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PreprintCC BY-NC-NDmedRxiv

Fibromuscular Dysplasia in Acute Type A Aortic Dissection: A Common yet Underrecognized Vascular Phenotype in Women

  1. 1Erasmus Medical Center

Abstract

Objectives: Fibromuscular dysplasia (FMD) is a non inflammatory, non atherosclerotic arterial disease characterized by multifocal arterial beading that predominantly affects women. Although FMD commonly involves the renal and carotid arteries, its relationship with acute type A aortic dissection (ATAAD) remains poorly understood. We investigated the proportion of patients with FMD among patients with ATAAD, focusing on sex based differences. Materials and Methods: Patients aged below 70 years with ATAAD and ECG triggered aortic CT angiography (CTA) were consecutively included in this retrospective single-center study. FMD was defined as multifocal arterial beading (string-of-beads appearance) in at least one vascular territory, according to international consensus criteria. The highest-quality CTA examination was evaluated by an experienced cardiovascular radiologist. All suspected FMD cases were independently reviewed by a second experienced cardiovascular radiologist, with the final diagnosis established by consensus. Proportions were reported with 95% confidence intervals and compared between sexes . Results: Of 214 patients screened, 200 were included (138 men and 62 women). FMD was confirmed in 18/200 patients (9%; 95% CI, 6 till 14%), none of whom had a prior FMD diagnosis. FMD was more frequent in women (12/62, 19%; 95% CI, 11 till 31%) than men (6/138, 4%; 95% CI, 2 till 9%; P<0.001). FMD predominantly involved the renal arteries. Additional carotid CTA was available in 49/200 patients (25%) and in 7/18 patients with FMD (39%); three of these seven patients had carotid FMD. Conclusion: FMD was observed in a substantial proportion of patients presenting with ATAAD, particularly women. These findings suggest that FMD is an underrecognized vascular phenotype in patients with ATAAD.

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BibTeX
@misc{weustink2026fibromuscular,
  title = {{Fibromuscular Dysplasia in Acute Type A Aortic Dissection: A Common yet Underrecognized Vascular Phenotype in Women}},
  author = {A. C. Weustink and A. R. Keijzer and R. P. J. Budde and J. Versmissen and T. W. Galema and K. M. Veen and J. J. M. Takkenberg and B. Arabkhani and A. Coenen},
  howpublished = {medRxiv},
  year = {2026},
  month = nov,
  doi = {10.64898/2026.11.03.26367049},
  url = {https://www.medrxiv.org/content/10.64898/2026.11.03.26367049v1},
  note = {CC BY-NC-ND},
}
RIS
TY  - UNPB
TI  - Fibromuscular Dysplasia in Acute Type A Aortic Dissection: A Common yet Underrecognized Vascular Phenotype in Women
AU  - A. C. Weustink
AU  - A. R. Keijzer
AU  - R. P. J. Budde
AU  - J. Versmissen
AU  - T. W. Galema
AU  - K. M. Veen
AU  - J. J. M. Takkenberg
AU  - B. Arabkhani
AU  - A. Coenen
PY  - 2026
DA  - 2026/11/03
PB  - medRxiv
DO  - 10.64898/2026.11.03.26367049
UR  - https://www.medrxiv.org/content/10.64898/2026.11.03.26367049v1
AB  - Objectives: Fibromuscular dysplasia (FMD) is a non inflammatory, non atherosclerotic arterial disease characterized by multifocal arterial beading that predominantly affects women. Although FMD commonly involves the renal and carotid arteries, its relationship with acute type A aortic dissection (ATAAD) remains poorly understood. We investigated the proportion of patients with FMD among patients with ATAAD, focusing on sex based differences. Materials and Methods: Patients aged below 70 years with ATAAD and ECG triggered aortic CT angiography (CTA) were consecutively included in this retrospective single-center study. FMD was defined as multifocal arterial beading (string-of-beads appearance) in at least one vascular territory, according to international consensus criteria. The highest-quality CTA examination was evaluated by an experienced cardiovascular radiologist. All suspected FMD cases were independently reviewed by a second experienced cardiovascular radiologist, with the final diagnosis established by consensus. Proportions were reported with 95% confidence intervals and compared between sexes . Results: Of 214 patients screened, 200 were included (138 men and 62 women). FMD was confirmed in 18/200 patients (9%; 95% CI, 6 till 14%), none of whom had a prior FMD diagnosis. FMD was more frequent in women (12/62, 19%; 95% CI, 11 till 31%) than men (6/138, 4%; 95% CI, 2 till 9%; P<0.001). FMD predominantly involved the renal arteries. Additional carotid CTA was available in 49/200 patients (25%) and in 7/18 patients with FMD (39%); three of these seven patients had carotid FMD. Conclusion: FMD was observed in a substantial proportion of patients presenting with ATAAD, particularly women. These findings suggest that FMD is an underrecognized vascular phenotype in patients with ATAAD.
ER  -