Spontaneous Coronary Artery Dissection Patients Research Articles

Abstract 12376: Coronary Artery Bypass Graft Surgery in Spontaneous Coronary Artery Dissection

Introduction: Spontaneous coronary artery dissection (SCAD) is a nonatherosclerotic cause of acute coronary syndrome. While most patients are managed conservatively, some patients may require percutaneous or surgical revascularization. Limited data exist on SCAD patients referred for coronary artery bypass graft (CABG) surgery. Hypothesis: Examine the clinical characteristics, management, and outcomes of SCAD patients referred for CABG. Methods: Retrospective study of patients enrolled in the Mayo Clinic SCAD Registry between 2010-2020 who underwent CABG during the initial management of acute SCAD episode. Results: Among the 81 patients with SCAD referred for CABG, 3 (4%) were men, mean age was 40.6 +/- 8.4 years; the majority were white (91%), and 24 (30%) had pregnancy-associated SCAD (P-SCAD). The most common presenting symptom was chest pain 80 (99%); 57 (70%) presented with ST elevation myocardial infarction (STEMI), and 21 (26%) presented with non-ST elevation MI. Multivessel dissection occurred in 40 (49%), left anterior descending artery (LAD) involvement occurred in 66 (81%) and left main (LM) in 32 (40%). Left ventricular ejection fraction was <50% in 40 patients (49%) at time of initial SCAD. The most common reasons for CABG were the presence of high-risk anatomy (56%), hemodynamic instability/active ischemia (7%), failed PCI (9%), and other/unknown (12%). Most patients received venous plus arterial bypass grafts 42 (52%), saphenous vein grafts in 20 (25%), and left internal mammary artery in 17 (21%). Screening for underlying arteriopathy was performed in 50 (62%) of whom 29 (36%) had fibromuscular dysplasia. Recurrent SCAD occurred in 10 (12%) after an average of 6.2 +/- 5 years after initial SCAD. Of those with recurrent SCAD, none required repeat surgical revascularization and 6 required percutaneous intervention (PCI). There were no deaths. Conclusions: Patients undergoing CABG for SCAD commonly present with STEMI, LM, or multivessel dissection, and failed percutaneous intervention attempts. In addition, CABG for P-SCAD was more frequent than expected and may relate to the increased prevalence of multivessel dissection. CABG was associated with low mortality, but recurrent SCAD may still occur.

Spontaneous Coronary Artery Dissection: An Underdiagnosed Clinical Entity-A Primer for Cardiac Imagers.

Spontaneous coronary artery dissection (SCAD) is a nonatherosclerotic cause of myocardial infarction in young and middle-aged women that has gained increasing awareness in recent years. Its diagnosis presents a challenge. Invasive coronary angiography is the primary imaging modality for diagnosing SCAD; however, it carries risk in these patients, who have an increased predisposition to complications. Advances in CT technology enable robust noninvasive evaluation of the coronary arteries at low radiation doses and have been increasingly utilized for the diagnosis or resolution of SCAD, in hemodynamically stable patients or when diagnosis of SCAD is uncertain at invasive angiography, particularly in proximal vessels. However, criteria for the diagnosis of SCAD with use of coronary CT angiography (CCTA) have not been currently established, and sensitivity and specificity for diagnosis have not yet been defined. The appearance of SCAD at CCTA can be subtle and can be missed, especially in distal small-caliber coronary arteries; hence utilization of other noninvasive imaging multimodalities may help solve this diagnostic challenge. Accurate and prompt diagnosis is vital, as management of SCAD differs significantly from that of traditional atherosclerotic acute coronary syndromes, with conservative management preferred for the majority of SCAD patients, and invasive treatment reserved for those with ongoing or recurrent ischemia, heart failure, or hemodynamic compromise. The goal of this review is twofold: (a) to discuss the potential role of CCTA in the diagnosis of SCAD, and (b) to discuss the role of multimodality imaging that may improve diagnostic yield, guide management, and enable subsequent surveillance. An invited commentary by Ordovas is available online. Online supplemental material is available for this article. ©RSNA, 2021.

