Spontaneous Coronary Artery Dissection Without Myocardial Infarction.

Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute myocardial infarction. We examine 4 atypical presentations of SCAD. These cases highlight treatment course and outcomes for patients with SCAD who do not present with acute coronary syndrome.

Early Effects of the COVID-19 Pandemic on Patients in a Virtual Multicenter Spontaneous Coronary Artery Dissection (SCAD) Registry.

Background: The COVID-19 pandemic effects among patients with a history of spontaneous coronary artery dissection (SCAD), a cause of acute coronary syndrome associated with emotional and physical stress, are unknown. Methods: For this cross-sectional cohort study, participants of the Mayo Clinic "Virtual" Multicenter SCAD Registry were surveyed about the COVID-19 pandemic. Results: Among 1352 participants, 727 (53.8%) completed surveys between June 2, 2021 and September 29, 2021. The majority of respondents (96.7%) were female, with a mean age of 54.9 ± 9.4 years. At the time of completing the survey, which was early in the pandemic, 91 respondents (12.6%) reported having prior COVID-19 symptoms, with < 1% experiencing hospitalization (n = 4) or cardiac complications (n = 6). A total of 14% had ≥ moderate anxiety symptoms, per the General Anxiety Disorder-7 item survey, and 11.8% had ≥ moderate depressive symptoms, per the Patient Health Questionnaire-9 item. Higher stress scores on the Likert scale were correlated with pandemic-related reduction in work hours and/or pay and/or unemployment (P = 0.013), remote work and/or change of job (P < 0.001), and loss of insurance and/or medical coverage (P = 0.025). A higher anxiety level, as measured on the Likert scale, was correlated with pandemic-related remote work and/or change of job (P = 0.007) and loss of insurance and/or medical coverage (P = 0.008). Since the start of the pandemic, 54% of respondents reported having at least monthly chest pain. Chest pain and COVID symptoms were each associated with higher scores on the General Anxiety Disorder-7 item survey and the Patient Health Questionnaire-9 item. Conclusions: Early in the pandemic, COVID-19 symptoms, hospitalization, and cardiac complications were uncommon among SCAD patients. The burden of anxiety and depressive symptoms was minimal to mild, similar to that in prior reports. Likert-scale measures of stress and anxiety were higher among persons with work and/or pay reduction and/or unemployment, remote work and/or change of job, and loss of insurance and/or medical coverage. Over half of respondents reported experiencing chest pain, which was correlated with depressive and anxiety symptoms, highlighting an overarching clinical need.

Contexte: On ne connaît pas les effets de la pandémie de COVID-19 sur les personnes ayant des antécédents de dissection spontanée de l'artère coronaire (DSAC), une cause du syndrome coronarien aigu qui est une source de stress physique et émotionnel. Méthodologie: Pour les besoins de cette étude de cohorte transversale, les participants au registre « virtuel ¼ multicentrique sur la DSAC de la clinique Mayo ont été interrogés dans le cadre d'une enquête sur la pandémie de COVID-19. Résultats: Parmi les 1 352 participants au registre, 727 (53,8 %) ont répondu à l'enquête entre le 2 juin et le 29 septembre 2021. La majorité des répondants (96,7 %) étaient des femmes, et l'âge moyen était de 54,9 ± 9,4 ans. Au moment de l'enquête, réalisée au début de la pandémie, 91 répondants (12,6 %) avaient indiqué avoir déjà présenté des symptômes de COVID-19, et < 1 % avaient été hospitalisés (n = 4) ou avaient présenté des complications cardiaques (n = 6). Au total, 14 % des participants présentaient des symptômes d'anxiété à tout le moins modérés d'après le questionnaire GAD-7 (General Anxiety Disorder-7 item) et 11,8 %, des symptômes dépressifs à tout le moins modérés d'après le questionnaire PHQ-9 (Patient Health Questionnaire-9 item). Une corrélation a été établie entre un score de stress plus élevé sur une échelle de Likert et une réduction des heures de travail et/ou du salaire, une période de chômage (p = 0,013), le télétravail et/ou un changement d'emploi (p < 0,001) et la perte de l'assurance et/ou de la couverture médicale (p = 0,025) en lien avec la pandémie. Une corrélation a également été établie entre un niveau d'anxiété plus élevé mesuré sur une échelle de Likert et le télétravail et/ou un changement d'emploi (p = 0,007) et la perte de l'assurance et/ou de la couverture médicale (p = 0,008) en lien avec la pandémie. Depuis le début de la pandémie, 54 % des répondants ont indiqué ressentir une douleur thoracique au moins une fois par mois. La douleur thoracique et les symptômes de la COVID-19 ont par ailleurs été associés à des scores plus élevés aux questionnaires GAD-7 et PHQ-9. Conclusions: Au début de la pandémie, les symptômes de la COVID-19, les hospitalisations et les complications cardiaques n'étaient pas fréquents chez les patients présentant une DSAC. Le fardeau des symptômes anxieux et dépressifs allait de minime à léger, comme en faisaient état les rapports précédents. L'anxiété et le stress mesurés sur une échelle de Likert ont été plus élevés chez les personnes ayant connu une réduction des heures de travail et/ou du salaire ou une période de chômage, ayant dû recourir au télétravail et/ou changer d'emploi, ou ayant perdu leur assurance et/ou couverture médicale. Plus de la moitié des répondants ont indiqué ressentir une douleur thoracique, laquelle a été mise en corrélation avec les symptômes d'anxiété et de dépression, ce qui souligne l'existence d'un besoin clinique important.

