • Aorta
      • Root 87%
      • Ascending 27%
      • Arch 10%
      • Desc thoracic 15%
      • Abdominal 12%
    • Vessel beyond Ao 30%
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Article of interest: Comparison of outcomes with open, fenestrated, and chimney graft repair of juxtarenal aneurysms: are we ready for a paradigm shift?

Katsargyris A, et al. Comparison of outcomes with open, fenestrated, and chimney graft repair of juxtarenal aneurysms: are we ready for a paradigm shift? J Endovasc Ther. 2013 Apr;20(2):159-69.

Full-text for Emory users.

Results: A total of 2465 vessels were targeted with fenestrations and 151 with chimney grafts (CG); intraoperative target vessel preservation was 98.6% and 98.0%, respectively. Cumulative 30-day mortality was 3.4%, 2.4%, and 5.3% for open surgery, F-EVAR and Ch-EVAR, respectively (p=NS). Impaired renal function was noted in 18.5%, 9.8%, and 12% following open surgery, F-EVAR, and Ch-EVAR, respectively (open vs. F-EVAR: p<0.001). New-onset dialysis was required postoperatively in 3.9%, 1.5%, and 2.1%, respectively (open vs. F-EVAR: p<0.001). Postoperative cardiac complications were noted in 11.3%, 3.7%, and 7.4%, respectively (open vs. F-EVAR: p<0.001). The incidence of ischemic stroke was 0.1% and 0.3% following open surgery and F-EVAR, but 3.2% after Ch-EVAR (open vs. Ch-EVAR: p=0.002; F-EVAR vs. Ch-EVAR: p=0.012). Early proximal type I endoleak was lower after F-EVAR compared to Ch-EVAR (4.3% vs. 10%, respectively, p=0.002).

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Surgical management of ruptured abdominal aortic aneurysms

Powell JT, Wanhainen A. Analysis of the Differences Between the ESVS 2019 and NICE 2020 Guidelines for Abdominal Aortic Aneurysm. Eur J Vasc Endovasc Surg. 2020 Jul;60(1):7-15.

Full-text for Emory users.

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See also: Surgical Grand Rounds: EVAR, FEVAR, and Open Repair: What to make of alphabet soup

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Surgical management of mycotic aortic aneurysms

Huang YK, et al. Clinical, microbiologic, and outcome analysis of mycotic aortic aneurysm: the role of endovascular repair. Surg Infect (Larchmt). 2014 Jun;15(3): 290-8.

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Results: All of the patients had positive blood cultures, radiologic findings typical of MAA, and clinical signs of infection (leukocytosis, fever, and elevated C-reactive protein). The mean age of the patients was 63.8±10.6 y and the mean period of their follow up was 35.7±39.3 mo. Twenty-nine patients with MAAs underwent traditional open surgery, 11 others received endovascular stent grafts, and four MAAs were managed conservatively. The most frequent causative pathogens were Salmonella (36/44 patients [81.8%]), in whom organisms of Salmonella serogroup C (consisting mainly of S. choleraesuis) were identified in 14 patients, organisms of Salmonella serogroup D were identified in 13 patients, and species without serogroup information were identified in nine patients. The overall mortality in the study population was 43.2% (with an aneurysm-related mortality of 18.2%, surgically related mortality of 13.6%, and in-hospital mortality of 22.7%).

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Surgical Grand Rounds: EVAR, FEVAR, and Open Repair: What to make of alphabet soup

Presented by Amanda Fobare, MD, Chief Resident

Department of Surgery, Emory University School of Medicine

February 27, 2020

The evidence: EVAR vs. open repair for elective AAA repair: 

  • EVAR 1 – Endovascular Aneurysm Repair Trial 1 (UK)- Lancet (2005, 2016)
  • DREAM – Dutch Randomized Endovascular Aneurysm Management Trial (Netherlands & Belgium)- NEJM (2004), JVS (2017)
  • OVER – Open versus Endovascular Repair Trial (USA)- JAMA (2009), NEJM (2012)

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AAA repair: retroperitoneal vs transperitoneal approach

One discussion this week included transperitoneal vs retroperitoneal  approach following AAA repair.

Reference: Buck DB, et al. Transperitoneal vs retroperitoneal approach for open abdominal aortic aneurysm repair in the targeted vascular NSQIP. Journal of Vascular Surgery. 2016 Sept;64(3):585-591. doi:10/1016/j.jvs.2016.01.055.

Summary: This study aims to identify the demographic and anatomical differences between patients currently selected for elective transperitoneal versus retroperitoneal AAA repair and to assess differences in intra-operative details, and perioperative mortality and complications.

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