A contemporary presentation of the incidence and management of spontaneous coronary artery dissection (SCAD) in New Zealand: an ANZACS-QI study

Abstract Background/Purpose Spontaneous coronary artery dissection (SCAD) is an underdiagnosed and poorly understood condition that frequently affects younger women without cardiovascular risk factors. In this project we aimed to describe the present landscape of SCAD in New Zealand (NZ). Methods All patients in NZ who were admitted to hospital with a diagnosis of Acute Coronary Syndrome (ACS) from July 2019 to December 2020 and underwent coronary angiography were identified from the All NZ Coronary Syndrome Quality Imporvment (ANZACS-QI) registry. Results Of 12,053 patients admitted to hospital with an ACS, 122 had SCAD (1.0%). 80% of those with SCAD were female, with mean age of 57 years, and fewer traditional cardiovascular risk factors. Non-ST elevation myocardial infarction was the most common presentation (82.0%), while 16.4% presented with ST elevation myocardial infarction. The majority of patients were managed conservatively (91.8%) while 8.2% underwent PCI. 56.6% of SCAD patients had normal LV function. Nearly 80% of patients were discharged on Acetylsalicylic acid (ASA) therapy, while 60.7% had a P2Y12 inhibitor. Beta blockers, Statins and ACEI/ARB were part of the management strategy in 62.5%, 59.8% and 42.6% of patients respectively while 47.5% had a combination of all 3. Amongst 122 patients, only 1 in-hospital death and 1 inpatient recurrent MI occurred, with 3 patients having bleeding of any kind. Conclusion In NZ, incidence of SCAD is approximately 1%. Most patients affected in NZ are female with fewer traditional cardiovascular risk factors. Management is predominantly conservative, and there is a low rate of early adverse outcomes. Funding Acknowledgement Type of funding sources: None.

Spontaneous coronary artery dissection: an Italian single centre experience.

Spontaneous coronary artery dissection (SCAD) is an emerging cause of acute myocardial infarction (AMI) in young women without a typical cardiovascular risk profile. Knowledge on SCAD is based on observational studies and is still scarce. The aim of this monocentric observational study was to evaluate the predisposing factors, clinical features and prognosis of SCAD patients. Between 2013 and 2020, 58 patients with angiographic diagnosis of SCAD were identified in our centre with an overall prevalence of 0.9% among patients admitted for AMI (58 of 6414 patients). The mean age was 54 ± 11 years and the majority were women (n = 50, 86%) with one or fewer cardiovascular risk factors (n = 35, 60%). The prevalence of Fibromuscolar Dysplasia (FMD) was 39% (7 of 18 screened patients). The rate of major adverse cardiovascular and cerebrovascular events (MACCEs) was used to assess the prognosis. Out-of-hospital cardiac arrest because of ventricular fibrillation was observed in four (7%) patients. The majority of patients (n = 51, 88%) were treated conservatively without revascularization. The in-hospital and 30-day clinical course was uneventful in most patients (n = 54, 93%) with two cardiac deaths. During a median follow-up of 12 months, there were no further deaths. The global rate of SCAD recurrence was significant (n = 8, 14%) but predictors have not been identified. Although overall survival seems good, SCAD is a potentially malignant, not rare disease, which can present with sudden cardiac death and not uncommon recurrence. Prognostic stratification and optimal management of SCAD patients remain to be defined.

Coronary Angiographic Manifestations and Outcomes in Spontaneous Coronary Artery Dissection Patients With and Without Fibromuscular Dysplasia

BackgroundFibromuscular dysplasia (FMD) is widely recognized as an important predisposing condition for spontaneous coronary artery dissection (SCAD). However, it remains unclear in SCAD patients with coexistent extracoronary FMD whether SCAD can be attributed to coronary FMD. MethodsWe retrospectively analyzed consecutive patients enrolled in our Vancouver SCAD registries between September 2009 and October 2019 who were screened for extracoronary FMD. We reviewed coronary angiograms for manifestations of coronary FMD that were previously described (ie, irregular stenosis, smooth stenosis, dilatation/ectasia, and severe tortuosity). Outcome of interest was major adverse cardiovascular event (MACE). ResultsWe included 346 SCAD patients, of these, 250 (72.3%) had extracoronary FMD. Patients with FMD were older (54.6 ± 9.5 vs 51.7 ± 9.8 years) and more likely to have prior history of myocardial infarction (7.2% vs 1.0%, P = 0.047) and stroke (4.4% vs 0%, P = 0.081) compared with non-FMD patients. On coronary angiography, severe tortuosity was more prevalent in patients with extracoronary FMD (58.4% vs 36.5%, P < 0.001). Rates of irregular stenosis, smooth stenosis, and dilatation/ectasia were numerically higher in patients with extracoronary FMD, but differences were not significantly different. The rate of MACE at median follow-up of 807 (interquartile range, 392-1096) days was not different between groups (19.6% vs 15.6%; non-FMD as a reference: hazard ratio 1.44; 95% confidence interval, 0.76-2.71, P = 0.261). ConclusionSCAD patients with extracoronary FMD were more likely to have coronary FMD manifestations on angiogram, especially severely tortuous vessels, compared with those without extracoronary FMD, with similar clinical outcomes. This may suggest that SCAD can occur at sites of pre-existent subclinical coronary FMD.