Coronary Artery Tortuosity and Spontaneous Coronary Artery Dissection: Association With Echocardiography and Global Longitudinal Strain, Fibromuscular Dysplasia, and Outcomes.

BACKGROUND: The etiology and significance of coronary artery tortuosity (TCA) among patients with spontaneous coronary artery dissection (SCAD) are unknown. The aim of this prospective imaging cohort study was to report echocardiographic findings and evaluate whether TCA correlates with cardiac anatomy and function among patients with SCAD. Comorbidities including fibromuscular dysplasia (FMD) and outcomes were also assessed. METHODS: TCA was determined on coronary angiography performed during the diagnosis of SCAD, and cardiac structure and function were evaluated using prospective comprehensive echocardiography. RESULTS: Among 116 patients with SCAD, the mean age at echocardiography was 50.8 ± 8.8 years, a median of 10.9 months after SCAD. Sixty-two patients (53.4%) had FMD, 41 (35.3%) had histories of hypertension, and 17 (14.8%) were hypertensive during echocardiography. Most patients (n = 78 [69%]) had normal left ventricular geometry with normal median ejection fraction (61%; interquartile range, 56% to 64%) and normal global longitudinal strain (-22.2%; interquartile range, -24.0% to -19.9%). Fifteen patients (13.4%) had diastolic dysfunction that was associated with hypertension at the time of echocardiography. Patients with TCA (n = 96 [82.8%]) were older (mean age, 52.1 ± 8.0 vs 44.7 ± 9.9 years; P < .001) with a higher prevalence of FMD (59.4% vs 25%, P = .007) but a similar prevalence of hypertension (35% vs 35%, P > .99) compared with patients without TCA. Across the age range (31.5 to 66.9 years), each decade of age was associated with an approximately 0.89-unit increase in coronary tortuosity score (P < .0001). Echocardiographic parameters were not significantly different between the two groups. Median follow-up duration was 4.4 years (95% CI, 3.8 to 5.2 years). The Kaplan-Meier 3-year SCAD recurrence rate was 9.4% (95% CI, 3.7% to 14.8%). There were no deaths. CONCLUSIONS: The majority of patients with SCAD had normal or near normal echocardiographic results, including global longitudinal strain, with no differences according to TCA. However, patients with SCAD with TCA were older, with a higher prevalence of FMD.

Identification of Rare Genetic Variants in Familial Spontaneous Coronary Artery Dissection and Evidence for Shared Biological Pathways.

Rare familial spontaneous coronary artery dissection (SCAD) kindreds implicate genetic disease predisposition and provide a unique opportunity for candidate gene discovery. Whole-genome sequencing was performed in fifteen probands with non-syndromic SCAD who had a relative with SCAD, eight of whom had a second relative with extra-coronary arteriopathy. Co-segregating variants and associated genes were prioritized by quantitative variant, gene, and disease-level metrics. Curated public databases were queried for functional relationships among encoded proteins. Fifty-four heterozygous coding variants in thirteen families co-segregated with disease and fulfilled primary filters of rarity, gene variation constraint, and predicted-deleterious protein effect. Secondary filters yielded 11 prioritized candidate genes in 12 families, with high arterial tissue expression (n = 7), high-confidence protein-level interactions with genes associated with SCAD previously (n = 10), and/or previous associations with connective tissue disorders and aortopathies (n = 3) or other vascular phenotypes in mice or humans (n = 11). High-confidence associations were identified among 10 familial SCAD candidate-gene-encoded proteins. A collagen-encoding gene was identified in five families, two with distinct variants in COL4A2. Familial SCAD is genetically heterogeneous, yet perturbations of extracellular matrix, cytoskeletal, and cell-cell adhesion proteins implicate common disease-susceptibility pathways. Incomplete penetrance and variable expression suggest genetic or environmental modifiers.

