{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T14:06:26Z","timestamp":1789740386292,"version":"4.0.1"},"reference-count":16,"publisher":"BMJ","issue":"2","license":[{"start":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T00:00:00Z","timestamp":1789689600000},"content-version":"unspecified","delay-in-days":79,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"John B. Lally Family Chair in Fibromuscular Dysplasia and Vascular Medicine"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Open Heart"],"accepted":{"date-parts":[[2026,8,28]]},"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Fibromuscular 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Single-centre, retrospective chart review of patients with multifocal FMD seen from January 2019 to July 2024. Patients \u226518 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 \u22654 cm, abdominal diameter \u22653 cm), ectasia, dissection, stenosis and anatomical anomalies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of 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 (&lt;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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Aortic 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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/openhrt-2026-004253","type":"journal-article","created":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T13:35:28Z","timestamp":1789738528000},"page":"e004253","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Prevalence of aortic findings among patients with multifocal fibromuscular dysplasia (FMD) seen at a specialised referral centre"],"prefix":"10.1136","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5811-5631","authenticated-orcid":false,"given":"Karen","family":"Ji","sequence":"first","affiliation":[{"name":"Case Western Reserve University School of Medicine, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dina","family":"Dejanovic","sequence":"additional","affiliation":[{"name":"University Hospitals Harrington Heart & Vascular Institute, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer E","family":"Murphy","sequence":"additional","affiliation":[{"name":"University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heather L","family":"Gornik","sequence":"additional","affiliation":[{"name":"Case Western Reserve University School of Medicine, Cleveland, Ohio, USA"},{"name":"University Hospitals Harrington Heart & Vascular Institute, Cleveland, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,9,18]]},"reference":[{"key":"2026091806351864000_13.2.e004253.1","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1177\/1358863X18821816","article-title":"First International Consensus on the diagnosis and management of fibromuscular dysplasia","volume":"24","author":"Gornik","year":"2019","journal-title":"Vasc Med"},{"key":"2026091806351864000_13.2.e004253.2","doi-asserted-by":"crossref","first-page":"365","DOI":"10.3400\/avd.cr.18-00036","article-title":"Abdominal Aortic Aneurysm Caused by Aortic Fibromuscular Dysplasia: A Case Report","volume":"11","author":"Kanamoto","year":"2018","journal-title":"Ann Vasc Dis"},{"key":"2026091806351864000_13.2.e004253.3","doi-asserted-by":"publisher","DOI":"10.1186\/1752-1947-5-22"},{"key":"2026091806351864000_13.2.e004253.4","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.carpath.2017.07.007","article-title":"Aortic fibromuscular dysplasia complicated by dissection: a case report and review of literature","volume":"31","author":"Tasaki","year":"2017","journal-title":"Cardiovasc Pathol"},{"key":"2026091806351864000_13.2.e004253.5","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.112.091223"},{"key":"2026091806351864000_13.2.e004253.6","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2016.04.044"},{"key":"2026091806351864000_13.2.e004253.7","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.121.023858","article-title":"Aortic Dimensions Are Larger in Patients With Fibromuscular Dysplasia","volume":"11","author":"Schwartz","year":"2022","journal-title":"J Am Heart Assoc"},{"key":"2026091806351864000_13.2.e004253.8","doi-asserted-by":"crossref","unstructured":"Szkamruk K , Kruk M , Kepka C , et al . Aortic invovement in fibromuscular dysplasia. 3-dimensional CT, case-control study. THE ARCADIA-POL BIS study. Eur Heart J 2020;41. doi:10.1093\/ehjci\/ehaa946.2327","DOI":"10.1093\/ehjci\/ehaa946.2327"},{"key":"2026091806351864000_13.2.e004253.9","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-015-3234-z"},{"key":"2026091806351864000_13.2.e004253.10","doi-asserted-by":"publisher","DOI":"10.1177\/1358863X15592192"},{"key":"2026091806351864000_13.2.e004253.11","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000001106"},{"key":"2026091806351864000_13.2.e004253.12","unstructured":"Uflacker R . Thoracic aorta and arteries of the trunk. In: Atlas of vascular anatomy: an angiographic approach. 2nd edn. Lippincott Williams & Wilkins, 2007."},{"key":"2026091806351864000_13.2.e004253.13","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2014.07.066"},{"key":"2026091806351864000_13.2.e004253.14","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.07.038"},{"key":"2026091806351864000_13.2.e004253.15","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCGEN.121.003496","article-title":"Fibromuscular Dysplasia and Abdominal Aortic Aneurysms Are Dimorphic Sex-Specific Diseases With Shared Complex Genetic Architecture","volume":"15","author":"Katz","year":"2022","journal-title":"Circ Genom Precis Med"},{"key":"2026091806351864000_13.2.e004253.16","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1038\/s44161-024-00533-w","article-title":"Integrative gene regulatory network analysis discloses key driver genes of fibromuscular dysplasia","volume":"3","author":"d\u2019Escamard","year":"2024","journal-title":"Nat Cardiovasc Res"}],"container-title":["Open Heart"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/openhrt-2026-004253","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T13:35:31Z","timestamp":1789738531000},"score":1,"resource":{"primary":{"URL":"https:\/\/openheart.bmj.com\/lookup\/doi\/10.1136\/openhrt-2026-004253"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":16,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,9,18]]},"published-print":{"date-parts":[[2026,7]]}},"alternative-id":["10.1136\/openhrt-2026-004253"],"URL":"https:\/\/doi.org\/10.1136\/openhrt-2026-004253","relation":{},"ISSN":["2053-3624"],"issn-type":[{"value":"2053-3624","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]}}}