Implantable cardioverter-defibrillator in patients with spontaneous coronary artery dissection presenting with sudden cardiac arrest.

The role of secondary prevention implantable cardioverter-defibrillator (ICD) remains uncertain in spontaneous coronary artery dissection (SCAD) patients presenting with sudden cardiac arrest (SCA). We aimed at assessing the outcomes following SCA and the role of ICD therapy in SCAD. The meta-analysis was performed using a meta-package for R version 4.0/RStudio version 1.2 and the Freeman-Tukey double arcsine method to establish the variance of raw proportions. Outcomes measured included-(1) incidence of ICD implantation, (2) appropriate and inappropriate ICD therapy, (3) recurrence of SCAD and SCA, and (4) all-cause mortality. Five studies, including 139 SCAD patients with SCA met study inclusion criteria. The mean age was 47.3 ± 12.8 years, mean left ventricular ejection fraction 43.8 ± 10.8%, 88% were female (12% had pregnancy-associated SCAD. Causes of SCA included ventricular arrhythmia (97.9%, n = 136) and pulseless electrical activity (2.1%, n = 3). Overall, 20% patients (95% confidence interval [CI]: 7.1%-36.6%, I2 = 68%) received ICD, of which 1.2% (95% CI: 0%-15.8%, I2 = 0%) and 1% (95% CI: 0%-15.3%, I2 = 0%) patients received appropriate and inappropriate ICD therapies, respectively, during follow-up period (4.1 ± 3.3 years). Incidence of recurrent SCAD was 9% (95% CI: 2.85%-17.5%, I2 = 25%), and recurrent SCA was 3.85% patients (95% CI: 0.65%-8.7%, I2 = 0%; one patient with appropriate ICD therapy). The pooled incidence of all-cause mortality was 6.2% (95% CI: 0.6%-15.1%, I2 = 44%). Although ICD therapy is beneficial in patients (all comers) presenting with cardiac arrest; the risk-benefit ratio of secondary prevention ICD arrest remains unclear. Patient-centered shared decision-making and risk-benefit ratio assessment should be performed before consideration for ICD implantation.

Antiplatelet therapy in patients with conservatively managed spontaneous coronary artery dissection from the multicentre DISCO registry.

The role of antiplatelet therapy in patients with spontaneous coronary artery dissection (SCAD) undergoing initial conservative management is still a matter of debate, with theoretical arguments in favour and against its use. The aims of this article are to assess the use of antiplatelet drugs in medically treated SCAD patients and to investigate the relationship between single (SAPT) and dual (DAPT) antiplatelet regimens and 1-year patient outcomes. We investigated the 1-year outcome of patients with SCAD managed with initial conservative treatment included in the DIssezioni Spontanee COronariche (DISCO) multicentre international registry. Patients were divided into two groups according to SAPT or DAPT prescription. Primary endpoint was 12-month incidence of major adverse cardiovascular events (MACE) defined as the composite of all-cause death, non-fatal myocardial infarction (MI), and any unplanned percutaneous coronary intervention (PCI). Out of 314 patients included in the DISCO registry, we investigated 199 patients in whom SCAD was managed conservatively. Most patients were female (89%), presented with acute coronary syndrome (92%) and mean age was 52.3 ± 9.3 years. Sixty-seven (33.7%) were given SAPT whereas 132 (66.3%) with DAPT. Aspirin plus either clopidogrel or ticagrelor were prescribed in 62.9% and 36.4% of DAPT patients, respectively. Overall, a 14.6% MACE rate was observed at 12 months of follow-up. Patients treated with DAPT had a significantly higher MACE rate than those with SAPT [18.9% vs. 6.0% hazard ratios (HR) 2.62; 95% confidence intervals (CI) 1.22-5.61; P = 0.013], driven by an early excess of non-fatal MI or unplanned PCI. At multiple regression analysis, type 2a SCAD (OR: 3.69; 95% CI 1.41-9.61; P = 0.007) and DAPT regimen (OR: 4.54; 95% CI 1.31-14.28; P = 0.016) resulted independently associated with a higher risk of 12-month MACE. In this European registry, most patients with SCAD undergoing initial conservative management received DAPT. Yet, at 1-year follow-up, DAPT, as compared with SAPT, was independently associated with a higher rate of adverse cardiovascular events (ClinicalTrial.gov id: NCT04415762).

