Evidence map›Paper›PMID 42760095›Full record

ArticleOpen heart2026

Prevalence of aortic findings among patients with multifocal fibromuscular dysplasia (FMD) seen at a specialised referral centre.

Karen Ji, Dina Dejanovic, Jennifer E Murphy, Heather L Gornik

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Article in Open heart, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

4 authors.

Karen JiCase Western Reserve University School of Medicine, Cleveland, Ohio, USA.ORCID http://orcid.org/0000-0002-5811-5631
Dina DejanovicUniversity Hospitals Harrington Heart & Vascular Institute, Cleveland, Ohio, USA.
Jennifer E MurphyUniversity Hospitals Cleveland Medical Center, Cleveland, Ohio, USA.
Heather L GornikCase Western Reserve University School of Medicine, Cleveland, Ohio, USA heather.gornik@uhhospitals.org.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundFibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that affects medium-sized arteries with multifocal (beaded) or focal stenotic lesions, aneurysms, dissections and tortuosity. The extent of aortic involvement in FMD is uncertain. This study evaluates the prevalence and characteristics of aortic findings in patients with multifocal FMD.

methodsSingle-centre, retrospective chart review of patients with multifocal FMD seen from January 2019 to July 2024. Patients ≥18 years with complete aortic cross-sectional imaging were included. Demographic, medical history, family history and vascular phenotype were collected. Aortic pathologies were characterised: aneurysms (thoracic diameter ≥4 cm, abdominal diameter ≥3 cm), ectasia, dissection, stenosis and anatomical anomalies.

resultsOf 360 patients (353 female, 98.1%), aortic involvement was identified in 36 patients (10.0%; 95% CI 7.3% to 13.5%): three acute aortic syndromes (<1%), eight aneurysms (2.2%), 17 ectasias (4.7%) and nine congenital anomalies (2.5%). Among those with aortic involvement (n=36), three (8.3%) were male, four (11.1%) had a family history of aneurysm, two (5.6%) had a family history of dissection and nine underwent clinical genetic testing for aortopathies, all of which were negative. All aortic aneurysms and ectasias were in the proximal thoracic aorta (eight aortic root, 17 ascending aorta). No aortic stenosis was found. Of the nine congenital anomalies, seven had an aberrant right subclavian artery, one had ductus diverticulum and one had coarctation. Compared with patients without aortic involvement, those with aortic involvement were more likely to be male and have non-aortic arterial aneurysms.

conclusionAortic findings, predominantly presenting as thoracic ectasia or aneurysm, were identified in 10% of patients with multifocal FMD and complete aortic imaging seen at a single specialised referral centre. These findings support recommendations for head-to-pelvis imaging for patients with FMD and highlight the need for further research into the genetic and systemic aspects of this disease.

Indexed as

Aortic Aneurysm, ThoracicAortic DiseasesFibromuscular DysplasiaReferral and ConsultationAdultAneurysm, Ascending AortaAortographyComputed Tomography AngiographyFemaleHumansMaleMiddle AgedPrevalenceRetrospective StudiesAortic AneurysmAortic DiseasesPeripheral Vascular Diseases

Identifiers

PMID42760095
PMCPMC13599887

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