Clinical Characteristics and Outcomes of Aortic Arch Emergencies: Takayasu Disease, Fibromuscular Dysplasia, and Aortic Arch Pathologies: A Retrospective Study and Review of the Literature

Abstract

Non-atherosclerotic aortic arch pathologies (NA-AAPs) and anatomical variants are characterized as rare cardiovascular diseases with a low incidence rate, below 1 case per 2000 population, but enormous heterogeneity in terms of anatomical variants, i.e., Takayasu disease (TAK) and fibromuscular dysplasia (FMD). In specific clinical scenarios, NA-AAPs constitute life-threatening disorders. Methods: In this study, 82 (1.07%) consecutive patients with NA-AAPs (including 38 TAKs, 26 FMDs, and 18 other AAPs) out of 7645 patients who underwent endovascular treatment (EVT) for the aortic arch and its side-branch diseases at a single institution between 2002 and 2022 were retrospectively reviewed. The recorded demographic, biochemical, diagnostic, operative, and postoperative factors were reviewed, and the functional outcomes were determined during follow-up. A systematic review of the literature was also performed. Results: The study group comprised 65 (79.3%) female and 17 (21.7%) male subjects with a mean age of 46.1 ± 14.9 years. Overall, 62 (75.6%) patients were diagnosed with either cerebral ischemia symptoms or aortic arch dissection on admission. The EVT was feasible in 59 (72%) patients, whereas 23 (28%) patients were referred for medical treatment. In EVT patients, severe periprocedural complications occurred in two (3.39%) patients, including one periprocedural death and one cerebral hyperperfusion syndrome. During a median follow-up period of 64 months, cardiovascular events occurred in 24 (29.6%) patients (5 deaths, 13 ISs, and 6 myocardial infarctions). Repeated EVT for the index lesion was performed in 21/59 (35.6%) patients, including 19/33 (57.6%) in TAK and 2/13 (15.4%) in FMD. In the AAP group, one patient required additional stent-graft implantation for progressing dissection to the iliac arteries at 12 months. A baseline white blood count (odds ratio [HR]: 1.25, 95% confidence interval [CI]: 1.11–1.39; p < 0.001) was the only independent prognostic factor for recurrent stenosis, while a baseline hemoglobin level (HR: 0.73, 95%CI: 0.59–0.89; p = 0.002) and coronary involvement (HR: 4.11, 95%CI: 1.74–9.71; p = 0.001) were independently associated with a risk of major cardiac and cerebral events according to the multivariate Cox proportional hazards regression analysis. Conclusions: This study showed that AAPs should not be neglected in clinical settings, as it can be a life-threatening condition requiring a multidisciplinary approach. The knowledge of prognostic risk factors for adverse outcomes may improve surveillance in this group of patients.

Coarctation of the aorta, carotid artery stenosis and aberrant right subclavian artery as a rare cause of cerebral ischemia in a primigravid woman

“…The reported patient with congenital AA anomaly and CoA represents one of 5 young female patients (0.065%), who underwent urgent endovascular procedure during the peri-partum and post-partum period in our registry of 82 (1.07%) out of 7,645 young patients who had endovascular procedure performed in our tertiary referral hospital in the department of vascular and endovascular surgery over the time period of 20 years ( 6 ). This demonstrates the rarity of such cases.…”

“…Among stroke patients between 18 to 50 years old, also other vascular diseases, like congenital or iatrogenic artery stenosis, Takayasu disease, fibromuscular dysplasia, premature atherosclerosis, moyamoya disease, cerebral venous thrombosis, and reversible cerebral vasoconstriction syndrome have been more frequently found compared to non-young adults ( 6 , 8 , 9 ).…”

Exploring the Effects of Local Air Pollution on Popliteal Artery Aneurysms

“…However, both pathologies also share similar risk factors, which limits the quantification of the potential inheritable risk. Nevertheless, these aneurysms feature both clinically and histologically distinct profiles different from aneurysms resulting from inherited congenital dysfunctions of the collagen/elastin tissue of the arterial wall, where adverse arterial events are mostly encountered in relatively young patients, frequently in emergency settings [ 37 ]. PAAs are most commonly identified in patients above 60, which indicates that the pathology is a result of long-lasting exposure to risk factors and causal mediators.…”

The Utility of Three-Dimensional Printing in Physician-Modified Stent Grafts for Aortic Lesions Repair

“…This includes the bovine arch, arteria lusoria, or common bicarotid trunk, which increases the risk of aortic dissection or cerebral ischemia. The long-term treatment effect, especially in younger patients, depends on the quality of implemented stent grafts [61]. The personalized approach of 3D printing might also be an indication of NA-AAPs increasing the precision of the endovascular procedures by tailoring the stent grafts to the specific needs of patient anatomical aortic variants.…”