Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Aug 27;17(6):508-515.
doi: 10.4244/EIJ-D-20-01046.

National French registry of spontaneous coronary artery dissections: prevalence of fibromuscular dysplasia and genetic analyses

Nicolas Combaret  1 , Edouard Gerbaud, François Dérimay, Geraud Souteyrand, Lucie Cassagnes, Sara Bouajila, Takiy Berrandou, Gregoire Rangé, Nicolas Meneveau, Brahim Harbaoui, Benoit Lattuca, Nabila Bouatia-Naji, Pascal Motreff
Affiliations

National French registry of spontaneous coronary artery dissections: prevalence of fibromuscular dysplasia and genetic analyses

Nicolas Combaret et al. EuroIntervention. .

Abstract

Background: Spontaneous coronary artery dissection (SCAD) is an increasingly reported but poorly understood condition. Few European data are available.

Aims: The aims of this study were to obtain European data on SCAD, determine the prevalence of fibromuscular dysplasia (FMD) and enable genetic analyses in this population.

Methods: Data from a national French registry of SCAD cases were analysed prospectively and retrospectively. Clinical and angiographic data and management strategy were collected. Major adverse cardiovascular events (MACE) were analysed after one year of follow-up. Subjects were screened for FMD and blood was collected for DNA extraction.

Results: From June 2016 to August 2018, 373 SCAD cases were confirmed by the core lab. Mean age was 51.5 years. Patients were mostly women (90.6%) and 54.7% of cases had less than two cardiovascular risk factors. At one year, 295 patients (79.1%) were treated conservatively, the MACE rate was 12.3%, and there were no cases of mortality. The recurrence rate of SCAD was 3.3%. FMD was found at ≥1 arterial site in 45.0% of cases. We also confirmed the genetic association between the PHACTR1 locus and SCAD (odds ratio=1.66, p=7.08×10-8).

Conclusions: Here we describe the DISCO registry, the largest European SCAD cohort where FMD was found in 45% of cases and the genetic association with PHACTR1 was confirmed. This nationwide cohort is a valuable resource for future clinical and genetic investigation to understand SCAD aetiology.

PubMed Disclaimer

Conflict of interest statement

The authors/study collaborators have no conflicts of interest to declare in relation to this work.

Figures

Figure 1
Figure 1
Cohort study flow diagram.
Figure 2
Figure 2
Management flow diagram.
See this image and copyright information in PMC

Comment in

  • Letter: Spontaneous coronary artery dissection in France.
    Alfonso F, García-Guimaraes M, Camacho-Freire SJ, Ojeda S, Bastante T, Rivero F. Alfonso F, et al. EuroIntervention. 2021 Aug 27;17(6):525. doi: 10.4244/EIJ-D-21-00026L. EuroIntervention. 2021. PMID: 34413011 Free PMC article. No abstract available.

References

    1. Mahmoud AN, Taduru SS, Mentias A, Mahtta D, Barakat AF, Saad M, Elgendy AY, Mojadidi MK, Omer M, Abuzaid A, Agarwal N, Elgendy IY, Anderson RD, Saw J. Trends of Incidence, Clinical Presentation, and In-Hospital Mortality Among Women With Acute Myocardial Infarction With or Without Spontaneous Coronary Artery Dissection: A Population-Based Analysis. JACC Cardiovasc Interv. 2018;11:80–90. doi: 10.1016/j.jcin.2017.08.016. - DOI - PubMed
    1. Hayes SN, Kim ESH, Saw J, Adlam D, Arslanian-Engoren C, Economy KE, Ganesh SK, Gulati R, Lindsay ME, Mieres JH, Naderi S, Shah S, Thaler DE, Tweet MS, Wood MJ American Heart Association Council on Peripheral Vascular Disease; Council on Clinical Cardiology; Council on Cardiovascular and Stroke Nursing; Council on Genomic and Precision Medicine; Stroke Council. Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association. Circulation. 2018;137:e523–57. - PMC - PubMed
    1. Michelis KC, Olin JW, Kadian-Dodov D, D’Escamard V, Kovacic JC. Coronary artery manifestations of fibromuscular dysplasia. J Am Coll Cardiol. 2014;64:1033–46. doi: 10.1016/j.jacc.2014.07.014. - DOI - PMC - PubMed
    1. Kiando SR, Tucker NR, Castro-Vega LJ, Katz A, D’Escamard V, Tréard C, Fraher D, Albuisson J, Kadian-Dodov D, Ye Z, Austin E, Yang ML, Hunker K, Barlassina C, Cusi D, Galan P, Empana JP, Jouven X, Gimenez-Roqueplo AP, Bruneval P, Hyun Kim ES, Olin JW, Gornik HL, Azizi M, Plouin PF, Ellinor PT, Kullo IJ, Milan DJ, Ganesh SK, Boutouyrie P, Kovacic JC, Jeunemaitre X, Bouatia-Naji N. PHACTR1 Is a Genetic Susceptibility Locus for Fibromuscular Dysplasia Supporting Its Complex Genetic Pattern of Inheritance. PLoS Genet. 2016;12:e1006367. doi: 10.1371/journal.pgen.1006367. - DOI - PMC - PubMed
    1. Adlam D, Olson TM, Combaret N, Kovacic JC, Iismaa SE, Al-Hussaini A, O'Byrne MM, Bouajila S, Georges A, Mishra K, Braund PS, d'Escamard V, Huang S, Margaritis M, Nelson CP, de Andrade M, Kadian-Dodov D, Welch CA, Mazurkiewicz S, Jeunemaitre X CARDIoGRAMPlusC4D Study Group. Association of the PHACTR1/EDN1 Genetic Locus With Spontaneous Coronary Artery Dissection. J Am Coll Cardiol. 2019;73:58–66. - PMC - PubMed