The spectrum of spontaneous coronary artery dissection: illustrated review of the literature.

Spontaneous coronary artery dissection or SCAD is a rare and challenging disease that is increasingly diagnosed. It is characterized by a non-traumatic, non-iatrogenic separation of the coronary artery wall and occurs predominantly in young and middle-aged women without traditional cardiovascular risk factors. SCAD is often associated with predisposing conditions such as the peripartum period, systemic inflammatory disease and heritable connective tissue disease. More recently, independent investigators have demonstrated an important association with fibromuscular dysplasia. Extreme emotional or physical stress as well as intense hormonal therapy or drug abuse have been pointed out as precipitating factors. The diagnosis of SCAD can be challenging and starts with clinical suspicion. Advanced imaging techniques such as intravascular ultrasound and optical coherence tomography are useful for the differentiation from atherosclerotic disease and are increasingly used for this indication. The proposed treatment in the acute setting is based on findings from single-centre retrospective series: in stable patients with a TIMI-flow ≥2 a conservative management is proposed because of the high risk of procedural failure and complications as well as a high probability of spontaneous healing. Long-term treatment is comparable to that in non-SCAD acute coronary syndromes (ACS) but dual antiplatelet therapy should only be started in case of stenting and should be kept as short as possible in patients with vascular Ehlers-Danlos syndrome. Prognosis seems to be better compared to non-SCAD ACS but there is a reasonable risk of recurrence. In this review, we discuss the current knowledge of SCAD and provide a clinical pathway for the diagnosis, management and work-up of SCAD patients.