Spontaneous coronary artery dissection: A review of medical management approaches.

Spontaneous coronary artery dissection (SCAD) is an underdiagnosed cause of acute coronary syndrome (ACS) that usually presents in young female patients. Risk factors include female sex, physical and emotional stressors, and fibromuscular dysplasia, and diagnosis is usually made by coronary angiography aided by intravascular ultrasound (IVUS) or optical coherence tomography (OCT). While conservative treatment is usually preferred over percutaneous coronary intervention or surgery, medical management of SCAD has been under debate. This comprehensive review aims to summarize findings from recent studies exploring various medical treatment approaches for the management of SCAD. Antiplatelet therapy with aspirin is generally safe and beneficial for SCAD patients, with dual antiplatelet (DAPT) being recommended for patients undergoing PCI. In the absence of intervention, DAPT may be given for a short period followed by a longer single-antiplatelet (SAPT) therapy with aspirin. Beta-blockers appear to be safe and effective for SCAD patients. On the other hand, fibrinolytics, anticoagulants, and glycoprotein IIa/IIIb inhibitors are contraindicated. Cardiovascular medications such as renin-angiotensin-aldosterone system (RAAS) inhibitors, mineralocorticoid receptor antagonists, and statins are not recommended in the absence of left ventricular dysfunction. Hormonal therapy is contraindicated for patients who develop SCAD during pregnancy and future pregnancy is discouraged in that patient population.

Diagnosis and Management of Congenital Coronary Artery Fistulas in Adults.

PURPOSE OF REVIEW: This review describes the presentation, diagnosis, and management of congenital coronary artery fistulas (CAFs) in adults. RECENT FINDINGS: CAFs are classified as coronary-cameral or coronary arteriovenous fistulas. Fistulous connections at the distal coronary bed are more likely to be aneurysmal with higher risk of thrombosis and myocardial infarction (MI). Medium-to-large or symptomatic CAFs can manifest as ischemia, heart failure, and arrhythmias. CAF closure is recommended when there are attributable symptoms or evidence of adverse coronary remodeling. Closure is usually achievable using transcatheter techniques, though large fistulas may require surgical ligation with bypass. Given their anatomic complexity, cardiac CT with multiplanar 3-D reconstruction can enhance procedural planning of CAF closure. Antiplatelet and anticoagulation are essential therapies in CAF management. CAFs are rare cardiac anomalies with variable presentations and complex anatomy. CAF management strategies include indefinite medical therapy, percutaneous or surgical CAF closure, and lifelong patient surveillance.

Spontaneous Coronary Artery Dissection in Clinical Practice: Pathophysiology and Therapeutic Approaches.

Spontaneous coronary artery dissection (SCAD) is a cause of myocardial infarction without obstructive coronary artery disease (MINOCA). It is determined by a coronary artery wall layers separation, which occurs regardless of traumatic or iatrogenic injuries. Even if it is often a missed diagnosis, its incidence is growing along with the improvement of intracoronary imaging techniques that allow for better detection. The main angiographical classification distinguishes three different forms, with slightly different prognoses at long-term follow up. SCAD is a recurrent condition, severely hampering the life quality of affected patients. The predominantly young age of patients with SCAD and the high prevalence of females among them have made the topic increasingly important, especially regarding therapeutic strategies. According to the data, the most recommended treatment is conservative, based on the use of antiplatelet agents and supportive anti-ischemic therapy. However, there are conflicting opinions concerning the need for dual antiplatelet therapy and its duration. In the case of invasive treatment, the choice between percutaneous coronary intervention and coronary artery bypass graft depends on the patient's clinical stability and the interested vessel. The purpose of the current review is to revise the pathophysiological mechanisms underlying SCAD and the current knowledge of its treatment.

Spontaneous Coronary Artery Dissection (SCAD) from an Interventionalist Perspective.

PURPOSE OF REVIEW: Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndrome (ACS), particularly among women < 50 years of age. Here, we aim to review the pathogenesis of SCAD, discuss SCAD as an initial manifestation of systemic arterial disease, and highlight invasive strategies as well as unique challenges in the care of women with SCAD. RECENT FINDINGS: A paradigm shift has occurred in the care of SCAD patients in the past decade as recommendations for conservative management have become widespread. Invasive interventions are reserved for patients with hemodynamic compromise or active ischemia due to increased periprocedural complications and failure rates. Certain patient populations have been identified for larger territory infarcts and proximal disease including patients with known connective tissue disease, premenopausal women, and patients with pregnancy-associated SCAD (P-SCAD). Current recommended management of SCAD is conservative. Despite a growing awareness of SCAD and its known association with systemic arteriopathies in women, evidence-based data remains scarce. Future studies focused on identifying genetic factors, optimal medical therapy after SCAD, and techniques to minimize interventional complications are needed.