Preliminary Support for Group Cognitive Behavioral Therapy (CBT) to Reduce Psychological Distress in Patients with Spontaneous Coronary Artery Dissection (SCAD)

Spontaneous coronary artery dissection (SCAD) can occur as an atypical cause of myocardial infarction. Preliminary evidence suggests that SCAD patients experience high rates of post-event psychological distress. It is unknown whether psychosocial interventions may reduce the distress burden. Seven SCAD patients (mean age = 53.3 years) completed a CBT-based support group. All seven participants completed measures for anxiety, depression, and cardiac-related quality of life at baseline and post-intervention, and five participants completed measures at 3-month follow-up. Six of 7 participants scored above the clinical threshold on a measure of anxiety at baseline and posttreatment. At follow-up, 3 of 5 participants scored below the clinical threshold. For depression, 3 of 7 reported elevated depressive symptoms at baseline. By follow-up, 1 of 5 endorsed elevated depressive symptoms. This is the first known psychosocial intervention study of patients with SCAD. Anxiety symptoms improved for most patients by follow-up with some patients having improved depressive symptoms. Although the sample size is limited, this pilot study suggests a potential benefit of group psychosocial interventions for SCAD survivors.

Recovering from spontaneous coronary artery dissection: Patient-reported challenges and rehabilitative intervention needs.

Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndrome that disproportionally affects younger women. The underlying etiology is incompletely understood, postmorbid psychological distress is high, and treatment plans are predominantly based on clinician experience. There remains uncertainty on how to adequately address the needs of patients with SCAD as part of secondary prevention. As a Define and Refine phase of the ORBIT model (Phase 1), this study investigated SCAD patients' challenges and rehabilitative intervention needs using a qualitative research design. Patients with SCAD were purposively recruited to participate in structured interviews that were analyzed using inductive thematic coding techniques. Patients with SCAD (n = 15; 86.7% female; mean age = 47.5 years; data saturation reached with patient sample) expressed challenges in (a) navigating uncertainty associated with the disease; (b) living with anxiety; (c) reconciling pre and post-SCAD identities; (d) accurately identifying symptoms and experiencing a sense of isolation in recovery due to gender and young age; and (e) managing changing family dynamics and family members' stress. Intervention needs included (a) addressing unique demographic and cardiovascular profiles when designing programs for cardiac rehabilitation; (b) providing more psychological and peer support resources to address anxiety and sense of isolation; (c) disseminating information on rapidly evolving SCAD research; and (d) acknowledging and providing support to the family system. The results signal curricula to be included in tailored SCAD programming and underscore the need for further study and dissemination of optimal secondary preventative care for this patient population. (PsycInfo Database Record (c) 2021 APA, all rights reserved).

Physical Activity and Exercise Patterns After Spontaneous Coronary Artery Dissection: Insights From a Large Multinational Registry.

