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

Author:

Wawak Magdalena1,Tekieli Łukasz1,Badacz Rafał12ORCID,Pieniążek Piotr13,Maciejewski Damian1,Trystuła Mariusz4,Przewłocki Tadeusz13,Kabłak-Ziembicka Anna25ORCID

Affiliation:

1. Department of Interventional Cardiology, The John Paul II Hospital, Prądnicka 80, 31-202 Kraków, Poland

2. Department of Interventional Cardiology, Institute of Cardiology, Jagiellonian University Medical College, św. Anny 12, 31-007 Kraków, Poland

3. Department of Cardiac and Vascular Diseases, Institute of Cardiology, Jagiellonian University Medical College, św. Anny 12, 31-007 Kraków, Poland

4. Department of Vascular and Endovascular Surgery, The John Paul II Hospital, Prądnicka 80, 31-202 Kraków, Poland

5. Noninvasive Cardiovascular Laboratory, The John Paul II Hospital, Prądnicka 80, 31-202 Kraków, Poland

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.

Funder

John Paul II Hospital, Cracow, Poland

Jagiellonian University

Publisher

MDPI AG

Subject

General Biochemistry, Genetics and Molecular Biology,Medicine (miscellaneous)

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