Twenty-five-year trends in incidence, angiographic appearance, and management of spontaneous coronary artery dissection.

BACKGROUND: Spontaneous coronary artery dissection (SCAD) has been described as an infrequent cause of acute coronary syndrome (ACS). Knowledge about the disease is still limited and SCAD might still be underdiagnosed. OBJECTIVES: Trends in incidence, presentation, angiographic appearance, management, and outcomes of SCAD over 25 years were analyzed. METHODS: Patients with SCAD between 1997 and 2021 at the University Hospital Zurich, Switzerland, were included. Incidences were assessed as total numbers and proportions of ACS cases. Clinical data were collected from medical records and angiographic findings were reviewed. Major adverse cardiac events (MACE) were defined as the composite of all-cause death, cardiac arrest, SCAD recurrence or progression, other myocardial infarction, and stroke. RESULTS: One hundred fifty-six SCAD cases were included in this study. The incidence increased significantly in total (p < 0.001) and relative to ACS cases (p < 0.001). This was based on an increase of shorter lesions (p = 0.004), SCAD type 2 (p < 0.001), and lesions in side branches (p = 0.014), whereas lesions in the left main coronary artery and proximal segments were decreasing (p-values 0.029 and < 0.001, respectively). There was an increase in conservative therapy (p < 0.001). The rate of MACE (24%) was stable, however, there was a reduced proportion of patients with a need for intensive care treatment (p = 0.017). CONCLUSIONS: SCAD represents an important entity of ACS that still might be underappreciated. The increasing incidence of SCAD is likely based on better awareness and familiarity with the disease. A lower need for intensive care treatment suggests positive effects of the increasing implementation of conservative management.

Diagnosis and Management of Congenital Coronary Artery Fistulas in Infants and Children.

PURPOSE OF REVIEW: Coronary artery fistulas (CAFs) are rare coronary anomalies that most often occur as congenital malformations in children. Although most children with CAFs are asymptomatic at the time of diagnosis, some present with symptoms of congestive heart failure in the setting of large left-to-right shunts. Others may develop additional complications including coronary artery ectasia and coronary thrombosis. Surgical and transcatheter closure techniques have been previously described. This review presents the classifications of CAFs in children and the short and long-term outcomes of CAF closure in children in the reported literature. We also summarize previously-reported angiographic findings and post-treatment remodeling characteristics in pediatric patients. RECENT FINDINGS: With advancements in cross-sectional imaging technologies, anatomic delineation of CAFs via these modalities has become crucial in procedural planning. Recent reports of surgical and transcatheter closure of CAFs in children have reported good procedural success and low rates of short-term morbidity and mortality. Distal-type CAFs have elevated risk for long-term sequelae post-closure compared to proximal-type CAFs. A recent report of a multi-institutional cohort also describes post-closure remodeling classifications which may predict long-term outcomes in these patients as well as guide individualized anticoagulation management. Invasive closure of significant CAFs via surgical or transcatheter techniques is feasible and safe in most children with good short and intermediate-term outcomes. However, close clinical and imaging follow-up is required to monitor for late complications even after successful closure. Antiplatelet and anticoagulation regimens remain important aspects of post-closure management, but the necessary intensity and duration of such therapy remains unknown.

[French National registry of spontaneous coronary artery dissections : ''DISCO registry'']. / Registre national des dissections coronaires spontanées : le registre DISCO.

AIM: Spontaneous coronary artery dissection (SCAD) is a form of acute coronary syndrome (ACS). The aim of this registry is to assess the clinical and angiographic features of SCAD, to describe the therapeutic management and prognosis, and to identify links with other vascular diseases. METHOD: From 2016 to 2018, 424 patients with a diagnosis of SCAD were included prospectively and retrospectively in 51 French cardiology centres. RESULTS: 373 patients with confirmed SCAD were included. The mean age was 51.5±10.3 years with 90.6% women. 54.7% of patients had <2 cardiovascular risk factors. ACS occurred in 96.2% of patients. 84.2% of patients were managed conservatively, 15.5% interventionally and 0.3% surgically. At 1-year follow-up, recurrence of SCAD occurred in 3.3%. No deaths occurred. The association with fibro-muscular dysplasia was found in 45% of cases and genetic analysis confirmed a strong relationship between the occurrence of SCAD and gene variations at the PHACTR1 locus. CONCLUSION: The DISCO registry is the largest European cohort of SCAD. It confirms that this disease mainly affects young women with few cardiovascular risk factors, and that there is a strong association with the presence of fibromuscular dysplasia (45%). Conservative management should be preferred, with a favourable prognosis (no deaths at 1 year; recurrence rate of 3.3%).

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.