Objective: The objective of the study was to assess the physical activity (PA) and exercise patterns among participants in a large multinational spontaneous coronary artery dissection (SCAD) registry. Patients and Methods: Participants with SCAD enrolled from March 2011 to November 2019 completed surveys including details regarding PA and exercise habits prior to SCAD, and PA counseling received from their provider after SCAD. Demographics and clinical characteristics were collected by electronic record review. Exercise prescribed to patients after SCAD was categorized according to exercise components: type, intensity, frequency, time/session, and extreme environmental conditions.Results: We included 950 participants; mean ± age was 46.8 ± 9.5 years old at the time of first SCAD; most (96.3%) were women and (77.0%) attended ≥1 cardiac rehabilitation session. Hyperlipidemia (34.3%), hypertension (32.8%), and elevated body weight (overweight = 27.0%; obesity = 20.0%) were the most common comorbidities. Prior to SCAD, 48.5% performed aerobic exercise ≥3 times/week, and only 32.0% performed strength-building exercise regularly. PA counseling details after SCAD in 299/950 participants showed that most (93.3%) patients received some form of counseling including exercise prescription (EXP), non-specific recommendations, and discouraged from any exercise. Limits regarding exercise type and intensity were the most common advice among participants who received EXP.Conclusion: Insights from our study suggest that only 48% of the patients performed some aerobic exercise three or more times per week, and 32.0% performed strength-building exercises, which suggest that most of them may not be as active as assumed. Furthermore, 70% of the SCAD patients have ≥1 cardiovascular risk factors. We suggest guiding patients based on individual assessment, taking into consideration baseline PA habits, treatment, and risk factors. SCAD-tailored PA guidelines are needed for optimal EXP without compromising patient safety.

Hospital Readmission in Patients With Spontaneous Coronary Artery Dissection

Spontaneous coronary artery dissection (SCAD) can present with various clinical symptoms, including chest pain, syncope, and sudden cardiac death, particularly in those without atherosclerotic risk factors. In this contemporary analysis, we aimed to identify the causes and predictors of 30-day hospital readmission in SCAD patients. We utilized the latest Nationwide Readmissions Database from 2016 - 2017 to identify patients with a primary discharge diagnosis of SCAD. The primary outcome was 30-day readmission. Among 795 patients admitted with a principal discharge diagnosis of SCAD, 85 (11.3%) were readmitted within 30 days of discharge from index admission (69.8% women, mean age of 54.3 ± 0.8). More than half of the readmissions (57%) were cardiac-related readmissions. Common cardiac causes for 30-day hospital readmission were acute coronary syndrome (27.3%), chest pain/unspecified angina (24.6%), heart failure (17.5%), and recurrent SCAD (8.3%). In conclusion, we found that following hospitalization for SCAD, almost one-tenth of patients were readmitted within 30 days, largely due to cardiac cause . Risk stratifying patients with SCAD, identifying high-risk features or atypical phenotypes of SCAD, and using appropriate management strategies may prevent hospital readmissions and reduce healthcare-related costs. Further studies are warranted to confirm these causes of readmission in SCAD patients.

Risks and benefits of percutaneous coronary intervention in spontaneous coronary artery dissection

ObjectiveTo investigate percutaneous coronary intervention (PCI) practice in an international cohort of patients with spontaneous coronary artery dissection (SCAD). To explore factors associated with complications and study angiographic and longer...

Outcomes of Percutaneous Coronary Intervention in Patients with Spontaneous Coronary Artery Dissection.

Objectives To compare outcomes of percutaneous coronary intervention (PCI) in spontaneous coronary artery dissection (SCAD) patients versus conservative therapy. Background SCAD is an important cause of myocardial infarction (MI) in young-to-middle-aged women. Percutaneous coronary intervention (PCI) is often pursued, but outcomes compared to conservative therapy are unclear. Methods 403 nonatherosclerotic SCAD patients were enrolled between 2011 and 2017 and prospectively followed up in our Vancouver General Hospital registries. Detailed baseline, hospital, PCI, and outcomes were recorded. We explored the outcomes of SCAD patients who underwent PCI during their initial presentation. Results PCI was performed in 75 patients, the average age was 48.9 ± 10.1 yrs, and 94.7% were women. All presented with MI; 50.7% STEMI, 49.3% NSTEMI, and 13.3% had VT/VF. PCI was successful in 34.7%, partially successful in 37.3%, and unsuccessful in 28.0%. Stents were deployed in 73.3%, 16.0% had balloon angioplasty alone, 10.7% had wiring attempts only, and 5.3% required bailout surgery. Major adverse cardiovascular event rates (MACE) were significantly higher with the PCI group in hospital (29.3% versus 2.8%, p < 0.001), and at median follow-up of 3.7 yrs (58.7% versus 22.6% (p < 0.001) compared to the non-PCI group. Conclusion PCI in SCAD patients was associated with high failure rate and MACE in hospital and at long-term follow-up. These findings support the recommendation of conservative therapy as first-line management unless high-risk features are present.

