Prevalence and Disease Spectrum of Extracoronary Arterial Abnormalities in Spontaneous Coronary Artery Dissection

Alexandre Persu, Marilucy Lopez-Sublet, Abtehale Al-Hussaini, Marco Pappaccogli, Ibtissem Radhouani, Patricia Van der Niepen, William Adair, Christophe Beauloye, Pierre-Yves Brillet, Nathan Chan, Patrick Chenu, Hannes Devos, Javier Escaned, Marcos Garcia-Guimaraes, Frank Hammer, Robert Jackson, Salma Jebri, Deevia Kotecha, Fernando Macaya, Ciara MahonNalin Natarajan, Kandiyil Neghal, Edward D Nicol, Kelly S Parke, Diluka Premawardhana, Averachan Sajitha, Joanne Wormleighton, Nilesh J Samani, Gerry P McCann, David Adlam

Research output: Contribution to journalArticlepeer-review

3 Citations (Scopus)


Importance: Spontaneous coronary artery dissection (SCAD) has been associated with fibromuscular dysplasia (FMD) and other extracoronary arterial abnormalities. However, the prevalence, severity, and clinical relevance of these abnormalities remain unclear.

Objective: To assess the prevalence and spectrum of FMD and other extracoronary arterial abnormalities in patients with SCAD vs controls.

Design, Setting, and Participants: This case series included 173 patients with angiographically confirmed SCAD enrolled between January 1, 2015, and December 31, 2019. Imaging of extracoronary arterial beds was performed by magnetic resonance angiography (MRA). Forty-one healthy individuals were recruited to serve as controls for blinded interpretation of MRA findings. Patients were recruited from the UK national SCAD registry, which enrolls throughout the UK by referral from the primary care physician or patient self-referral through an online portal. Participants attended the national SCAD referral center for assessment and MRA.

Exposures: Both patients with SCAD and healthy controls underwent head-to-pelvis MRA (median time between SCAD event and MRA, 1 [IQR, 1-3] year).

Main Outcome and Measures: The diagnosis of FMD, arterial dissections, and aneurysms was established according to the International FMD Consensus. Arterial tortuosity was assessed both qualitatively (presence or absence of an S curve) and quantitatively (number of curves ≥45%; tortuosity index).

Results: Of the 173 patients with SCAD, 167 were women (96.5%); mean (SD) age at diagnosis was 44.5 (7.9) years. The prevalence of FMD was 31.8% (55 patients); 16 patients (29.1% of patients with FMD) had involvement of multiple vascular beds. Thirteen patients (7.5%) had extracoronary aneurysms and 3 patients (1.7%) had dissections. The prevalence and degree of arterial tortuosity were similar in patients and controls. In 43 patients imaged with both computed tomographic angiography and MRA, the identification of clinically significant remote arteriopathies was similar. Over a median 5-year follow-up, there were 2 noncardiovascular-associated deaths and 35 recurrent myocardial infarctions, but there were no primary extracoronary vascular events.

Conclusions and Relevance: In this case series with blinded analysis of patients with SCAD, severe multivessel FMD, aneurysms, and dissections were infrequent. The findings of this study suggest that, although brain-to-pelvis imaging allows detection of remote arteriopathies that may require follow-up, extracoronary vascular events appear to be rare.

Original languageEnglish
Pages (from-to)159-166
Number of pages8
JournalJAMA Cardiology
Issue number2
Early online date24 Nov 2021
Publication statusPublished - 1 Feb 2022

Bibliographical note

Funding Information:
receivinggrantsfromtheBritishHeartFoundation,the NationalInstituteforHealthResearch(NIHR),andMedi-cal Research Council outside the submitted work; and wasacollaboratoronresearchwithCircleCVi.DrAdlam reportedreceivinggrantsfromAbbottVasculartosup-port a clinical research fellow, honoraria from General Electric as a consultant to support general research funds,grantsfromAstraZenecatosupportunrelatedre-search, and nonfinancial support from AstraZeneca to supportspontaneouscoronaryarterydissection(SCAD) geneticsresearchoutsidethesubmittedwork;inaddi-tion,DrAdlamhadpatentsissuedforEP3277337A1and PCT/GB2017/050877 unrelated to this work. No other disclosures were reported. Funding/Support: This work was supported by BeatSCAD, the British Heart Foundation PG/13/96/ 30608, NIHR rare disease translational collaboration, and the Leicester NIHR Biomedical Research Centre. Dr McCann is directly funded by the NIHR (CDF-2014-07-45 and RP-2017-08-ST2-007). Role of the Funder/Sponsor: The funding organizations had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. Additional Contributions: We acknowledge the lead-ershipoftheEuropeanSocietyofCardiology-Association forAcuteCardiovascularCareStudyGroup.Wearegrate-fulforthesupportofthestudyparticipantsandthankour clinicalcolleaguesthroughouttheUKwhoreferredpa-tientswithSCADtoourstudy.Wespecificallyacknowl-edgethesupportofresearchnursesornursingassistants EllieClarke,BSc,DipHEAdultNursing,JanePlumeand DonnaAlexander,MSc;SCADstudyadministratorsJenny Middleton,BA(Hons),andRebeccaWrack;SueSterland, RichardBramley,BSc(Hons),DiplomainComputingfor CommerceandIndustry,andDanielLawdayforbuilding and supporting the study eCRF; Emma Beeston, BSc, Diploma Adult Nursing, Tara Maitland, BA(Hons), and Andrea Marshall for study management and research governance support (all affiliated with the University HospitalsofLeicester,UK),andBrunoMorgan,BM,MA, PhD, for technical support and advice on secure image anonymizationandtransfer(affiliatedwiththeUniver-sityofLeicester,UK).Wearegratefultothemforalltheir support for SCAD research. Marilucy Lopez-Sublet is gratefultoRobinDhote,MD,PhD(DepartmentofInter-nal Medicine, CHU Avicenne, AP-HP, Bobigny, France), forhisconstantsupport.AlexandrePersuacknowledges hisformerstudentXiangLi,MD(DivisionofCardiology, CliniquesUniversitairesSaint-Luc,Brussels,Belgium),for his contribution to the protocol of analysis of arterial tortuosity. None of the contributors received financial compensation. AdditionalInformation:Phenotypingwasperformed in the NIHR Leicester Clinical Research Facility.

Publisher Copyright:
© 2022 American Medical Association. All rights reserved.

Copyright 2022 Elsevier B.V., All rights reserved.


  • Spontaneous coronary artery dissection
  • fibromuscular dysplasia
  • extracoronary arterial abnormalities
  • prevalence
  • severity
  • clinical relevance
  • imaging


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