Elsevier

Canadian Journal of Cardiology

Volume 29, Issue 9, September 2013, Pages 1027-1033
Canadian Journal of Cardiology

Review
Spontaneous Coronary Artery Dissection

https://doi.org/10.1016/j.cjca.2012.12.018Get rights and content

Abstract

Spontaneous coronary artery dissection (SCAD) is an infrequent condition that is underdiagnosed. There is a predilection for young women without traditional cardiovascular risk factors, and it is increasingly diagnosed in women who are not peripartum. We discovered an association between SCAD and fibromuscular dysplasia (FMD), in which most women with nonatherosclerotic SCAD were found to have FMD in another vascular territory. We suspect that these seemingly healthy patients have underlying coronary FMD that predisposed them to coronary dissection. Medical treatment of SCAD includes antiplatelet therapy and β-blockade. Revascularization of SCAD patients might be challenging, and the recommendation for stenting or surgery depends on their clinical status and the dissected coronary anatomy. The long-term outcome of patients who survived their SCAD event is generally good, however, they are at risk for recurrent dissection and major cardiovascular events, and thus should be closely monitored by cardiovascular specialists. This review summarizes the epidemiology, associated etiology, diagnosis, management, and outcome of patients with SCAD.

Résumé

La dissection spontanée de l'artère coronaire (DSAC) est une affection rare qui est sous-diagnostiquée. Il existe une prédilection de cette affection chez les jeunes femmes sans facteurs de risque cardiovasculaire traditionnels, et elle est de plus en plus diagnostiquée chez les femmes qui sont en dehors d'une période périnatale. Nous avons découvert un lien entre la DSAC et la dysplasie fibromusculaire (DFM) qui a montré que la plupart des femmes ayant une DSAC non athéroslérotique ont une DFM dans un autre territoire vasculaire. Nous suspectons que ces patientes apparemment en santé ont une DFM coronarienne sous-jacente qui les a prédisposées à la dissection de l'artère coronaire. Le traitement médical de la DSAC inclut le traitement antiplaquettaire et le blocage des récepteurs bêta-adrénergiques. La revascularisation des patientes ayant une DSAC pourrait être difficile, et la recommandation d'implantation d'un tuteur ou de chirurgie dépend de leur état clinique et de l'anatomie de l'artère coronaire disséquée. L'évolution à long terme des patientes qui survivent à leur événement de DSAC est généralement bonne. Cependant, elles sont exposées à un risque de dissection récurrente et d'événements cardiovasculaires majeurs. Par conséquent, elles doivent étroitement être surveillées par des spécialistes cardiovasculaires. Cette revue résume l'épidémiologie associée à l'étiologie, au diagnostic, à la prise en charge et à l'évolution des patientes ayant une DSAC.

Section snippets

Definition and Epidemiology

SCAD is defined as a nontraumatic and noniatrogenic separation of the coronary arterial wall by intramural hemorrhage creating a false lumen, with or without an intimal tear. The separation can occur between the intima and media, or between the media and adventitia. The resulting intramural hematoma (IMH) compresses the arterial lumen, compromising antegrade blood flow to varying degrees, and subsequent myocardial ischemia or infarction.

SCAD is considered a rare cause of acute coronary

Pathogenesis and Pathophysiology

There are 2 proposed mechanisms of SCAD: (1) initiation of a medial dissection and hemorrhage by an intimal tear and creation of a false lumen; and (2) an intramedial hemorrhage without an intimal tear causing a medial dissection, perhaps caused by rupture of the vaso vasorum (Fig. 1).12 The first mechanism might appear angiographically as multiple radiolucent lumens, typically with slow clearing or hang-up of contrast, and rarely have an intimal tear appearance. The second mechanism might

Diagnosis of SCAD

Despite the limitations of angiography, it remains an indispensable diagnostic test for SCAD and should be done promptly in suspected cases. Coronary angiography is a 2-dimensional luminogram, and often inadequate to differentiate atherosclerotic from nonatherosclerotic forms of luminal obstruction. For patients with an intimal tear, SCAD might appear as multiple radiolucent lines separating true and false lumens, with or without slow contrast clearing (Fig. 2). However, patients with IMH

Management of SCAD

Early diagnosis is crucial for managing SCAD patients, because it might preempt the use of unnecessary and potentially harmful pharmacologic therapies. Unfortunately, unlike in the case of atherosclerotic coronary artery disease (CAD), there are no prospective randomized data that specifically address the management of SCAD.

Long-term Prognosis of SCAD

The prognosis of patients with SCAD has not been well studied, with limited case reports and small retrospective case series published. Older reports had suggested high mortality risks with SCAD, with cases often reported as autopsies. However, recent retrospective series show that most patients survive their initial SCAD hospitalization, with low in-hospital mortality ranging from 0 to 4%.6, 9, 10, 11 This variability in acute SCAD outcome is likely a combination of heterogeneity of the

Summary

SCAD remains an infrequent condition that is underdiagnosed among patients presenting with ACS. There is a predilection for young women without traditional cardiovascular risk factors, and SCAD is increasingly diagnosed in women who are not peripartum. We discovered a strong association between SCAD and FMD, where approximately 90% of women with SCAD have noncoronary FMD. We suspect that these seemingly healthy patients have underlying coronary FMD that predisposed them to SCAD. The long-term

Disclosures

The author has no conflicts of interest to disclose.

References (35)

  • D. Hering et al.

    Prospective study of the incidence, pathogenesis and therapy of spontaneous, by coronary angiography diagnosed coronary artery dissection [in German]

    Z Kardiol

    (1998)
  • S.F. Hill et al.

    Non-atherosclerotic coronary artery disease associated with sudden cardiac death

    Heart

    (2010)
  • G. Vanzetto et al.

    Prevalence, therapeutic management and medium-term prognosis of spontaneous coronary artery dissection: results from a database of 11,605 patients

    Eur J Cardiothorac Surg

    (2009)
  • J. Saw et al.

    Non-atherosclerotic coronary artery disease in young women

    J Am Coll Cardiol

    (2011)
  • A.K. Koul et al.

    Coronary artery dissection during pregnancy and the postpartum period: two case reports and review of literature

    Catheter Cardiovasc Interv

    (2001)
  • C.J. Vrints

    Spontaneous coronary artery dissection

    Heart

    (2010)
  • M.S. Tweet et al.

    Clinical features, management and prognosis of spontaneous coronary artery dissection

    Circulation

    (2012)
  • Cited by (162)

    • Cardiovascular Complications of Pregnancy

      2023, Emergency Medicine Clinics of North America
    View all citing articles on Scopus

    See editorial by Mintz, pages 1017-1020 of this issue.

    See page 1032 for disclosure information.

    View full text