Acute Myocardial Infarction Caused by Spontaneous Coronary Artery Dissection of the First Septal Perforator.

Spontaneous coronary artery dissection of the septal arteries is rare and may be overlooked on coronary angiogram. Additionally, dedicated intracoronary imaging may not be feasible due to artery size. Cardiac magnetic resonance imaging has an emerging role in diagnosis, which is critical because management changes significantly if spontaneous coronary artery dissection is diagnosed. (Level of Difficulty: Beginner.).

Genome-wide association meta-analysis of spontaneous coronary artery dissection identifies risk variants and genes related to artery integrity and tissue-mediated coagulation.

Spontaneous coronary artery dissection (SCAD) is an understudied cause of myocardial infarction primarily affecting women. It is not known to what extent SCAD is genetically distinct from other cardiovascular diseases, including atherosclerotic coronary artery disease (CAD). Here we present a genome-wide association meta-analysis (1,917 cases and 9,292 controls) identifying 16 risk loci for SCAD. Integrative functional annotations prioritized genes that are likely to be regulated in vascular smooth muscle cells and artery fibroblasts and implicated in extracellular matrix biology. One locus containing the tissue factor gene F3, which is involved in blood coagulation cascade initiation, appears to be specific for SCAD risk. Several associated variants have diametrically opposite associations with CAD, suggesting that shared biological processes contribute to both diseases, but through different mechanisms. We also infer a causal role for high blood pressure in SCAD. Our findings provide novel pathophysiological insights involving arterial integrity and tissue-mediated coagulation in SCAD and set the stage for future specific therapeutics and preventions.

Tale of two hearts: a TNNT2 hypertrophic cardiomyopathy case report.

Hypertrophic cardiomyopathy (HCM) is a heritable cardiomyopathy that is predominantly caused by pathogenic mutations in sarcomeric proteins. Here we report two individuals, a mother and her daughter, both heterozygous carriers of the same HCM-causing mutation in cardiac Troponin T (TNNT2). Despite sharing an identical pathogenic variant, the two individuals had very different manifestations of the disease. While one patient presented with sudden cardiac death, recurrent tachyarrhythmia, and findings of massive left ventricular hypertrophy, the other patient manifested with extensive abnormal myocardial delayed enhancement despite normal ventricular wall thickness and has remained relatively asymptomatic. Recognition of the marked incomplete penetrance and variable expressivity possible in a single TNNT2-positive family has potential to guide HCM patient care.

Association of Spontaneous Coronary Artery Dissection With Atrial Arrhythmias.

The co-morbidities and long-term complications of spontaneous coronary artery dissection (SCAD) are incompletely understood. This study investigated the association of atrial arrhythmias (AA), defined as atrial fibrillation and atrial flutter, with SCAD in a patient registry and population-based cohort. This observational study was performed in 2 parts. The first was a retrospective study reviewing patients diagnosed with AA in the Mayo Clinic SCAD Registry. The second was a population-based, case-control study to assess AA in patients with SCAD compared with age- and gender-matched controls. Of 1,214 patients in the Mayo Clinic SCAD Registry, 45 patients (3.7%) with SCAD were identified with an AA. A total of 8 of those patients (17.8%) had a pre-SCAD AA; 20 (44.4%) had a peri-SCAD AA; and 17 (37.8%) had a post-SCAD AA. The univariate analysis did not reveal significant associations with traditional cardiovascular risk factors. In the population-based cohort, 5 patients with SCAD (4%) and 4 controls (1%) developed an AA before the date of SCAD for each patient (odds ratio 4.5, 95% confidence interval [CI] 1.05 to 19.0, p = 0.04). A total of 5 patients with SCAD (4%) and 3 controls (1%) developed an AA in the 10 years after SCAD (hazard ratio 6.3, 95% CI 1.2 to 32.8, p = 0.03). A subgroup of patients with SCAD experienced AA before and after SCAD. Patients with a history of SCAD were more likely to develop AA in the next 10 years than were age- and gender-matched healthy controls.