Anomalous right coronary artery originating from the opposite sinus of Valsalva: Fractional flow reserve and intravascular ultrasound-guided management.

There remains significant controversy in the risk stratification and management of patients with anomalous right coronary artery originating from the opposite sinus (R-ACAOS). We present the case of a patient with an inferior ST-elevation myocardial infarction, found to have R-ACAOS and severe atherosclerotic right coronary artery disease, treated with fractional flow reserve and intravascular ultrasound-guided percutaneous coronary intervention.

Coronary Artery Anomalies - State of the Art Review.

Coronary artery anomalies (CAAs) comprise a wide spectrum of anatomic entities, with diverse clinical phenotypes. We present a case of an anomalous right coronary artery arising from the left aortic sinus with an interarterial course, a potentially fatal condition that can precipitate ischemia and sudden cardiac death. CAAs are increasingly detected in adults, mostly as incidental findings in the course of cardiac evaluation. This is due to the expanding use of invasive and noninvasive cardiac imaging, usually in the work-up for possible CAD. The prognostic implications of CAAs in this group of patients remain unclear. In AAOCA patients, appropriate work-up with anatomical and functional imaging should be performed for risk stratification. An individualized approach to management should be adopted, considering symptoms, age, sporting activities and the presence of high-risk anatomical features and physiologic consequences (such as ischemia, myocardial fibrosis, or cardiac arrhythmias) detected on multimodality imaging or other functional cardiac investigations. This comprehensive and up to date review seeks to crystallize current data in the recent literature, and proposes a clinical management algorithm for the clinician faced with the conundrum of managing such conditions.

Congenital coronary artery anomalies in sports medicine. Why to know them.

The anomalous origin of a coronary artery (AOCA) is a challenging topic, due to its rarity, the complexity of the pathophysiological aspects, the clinical presentation (often silent), the difficulty of diagnosis, and the potential risk of causing acute cardiovascular events up to sudden cardiac death, particularly when triggered by heavy physical exercise or sport practice. Increasing interest in sport medical literature is being given to this topic. This paper reviews current knowledge of AOCAs in the specific context of the athletic setting addressing epidemiological and pathophysiological aspects, diagnostic work-up, sports participation, individual risk assessment, therapeutic options, and return to play decision after surgery.

Balloon assisted hematoma fenestration in spontaneous coronary artery dissection. Case presentation and literature review.

The management of spontaneous coronary artery dissection (SCAD) is lacking randomized data. This also holds for SCAD with ST-segment elevation myocardial infarction where stenting has been used to restore coronary flow. This approach can be associated with many drawbacks. Therefore, we present an alternative approach to stenting when coronary flow cannot be restored using cutting balloons alone.

Narrative Review of Anomalous Origin of Coronary Arteries: Pathophysiology, Management, and Treatment.

Coronary artery anomalies (CAA) are a diverse group of congenital anomalies and are the second most common cause of sudden cardiac death in the young population after Hypertrophic Cardiomyopathy (HCM). Symptoms range from chest pain, syncope, or sudden cardiac arrest to completely asymptomatic. The prevalence of congenital coronary artery anomalies in the general population is estimated to be between 1% and 2%. CAA often gets underdiagnosed due to the lack of knowledge of the disease process. Approximately 5% of patients with acute myocardial infarction do not have atherosclerotic coronary artery disease or luminal narrowing due to other causes. Congenital coronary artery anomalies account for 50-60% of this 5% of patients. Most patients are asymptomatic for most of their lives, and chest pain is the most common symptom in symptomatic patients when referred for coronary angiography, typically when the diagnosis is typically made. The malignant coronary artery is a rare presentation of a coronary anomaly when associated with atherosclerotic coronary artery disease or valvular heart disease. Patients with symptoms of an abnormal coronary artery origin will receive medical treatment/observation, exercise restriction, coronary angioplasty with stent deployment, or surgical repair.

Pregnancy-related spontaneous coronary artery dissection: a rare cause of acute coronary syndrome in the third trimester.

BACKGROUND: Spontaneous Coronary Artery Dissection (SCAD) is a rare cause of myocardial infarction and sudden cardiac death that is mostly seen in younger patients without significant cardiac risk factors. The mechanism by which SCAD causes an acute coronary event is related to the compromise of the coronary artery lumen as a result of hematoma within the vessel wall. In comparison to their non-pregnant counterparts, when SCAD is associated with pregnancy, it has been associated with an increased risk of life-threatening arrhythmias, cardiogenic shock, and death. The underlying mechanism behind SCAD is not yet fully understood, and despite the condition's high mortality rate, it remains underdiagnosed. CASE PRESENTATION: Our case features a 38-year-old woman at 29 weeks of gestation presenting with chest pain that persisted despite initial management. Coronary angiography revealed a Type 2a spontaneous dissection of the left anterior descending artery. Given the risks of percutaneous coronary intervention in SCAD management and overall clinical stability, the patient was treated with conservative management. CONCLUSION: SCADs are a rare cause of acute coronary syndrome that can be found in patients without any prior cardiac risk factors. It is important to have a high index of suspicion when diagnosing SCADs given, they can cause life-threatening arrhythmias, cardiogenic shock, and death. This case highlights considerations that must be taken into account when treating P-SCAD, as opposed to SCAD in the postpartum period.

