Spontaneous Coronary Artery Dissection: A Review of Epidemiology, Pathophysiology and Principles of Management
Introduction
Spontaneous coronary artery dissection (SCAD) is defined as an acute tear separating the inner layers from the outer layers of coronary artery wall leading to a false lumen and an intramural hematoma formation.1 These anatomic changes compressing the coronary lumen may result in acute coronary syndrome, arrhythmia, and sudden death, especially in women.2 Whilst it was first reported in 1931 on autopsy of a young lady,3 the exact cause of SCAD remains unknown. However, several conditions were identified as precipitant factors for SCAD, like fibromuscular dysplasia, inherited connective tissue diseases (autosomal dominant polycystic kidney disease, Ehlers-Danlos, Marfan syndrome, Loeys-Dietz syndrome), exogenous hormone (oral contraceptives or infertility treatments), systemic inflammatory disease, arteriopathy, corticosteroids, recreational drugs use, genetic predisposition, hypothyroidism, migraine, and pregnancy4, 5, 6, 7, 8, 9, 10 (Fig 1). To date, the absence of randomized clinical trials has resulted in personalized heterogeneous clinical practice. Available data derived from anecdotal evidence such as case series, observational studies, and expert consensus. The purpose of this review is to increase the awareness of early recognition and appropriate approach of this nonatherosclerotic setting of acute coronary syndrome among health care providers in the departments of cardiology. Therefore, it summarizes the existing literature on epidemiology, physiopathology, diagnostic modalities, outcomes, and principles of management of SCAD including conservative therapy, revascularization strategy, and long-term follow-up.
Section snippets
Epidemiology
SCAD accounts for up to 4% of acute coronary syndrome overall,11,12 43% of pregnancy-related acute myocardial infarction,13,14 and 35% of acute coronary syndrome in women under 60 years of age.15,16 SCAD is no longer considered as a rare condition as initially thought to be, but its true prevalence remains unknown, largely due to underdiagnosis. It is forcefully seen in young and middle-aged women (age 44-62) lacking typical cardiovascular risk factors, thereby just 10% of patients presenting
Physiopathology
To date, the pathogenesis of SCAD is based on two hypotheses: “inside-out” and “outside-in” mechanisms21,23 (Fig 1). The first consists of an intimal tear in the most inner layer of coronary wall (tunica intima) generating a blood flow into a false lumen that ends with an intramural hematoma. The latter consists of a spontaneous rupture of the vasa vasorum in the most outer layer (tunica adventitia) resulting in intra-arterial wall hemorrhage, and subsequently intramural hematoma. The
Diagnosis
A high level of suspicion is required for diagnosing SCAD among patients presenting with acute coronary syndrome. The gold standard diagnostic modality is coronary angiography. Three different types have been identified on angiography.1 Type 1 is characterized by several radiolucent lumens with extraluminal contrast staining, and it is pathognomonic of SCAD but found in a small proportion of cases. Type 2 is defined by a diffuse coronary narrowing of more than 20 mm of length with variant
Conservative Approach
Current data support the conservative medical management of SCAD as it was associated with a reduction in major adverse cardiovascular events during the first month.31 Betablockers are the cornerstone of medical therapy and act by reducing coronary wall shear stress, lowering blood pressure, and modulating heart rate which result in a significant decrease in the risk of propagation and recurrence.15,32 View the lake of standard guidelines, expert consensus on duration of dual antiplatelet
Outcomes and Prognosis of SCAD
The estimated mortality rate of SCAD is about 1% over 33 months of follow-up.50 Males, smoking, and STEMI presentation are associated with an increased risk of death.50 A prevalence of adverse cardiovascular outcomes of 5.8 events/100 person-years is reported after SCAD.41 The observed recurrence rate of de novo SCAD was of 10.4%.15 Systemic hypertension was the predictor of recurrence while beta-blockers use has a protective effect.15 The rates of all-cause mortality and recurrence did not
Conclusion
To summarize, the diagnosis of SCAD requires a high level of suspicion, and its management is a personalized care analysed on case-by-case basis. Conservative approach combining beta-blockers with long-term single anti-platelet therapy is the best therapeutic option. Revascularization procedures are reserved to selected category of patients with specific conditions. SCAD is associated with favourable outcomes and overall survival. Further prospective randomized clinical trials are warranted to
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The authors have no conflicts of interest to disclose.