Depression and Perceived Stress After Spontaneous Coronary Artery Dissection and Comparison With Other Acute Myocardial Infarction (the VIRGO Experience).

Data on depression and stress among patients with spontaneous coronary artery dissection (SCAD) are limited. Using data from the VIRGO (Variation in Recovery: Role of Gender on Outcomes of Young AMI Patients) study, which prospectively enrolled 3,572 acute myocardial infarction (AMI) patients between 18 and 55 years of age, we identified 67 SCAD cases. We compared Patient Health Questionnaire-9 (PHQ-9) and 14-item Perceived Stress Scale (PSS-14) scores obtained at baseline, 1 month, and 12 months between SCAD and AMI of all other causes. Using longitudinal linear mixed-effects analysis, we compared depression and stress scores between SCAD and other AMI, adjusting for time and selected covariates. Patients with SCAD had lower baseline PHQ-9 scores (6.1 ± 6.0 vs 7.7 ± 6.4 for other patients with AMI, p = 0.03), similar 1-month scores, and lower 12-month scores (3.2 ± 4.3 vs 4.9 ± 5.5, p = 0.004). At baseline and 1 month, patients with SCAD had similar PSS-14 scores to those of other patients with AMI. At 12 months, patients with SCAD had lower scores (18.4 ± 8.8 vs 21.5 ± 9.3 for other patients with AMI, p = 0.009). After adjustment for cardiovascular risk factors, co-morbidities, and clinical acuity, no differences in PHQ-9 or PSS-14 scores remained between SCAD and other AMI. Similar results were obtained in a subgroup analysis of only women with SCAD and other AMI. In conclusion, patients with SCAD had a relatively lower burden of depression and perceived stress than other patients with AMI, potentially because of fewer co-morbidities and favorable socioeconomic factors. However, given high depression and stress burden in both SCAD and other patients with AMI, routine screening can help identify and treat these patients.

Health status outcomes after spontaneous coronary artery dissection and comparison with other acute myocardial infarction: The VIRGO experience.

BACKGROUND: Data on health status outcomes after spontaneous coronary artery dissection (SCAD) are limited. METHODS AND FINDINGS: Using the Variation in Recovery: Role of Gender on Outcomes of Young AMI Patients (VIRGO) study we compared patients with SCAD and other acute myocardial infarction (AMI) at presentation (baseline), 1-month, and-12 months using standardized health status instruments. Among 3572 AMI patients ≤ 55 years, 67 had SCAD. SCAD patients were younger (median age (IQR) 45 (40.5-51) years vs. 48 (44-52) in other AMI, p = 0.003), more often female (92.5% vs. 66.6%), have college education (73.1% vs. 51.7%) and household income >$100,000 (43.3% vs. 17.7% (All p<0.001). SCAD patients at baseline had higher mean ± SD Short Form-12 [SF-12] physical component scores [PCS] (48.7±10.2 vs. 43.8±12.1, p<0.001) and mental component scores [MCS] (49.6±12.4 vs. 45.4±12.5, p = 0.008), and at 12-months [PCS (50.1±9.0 vs. 44.3±12.3, p<0.001) and MCS (53±10.1 vs 50.2±11.0, p = 0.045)]. The Euro-Quality of Life Scale [EQ-5D] VAS and EQ-5D index scores were similar at baseline, but higher at 12-months for SCAD (EQ-5D VAS: 82.2±10.2 vs. 72.3±21.0, p<0.001; EQ-5D index scores; 90.2±15.3 vs. 83.7±19.8, p = 0.012). SCAD patients had better baseline Seattle Angina Questionnaire [SAQ] physical limitation (88.8±20.1 vs. 81.2±25.4, p = 0.017). At 12-months SCAD patients had better physical limitation (98.0±8.5 vs. 91.4±18.8, p = 0.007), angina frequency (96.4±8.8 vs. 91.3±16.8, p = 0.018) and quality of life scores (80.7±14.7 vs 72.2±23.2, p = 0.005). Magnitude of change in health status from baseline to 12-months was not statistically different between the groups. After adjustment for time and comorbidities there remained no difference in most health status outcomes. CONCLUSIONS: SCAD patients fare marginally better than other AMI patients on most health status instruments and have similar 12-month health status recovery. Better pre-event health status suggests a need to modify exercise prescriptions and cardiac rehabilitation protocols to better assist this physically active population to recover.