Imaging of Spontaneous Coronary Artery Dissection and Counseling Patients of Reproductive Age

This review highlights issues relevant to women of reproductive age with a history of spontaneous coronary artery dissection (SCAD). Current topics regarding the care of SCAD patients include multimodality imaging, pregnancy, lactation, SCAD recurrence, reproductive counseling, migraines, cardiac rehabilitation, and mental health. While no single disease-causing gene for SCAD has been discovered, recent genome-wide association studies show promise for future understanding of underlying mechanisms and risk for SCAD. Patients with history of SCAD require dedicated, multidisciplinary care, and women of reproductive age necessitate specific discussion regarding pregnancy, contraception, and menses. Imaging to detect fibromuscular dysplasia and other arteriopathies is recommended in all patients. Pregnancy after SCAD is discouraged, although counseling must be tailored. Systemic exogenous hormones have not been shown to definitively increase the risk of SCAD, but nonhormonal treatment approaches are preferred. Triptan therapy should be discouraged in SCAD patients with migraines, and more research is needed to understand the safety of newer agents. Cardiac rehabilitation after SCAD is safe and encouraged. All SCAD survivors should be screened for post-traumatic stress disorder, depression, and anxiety regardless of time from initial event.

Machine learning and deep learning to predict mortality in patients with spontaneous coronary artery dissection

Machine learning (ML) and deep learning (DL) can successfully predict high prevalence events in very large databases (big data), but the value of this methodology for risk prediction in smaller cohorts with uncommon diseases and infrequent events is uncertain. The clinical course of spontaneous coronary artery dissection (SCAD) is variable, and no reliable methods are available to predict mortality. Based on the hypothesis that machine learning (ML) and deep learning (DL) techniques could enhance the identification of patients at risk, we applied a deep neural network to information available in electronic health records (EHR) to predict in-hospital mortality in patients with SCAD. We extracted patient data from the EHR of an extensive urban health system and applied several ML and DL models using candidate clinical variables potentially associated with mortality. We partitioned the data into training and evaluation sets with cross-validation. We estimated model performance based on the area under the receiver-operator characteristics curve (AUC) and balanced accuracy. As sensitivity analyses, we examined results limited to cases with complete clinical information available. We identified 375 SCAD patients of which mortality during the index hospitalization was 11.5%. The best-performing DL algorithm identified in-hospital mortality with AUC 0.98 (95% CI 0.97–0.99), compared to other ML models (P < 0.0001). For prediction of mortality using ML models in patients with SCAD, the AUC ranged from 0.50 with the random forest method (95% CI 0.41–0.58) to 0.95 with the AdaBoost model (95% CI 0.93–0.96), with intermediate performance using logistic regression, decision tree, support vector machine, K-nearest neighbors, and extreme gradient boosting methods. A deep neural network model was associated with higher predictive accuracy and discriminative power than logistic regression or ML models for identification of patients with ACS due to SCAD prone to early mortality.

Clinical implications of arterial hypertension in patients with spontaneous coronary artery dissection.

Spontaneous coronary artery dissection (SCAD) is a rare but increasingly recognized cause of acute coronary syndrome. Many patients with SCAD have associated coronary risk factors. However, the implications of arterial hypertension in SCAD patients remain unknown. This study sought to assess the clinical implications of arterial hypertension in a nationwide cohort of patients with SCAD. The Spanish SCAD registry (NCT03607981) prospectively enrolled 318 consecutive patients. All coronary angiograms were centrally analyzed to confirm the diagnosis of SCAD. Patients were classified according to the presence of arterial hypertension. One-hundred eighteen patients (37%) had a diagnosis of arterial hypertension. Hypertensive SCAD patients were older (60 ± 12 vs. 51 ± 9 years old) and had more frequently dyslipidemia (56 vs. 23%) and diabetes (9 vs. 3%) but were less frequently smokers (15 vs. 35%) than normotensive SCAD patients (all P < 0.05). Most patients in both groups were female (90 vs. 87%, NS) and female patients with hypertension were more frequently postmenopausal (70 vs. 47%, P < 0.05). Hypertensive SCAD patients had more severe lesions and more frequently multivessel involvement (15 vs. 7%, P < 0.05) and coronary ectasia (19 vs. 7%, P < 0.05) but showed a similar prevalence of coronary tortuosity (34 vs. 26%, NS). Revascularization requirement was similar in both groups (17 vs. 26%, NS) but procedural success was significantly lower (65 vs. 88%, P < 0.05) and procedural-related complications more frequent (65 vs. 41%, P < 0.05) in SCAD patients with hypertension. Patients with SCAD and hypertension are older, more frequently postmenopausal and have more coronary risk factors than normotensive SCAD patients. During revascularization SCAD patients with hypertension obtain poorer results and have a higher risk of procedural-related complications (NCT03607981).