Management of spontaneous coronary artery dissection: Trends over time.

BACKGROUND: Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndrome. Guidance regarding the optimal management of patients with SCAD has been published over the past 10 years, but the impact on clinical practice has not been evaluated. The present study aims to examine if approaches to invasive management, medical therapy, and vascular imaging have changed over time. METHODS: This is a retrospective cohort study of 157 patients treated for SCAD between 2005 and 2019 at an academic health system in Philadelphia, Pennsylvania. We aimed to examine change in management over time, including rates of coronary revascularization, discharge medications, and vascular imaging. RESULTS: Conservative management of SCAD increased over time from 35% before 2013 to 89% in 2019, p < 0.001. Revascularization was associated with younger age, pregnancy-associated SCAD, and lesions of the left main artery, left anterior descending artery, and multiple vessels, p < 0.05 for all. Partial imaging for extracoronary vascular abnormalities ranged from 33% before 2013 to 71% in 2018, p = 0.146. The rate of comprehensive vascular imaging (cross-sectional head to pelvis imaging) remained low in all time categories (10-18%) and did not change over time. Patients who underwent comprehensive imaging were more likely to be diagnosed with fibromuscular dysplasia (FMD) compared to those with partial imaging (63% vs 15%, p < 0.001). CONCLUSION: Management of spontaneous coronary artery dissection has changed over time. More patients are being managed conservatively and undergo screening for extracoronary vascular abnormalities such as FMD. Future efforts should focus on improving rates of comprehensive vascular screening.

Decision making in anomalous aortic origin of a coronary artery.

INTRODUCTION: There are many uncertainties surrounding anomalous aortic origin of a coronary artery (AAOCA) including the pathophysiology of sudden cardiac death, how to best risk stratify patients, how to best evaluate patients, who would benefit from exercise restriction, who should undergo surgical intervention, and which operation to perform. AREAS COVERED: The goal of this review is to provide a comprehensive but succinct overview of AAOCA to help clinicians with the difficult task of navigating optimal evaluation and treatment of an individual patient with AAOCA. EXPERT OPINION: Beginning in year 2012, some of our authors proposed an integrated, multi-disciplinary working group which has become the standard management strategy for patients diagnosed with AAOCA. A multi-disciplinary team with a focus on shared decision-making with the patients/families is likely necessary to optimize outcomes. Long-term follow-up and research are needed to improve our understanding of AAOCA.

Clinical and angiographic features of SCAD type 4.

INTRODUCTION: The angiographic type 4 in SCAD is described as a total occlusion of the coronary artery and its management may differ according to its clinical presentation. We previously have observed that these patients present a low incidence of adverse events. Our objective was to describe clinical and angiographic characteristics of this condition, according to its initial management in the DISCO registry. METHODS: We conducted an observational study of consecutive SCAD patients from 26 centres of Italy and Spain (DISCO registry). Angiotype 4 SCAD cases were selected and classified according to the initial treatment chosen: conservative management vs. percutaneous coronary intervention (PCI). Clinical and angiographic characteristics were compared. RESULTS: We recruited 81 (mean age 52.6 ± 11 years) patients with SCAD angiotype 4 out of 302 patients of the DISCO registry. Thirty-eight (46.9%) patients received conservative management and 43 (53.1%) received PCI. Nearly all patients undergoing PCI had ST-segment elevation (93% vs 47.4%, p < 0.0001), the left anterior descending artery (LAD) was more commonly involved (67.4% vs. 42.1%, p = 0.006), and they had more frequent proximal segment involvement (25.6% vs 2.7%, p = 0.004) and longer lesions (46.5 ± 23.2 mm vs 26.4 ± 18.8 mm, p = 0.017). On the other hand, non-ST-segment elevation myocardial infarction (52.6% vs 2.3%, p = 0.001) and isolated involvement of secondary branches (55.3 vs 4.7, p < 0.0001) were more common in the conservative management group. CONCLUSIONS: Patients with SCAD angiotype 4 who underwent PCI had a higher frequency of STEMI and involvement of proximal and longer coronary segments, particularly affecting the left anterior descending artery. NSTEMI and isolated involvement of secondary branches were more frequently found in those managed conservatively.