The presentation of spontaneous coronary artery dissection in the emergency department: Signs and symptoms in an unsuspecting population.

OBJECTIVES: Spontaneous coronary artery dissection (SCAD) has emerged as a common cause of acute coronary syndrome (ACS) in young women, although it is rarely discussed in the differential diagnosis for chest pain in the emergency department (ED). In a population otherwise considered low risk for myocardial infarction, there is a danger of incomplete workup and missed diagnosis. In this study, we aim to describe the clinical presentation of those who present to the ED with SCAD to increase awareness of this potentially fatal diagnosis among emergency practitioners. METHODS: Data were queried from the Mayo Clinic "Virtual" Multicenter SCAD Registry, a large multisite international disease registry. The registry includes demographic information as well as data from both medical records and surveys administered following the SCAD event. Symptom presentation was abstracted from survey narrative responses. Data analysis was performed using descriptive statistics. RESULTS: Of 1196 subjects included, chest pain was reported during initial SCAD event in 95.7%. Most common chest symptoms descriptors were pain, pressure/weight, and tightness, with radiation most often in one or both arms/shoulders. Other common symptoms included nausea, shortness of breath, and diaphoresis. Most common electrocardiogram (ECG) findings reported were ST elevation, T-wave abnormality, and normal ECG. Initial troponin values were within normal range in 20.1% of patients. CONCLUSION: With young healthy women often considered "low risk" for ACS, it is important to understand that SCAD is a cause of ACS, and familiarity with presentation can improve awareness among emergency physicians. Our data can provide insight in helping to identify young women who present with chest pain due to SCAD so they can be appropriately evaluated.

Rethinking Rehabilitation: A REVIEW OF PATIENT POPULATIONS WHO CAN BENEFIT FROM CARDIAC REHABILITATION.

Although cardiac rehabilitation (CR) is safe and highly effective for individuals with various cardiovascular health conditions, to date there are only seven diagnoses or procedures identified by the Centers for Medicare & Medicaid Services that qualify for referral. When considering the growing number of individuals with cardiovascular disease (CVD), or other health conditions that increase the risk for CVD, it is important to determine the extent for which CR could benefit these populations. Furthermore, there are some patients who may currently be eligible for CR (spontaneous coronary artery dissection, left ventricular assistant device) but make up a relatively small proportion of the populations that are regularly attending and participating. Thus, these patient populations and special considerations for exercise might be less familiar to professionals who are supervising their programs. The purpose of this review is to summarize the current literature surrounding exercise testing and programming among four specific patient populations that either do not currently qualify for (chronic and end-stage renal disease, breast cancer survivor) or who are eligible but less commonly seen in CR (sudden coronary artery dissection, left ventricular assist device). While current evidence suggests that individuals with these health conditions can safely participate in and may benefit from supervised exercise programming, there is an immediate need for high-quality, multisite clinical trials to develop more specific exercise recommendations and support the inclusion of these populations in future CR programs.

Spontaneous Coronary Artery Dissection: Pitfalls of Angiographic Diagnosis and an Approach to Ambiguous Cases.

Spontaneous coronary artery dissection (SCAD) is a pathophysiologically distinct cause of acute coronary syndromes (ACS). It is increasingly recognized that optimal management is different from that for atherosclerotic ACS and that a SCAD diagnosis has specific long-term prognostic and therapeutic implications. Accurate diagnosis is therefore essential to ensure the best treatment of patients. At present this relies on the recognition of typical features of SCAD identified on invasive coronary angiography. Although most SCAD can be readily distinguished angiographically from alternative causes of ACS, false positive and false negative diagnoses remain common. In particular, sometimes non-SCAD presentations, including atherothrombosis, takotsubo cardiomyopathy, coronary embolism, coronary vasospasm, contrast streaming, and myocardial infarction with nonobstructive coronary arteries, can mimic angiographic features usually associated with SCAD. The authors present the combined experience from European and US SCAD referral centers reviewing the classical angiographic appearances of SCAD, presenting potential diagnostic pitfalls and exemplars of SCAD mimickers. The authors further review the benefits and limitations of intracoronary imaging in the context of SCAD. Finally, the authors discuss the investigation of ambiguous cases and an approach to minimize misdiagnosis in difficult cases.

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.