PREVALENCE AND NATURE OF EXTRA-CORONARY ARTERIAL ABNORMALITIES IN PATIENTS WITH SPONTANEOUS CORONARY ARTERY DISSECTION VS. CONTROLS

Abstract Objective: Spontaneous Coronary Artery Dissection (SCAD) has been associated with a high prevalence of Fibromuscular Dysplasia (FMD) and other extra-coronary arterial abnormalities. However, the prevalence, severity and clinical relevance of these abnormalities is still unclear. The objectives of the current study were: (i) to assess extra-coronary arterial abnormalities by MRA in patients with SCAD compared to controls; (ii) to compare the performance of MRA vs. CTA. Design and method: All images were jointly analysed by a vascular medicine specialist and a radiologist blinded for subject status and characteristics. In addition to qualitative analysis of images, arterial tortuosity was assessed by number of angles &gt; 45% and tortuosity index in the main vascular beds. Results: MRA from brain to pelvis was available for 166 patients with SCAD (97% women, age at diagnosis: 44.4 ± 7.8 years) and 42 healthy controls matched for age and gender. The prevalence of FMD in patients with SCAD was 30.7% vs. 9.5% in controls (p = 0.005). In the SCAD group, the most frequently affected vascular beds were renal (13.9%), cerebrovascular (12.0%) and iliac (10.8%) arteries. Only 22% of SCAD patients had FMD lesions in 2 or more arterial beds. Nine SCAD patients (5.4%) had aneurysms vs. one (2.4%) in the control group (p = 0.5). No case of arterial dissection was identified. The prevalence and degree of arterial tortuosity was similar in cases and controls, irrespective of measurement method or arterial bed. Finally, in 35 patients explored both by CTA and MRA, the performance of MRA for detection of extra-coronary arterial abnormalities was similar to that of CTA. Conclusions: Based on the current blinded analysis of a large series of fully explored patients with SCAD compared to controls: (i) extra-coronary FMD is three times more frequent in patients with SCAD than in apparently healthy controls; (ii) FMD associated with SCAD is less often multivessel and associated with less severe arterial manifestations than “classical” FMD; (iii) patients with SCAD are not prone to extra-coronary arterial tortuosity; (iv) the performance of MRA is similar to that of CTA for detection of extra-coronary abnormalities in patients with SCAD.

RARE LOSS-OF-FUNCTION MUTATIONS OF PTGIR IDENTIFIED IN FIBROMUSCULAR DYSPLASIA AND SPONTANEOUS CORONARY ARTERY DISSECTION

Objective: Fibromuscular Dysplasia (FMD) and Spontaneous Coronary Artery Dissection (SCAD) are related, non-atherosclerotic arterial diseases mainly affecting middle-aged women. Little is known about their physiopathological mechanisms. We aimed to identify rare genetic causes to elucidate molecular mechanisms implicated in FMD and SCAD. Design and method: We analyzed 29 exomes that included familial and sporadic FMD. Follow-up was conducted by targeted or Sanger sequencing (1,071 FMD and 365 SCAD patients) or lookups in exome (264 FMD) or genome sequences (488 SCAD), all independent and unrelated. We used TRAPD burden test to test for enrichment in patients compared to gnomAD controls. The biological effects of variants on receptor signaling and protein expression were characterized using transient overexpression in human cells. Results: We identified one rare loss-of-function variant (LoF) (MAFgnomAD = 0.000075) shared by two FMD sisters in the prostaglandin I2 receptor (hIP) gene (PTGIR), a key player in vascular remodeling. Follow-up in &gt;1,300 FMD patients revealed four additional LoF allele carriers and a putative enrichment in FMD (PTRAPD = 8 × 10-4), in addition to several rare missense variants. We confirmed the LoFs (Q163X and P17RfsX6) and one missense (L67P) to severely impair hIP function in vitro. Genetic analyses of PTGIR in SCAD revealed one patient who carries Q163X, one with L67P and one carrying a rare splicing mutation (c.768+1C&gt;G), but not a significant enrichment (PTRAPD = 0.12) in SCAD. Conclusions: Our study shows that rare genetic mutations in PTGIR are enriched among FMD patients and found in SCAD patients, suggesting a role for prostacyclin signaling in non-atherosclerotic stenosis and dissection.

