ReviewSpontaneous coronary artery dissection
Introduction
Spontaneous coronary artery dissection (SCAD) is a vessel wall lesion characterized by an intramural hematoma (ie., false lumen) flattening the lumen (ie., true lumen) through the shift of the inner media against the opposite wall, which by definition requires the absence of an iatrogenic, non-coronary or traumatic etiology [1], [2]. According to the underlying pathophysiology, SCAD has also been named “primary” coronary artery dissection, in contrast to the definitely more common “secondary” coronary artery dissection, which in contrast is explained by known factors, such as coronary catheterization, percutaneous coronary intervention (PCI), cardiac surgery, extended aortic root dissection or chest trauma (Fig. 1) [3], [4], [5], [6].
SCADs generally present an intimal–media tear producing a communication between the vessel lumen and the intramural hematoma (“SCAD with entry door”) (Fig. 2, left). Blood coming into the false lumen clots and spreads the flap of detached tissue. The dissection plane, located between the intima and the media or more commonly in the outer media, delimits a new hollow (false lumen) causing usually severe luminal narrowing and distortion [1], [2], [7]. However, several SCADs at least initially are only intramural hematomas that compress the lumen from the outside reducing the blood flow (“angiographically-invisible SCAD”). Subsequently some of these hematomas can evolve as a result of continuous intramural bleeding and/or structural abnormalities (ie., inflammatory infiltrates, cystic medial necrosis) with the final intimal–media disruption promoting suddenly a further path of growth [1], [2], [7], [8], [9] (Fig. 2, right).
The typical angiographic sign of coronary dissection is a radiolucent intimal–media flap (double lumen), frequently associated with persistent extra-luminal filling and/or delayed clearance of contrast media from the lumen, detected in at least 2 orthogonal projections [1], [2], [10] (Fig. 3A). However, as mentioned above, SCAD without luminal entry door frequently determines exclusively a smooth and regular coronary narrowing due to intramural hematoma eversion into the lumen [11], [12]. These lesions not fulfilling the classical angiographic pattern of coronary dissection can be detected with tomographic imaging techniques, such as intravascular ultrasound (IVUS) and optical coherence tomography (OCT) (Fig. 3B, C) [11], [12].
The aim of this article is to provide a comprehensive description of SCADs, review their presentation and outcomes from the available literature, and critically appraise current diagnostic and therapeutic options.
Section snippets
Epidemiology of SCAD
SCAD is a relatively rare presentation of coronary disease with an estimated prevalence of 0.07–0.28% [12], [13], [14], [15], [16], [17] and an annual incidence of 0.26 cases per 100,000 persons (0.33 in women, 0.18 in men) among US subjects [18]. Since the first case described by Pretty in 1931 [19], a total of only ~ 1125 SCADs (Appendix A) have been reported in literature consisting largely of individual cases or case series and rarely of small single-center registries (< 90 patients).
Patients
Classification
Patients with SCAD are traditionally grouped into three subsets: those affected by coronary atherosclerotic disease (CAD), young women in the peripartum period and patients without known underlying coronary disease (idiopathic SCAD) [27] (Fig. 1).
Pathophysiology and risk factors of SCAD
The etiology of SCAD is still unknown but certainly multi-factorial and complex (Fig. 6). Recurrent and multi-vessel SCADs might suggest a pathologic condition involving all coronary segments; however the superimposed mechanism leading to SCAD in some patients, differently from others with equivalent risk conditions, is unknown.
Clinical presentation and laboratory findings of SCAD
SCAD presentation encompasses the entire spectrum of coronary syndromes, ranging from asymptomatic patients, across those presenting stable angina, NSTEMI, STEMI, to SCD. Regardless of the type and cause, SCAD impairs myocardial perfusion, reducing the vessel lumen area similarly to atherosclerotic stenoses; therefore symptoms usually do not differ from those observed in ACS for CAD, but sometimes the clinical setting can be atypical and even misleading. Chest pain is the main symptom (91%)
Diagnosis of SCAD
The diagnosis of SCAD is almost exclusively incidental during coronary angiography following STEMI or UA/NSTEMI. Coronary angiography represents the first line test because it can rule out suspected SCAD. The angiographic pattern, however, is highly variable ranging from long and multiple dissections in an otherwise physiologic coronary tree to a single short dissection in atherosclerotic and calcific coronary vessels. Coronary angiography becomes less sensitive in specific conditions such as
Treatment of SCAD
The lack of consensus surrounding the treatment of SCAD stems from its clinical heterogeneity and the evidence of different successful therapeutic strategies. In non-life-threatening SCAD, long-term benefits of invasive revascularization compared to medical treatment are uncertain. Management decision should be made case-by-case, integrating clinical conditions and echocardiographic parameters with angiography and endovascular imaging to recognize SCAD location, extent and characteristics as
Prognosis of SCAD
Early analyses defined SCAD as a rare coronary disease, diagnosed in 69% of cases only post-mortem, characterized by a poor prognosis and a strong association with SCD (75%) [3], [20]. Furthermore a twenty-year post-mortem registry classified SCAD as the second most common cause of non-atherosclerotic SCD [187]. Later, a comprehensive review reported an approximate mortality of 50%; patients who survived the main episode presented a survival probability of 80% which decreased approximately to
Conclusion
SCAD is a relatively rare type of coronary artery disease. The available information derives from case reports, case series and small retrospective mono-center registries. The optimal management of SCAD is uncertain because of the limited amount of data in literature and the heterogeneous presentation. According to clinical conditions and lesion characteristics, a case-specific treatment seems the most reasonable strategy. Prospective multicenter registry involving a larger number of patients
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2019, International Journal of CardiologyCitation Excerpt :In case of uncertainty intracoronary imaging with OCT/IVUS can be done to establish the correct diagnosis. In almost 60% the LAD is affected, followed by the RCA (26%) and RCX (19%) [6]. Although hypertension is present in one third of SCAD patients, other traditional cardiovascular disease (CVD) risk factors are less often reported than in traditional ACS [1].