Spontaneous Coronary Artery Dissection and Fibromuscular Dysplasia: Vasculopathies With a Predilection for Women

Abstract

The burden of cardiovascular disease in women is being increasingly appreciated. Nevertheless, both clinicians and the general public are largely unaware that cardiovascular disease is the leading cause of death worldwide in women in all countries and that outcomes after a heart attack are worse for women than men. Of note, certain types of cardiovascular disease have a predilection for women, including spontaneous coronary artery dissection (SCAD) and fibromuscular dysplasia (FMD). Although uncommon, SCAD is being increasingly recognised as the cause of an acute coronary syndrome (ACS) and can recur. It is a potentially fatal, under-diagnosed condition that affects relatively young women, who often have few traditional risk factors, and is the commonest cause of a myocardial infarction associated with pregnancy. In contrast, FMD often remains silent but when manifested can also cause major sequelae, including renal infarction, stroke, cervical artery dissection and gut infarction. Here we provide an update on the diagnosis, aetiology and management of these important disorders that overwhelmingly affect women.

Keywords: Fibromuscular dysplasia; Heart disease; Spontaneous coronary artery dissection; Women.

Figure 1:

Left anterior descending coronary artery occluded by a dissecting haematoma in a 43-year old woman, who died suddenly at rest. Azan-Mallory stain, 17.5 x magnification (from [15] with permission). Abbreviation: LAD, left anterior descending

Figure 2:

Coronary angiograms showing different types of SCAD. (A) Arrows show a dissection flap in the obtuse marginal branch of the circumflex coronary artery consistent with type 1 SCAD. (B) Arrows show diffuse stenosis of the left anterior descending coronary artery consistent with type 2A SCAD. (C) Arrow shows diffuse stenosis to the apical tip of the obtuse marginal branch of the circumflex coronary artery consistent with type 2B SCAD. (D) Arrow shows focal tubular stenosis of the obtuse marginal branch of the circumflex coronary artery consistent with type 3 SCAD. This figure was published in Heart, Lung and Circulation, Vol 27, Graham RM, McGrath-Cadell L, Muller DWM, Holloway CJ, The Mystery and Enigma of Spontaneous Coronary Artery Dissection, pp 401–405, Copyright Elsevier (2018) [10]. Abbreviations: LAD, left anterior descending; SCAD, spontaneous coronary artery dissection

Figure 3:

Pedigree of a family in which three first cousins (generation III) had a spontaneous coronary artery dissection (SCAD) episode at age 40 (propositus; indicated by the arrowhead), 51 and 37 years.

Figure 4:

Typical angiographic appearance of multi-focal fibromuscular dysplasia (FMD) (indicated by white arrows) affecting (A) carotid and (B) renal arteries (from [61] with permission).