Invasive versus conservative management in spontaneous coronary artery dissection: A meta-analysis and meta-regression study

BackgroundData regarding the best treatment for spontaneous coronary artery dissection (SCAD) are limited. The aim of the present study was to compare the clinical outcomes of conservative versus invasive treatment in SCAD patients. MethodsWe systematically searched the literature for studies evaluating the comparative efficacy and safety of invasive revascularization versus medical therapy for the treatment of SCAD from 1990 to 2020. The study endpoints were all-cause death, cardiovascular death, myocardial infarction, heart failure, SCAD recurrence and target vessel revascularization (TVR) rates. Random effect meta-analysis was performed by comparing the clinical outcomes between the two groups. A univariate meta-regression analysis was also performed. ResultsTwenty-four observational studies with 1720 patients were included. After 28 ± 14 months, a conservative approach was associated with lower TVR rate compared with invasive treatment (OR = 0.50; 95%CI 0.28–0.90; P = 0.02). No statistical difference was found regarding all-cause death (OR = 0.81; 95%CI 0.31–2.08; P = 0.66), cardiovascular death (OR = 0.89; 95%CI 0.15–5.40; P = 0.89), myocardial infarction (OR = 0.95; 95%CI 0.50–1.81; P = 0.87), heart failure (OR 0.96; 95%CI 0.41–2.22; P = 0.92) and SCAD recurrence (OR = 0.94; 95%CI 0.52–1.72; P = 0.85). The meta-regression analysis suggested that male gender, diabetes mellitus, smoking habit, prior coronary artery disease, left main coronary artery involvement, lower ejection fraction and low TIMI flow at admission were related with high overall mortality, whereas SCAD recurrence was higher among patients with fibromuscular dysplasia. ConclusionsA conservative approach was associated with similar clinical outcomes and lower TVR rates compared with an invasive strategy in SCAD patients; future prospective studies are needed to confirm these results.

Clinical outcomes by angiographic type of spontaneous coronary artery dissection.

Spontaneous coronary artery dissection (SCAD) is an increasingly diagnosed cause of myocardial infarction. Although different SCAD angiographic classifications exist, their clinical impact remains unknown. The aim of this study was to evaluate the relationship between an angiographic classification and the development of adverse clinical events during the follow-up of a large, unselected cohort of patients with SCAD. We conducted an observational study of consecutive SCAD patients from 26 centres across Italy and Spain. Cases were classified into five different angiotypes according to the latest classification endorsed by the European Society of Cardiology. The main composite endpoint included all-cause death, non-fatal myocardial infarction (MI), and any unplanned revascularisation. In total, 302 SCAD patients (mean age 51.8±19 years) were followed up for a median of 22 months (IQR 12-48). At 28 days, the composite outcome was higher for the angiotypes with a circumscribed contained intramural haematoma (2A and 3): 20.0% vs 5.4%, p<0.001 (non-fatal MI: 11.0% vs 3.5%, p=0.009; unplanned revascularisation: 11.0% vs 2.5%, p<0.001). This was sustained during follow-up (24.5% vs 9.9%, p=0.001). There were no differences in mortality (0.3% overall). The presence of an angiotype 2A or 3 was an independent predictor of a higher incidence of the composite outcome (adjusted HR 2.44, CI: 1.24-4.80, p=0.010). The SCAD angiographic classification correlates with outcome. Those presenting with an angiographically circumscribed contained intramural haematoma (angiotypes 2A and 3) showed an increased risk of short-term adverse clinical events that was maintained during follow-up.