Spontaneous coronary artery dissection in patients with prior psychophysical stress: a systematic review of case reports and case series.

BACKGROUND: Spontaneous coronary artery dissection (SCAD) is an underdiagnosed cause of acute coronary syndrome, particularly in younger women. Due to limited information about SCAD, case reports and case series can provide valuable insights into its features and management. This study aimed to comprehensively evaluate the features of SCAD patients who experienced psychophysical stress before the SCAD event. METHODS: We conducted an electronic search of PubMed, Scopus, and Web of Science from inception until January 7, 2023. We included case reports or series that described patients with SCAD who had experienced psychophysical stress before SCAD. Patients with pregnancy-associated SCAD were excluded from our analysis. RESULTS: In total, we included 93 case reports or series describing 105 patients with SCAD. The average patient age was 44.29 ± 13.05 years and a total of 44 (41.9%) of patients were male. Among the included SCAD patients the most prevalent comorbidities were fibromuscular dysplasia (FMD) and hypertension with the prevalence of 36.4 and 21.9%, respectively. Preceding physical stress was more frequently reported in men than in women; 38 out of 44 (86.4%) men reported physical stress, while 36 out of 61 (59.1%) females reported physical stress (p value = 0.009). On the other hand, the opposite was true for emotional stress (men: 6 (13.6%)), women: 29 (47.6%), p value < 0.001). Coronary angiography was the main diagnostic tool. The most frequently involved artery was the left anterior descending (LAD) (62.9%). In our study, recurrence of SCAD due to either the progression of a previous lesion or new SCAD in another coronary location occurred more frequently in those treated conservatively, however the observed difference was not statistically significant (p value = 0.138). CONCLUSION: While physical stress seems to precede SCAD in most cases, emotional stress is implicated in females more than males.

Anomalous origin of the coronary artery: prevalence and coronary artery disease in adults undergoing coronary tomographic angiography.

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital coronary anomaly with the potential to cause adverse cardiac events. However, there is limited data on the association between AAOCA and coronary artery disease (CAD). Therefore, the aim of this study is to determine the prevalence and symptoms of patients with AAOCA, as well as investigate the correlation between AAOCA and CAD in a population referred for coronary computed tomographic angiography (CTA). METHODS AND RESULTS: All consecutive patients who underwent CTA from 2010 to 2021 were included. Characteristics, symptoms, coronary related adverse events and CTA information were reviewed by medical records. Separate multivariable cumulative logistic regressions were performed, using the stenosis severity in each of the four coronaries as individual responses and as a combined patient clustered response. Finally, we identified 207 adult patients with AAOCA, the prevalence of AAOCA is 0.23% (207/90,501). Moreover, this study found no significant association between AAOCA and CAD. AAOCA did not contribute to higher rates of hospitalization or adverse cardiac events, including calcification. CONCLUSION: AAOCA is a rare congenital disease that is not associated with increased presence of obstructive CAD in adults.

Spontaneous coronary artery dissection outcomes among pregnant vs. non-pregnant women.

AIMS: Spontaneous coronary artery dissection (SCAD) has become increasingly recognized. It accounts for <1-4% of acute coronary syndrome presentations. Overall, however, it makes up over 40% of pregnancy-associated myocardial infarction. Furthermore, pregnancy-associated spontaneous coronary artery dissection (P-SCAD) is described to have a greater degree of clinical manifestations, including left ventricular dysfunction, shock, and left main or multivessel involvement. The findings are disconcerting, though many studies evaluating P-SCAD are based on case series data or are single centre studies. METHODS AND RESULTS: The aim of this study was to evaluate a larger national dataset to evaluate the outcomes of SCAD and specifically P-SCAD in an attempt to better characterize the severity and clinical nature of this condition. To conduct this study, we analysed the National Readmission Database from January 2016 to December 2020. Propensity matching was done using the Greedy 1:1 method. Multivariate logistics and time-to-event Cox regression analysis models were built by including all confounders significantly associated with the outcome on univariable analysis with a cut-off P-value of 0.2. In multivariate regression analysis, P-SCAD patients had a non-propensity matched odds ratio (OR) of 0.21 (0.3-1.54, P = 0.123) of dying and a propensity matched OR of 0.11 (0.02-0.61, P = 0.012) of dying. Thirty-day readmission rate for P-SCAD was 15.8% (n = 93) and for non-pregnant spontaneous coronary artery dissection (NP-SCAD) was 11.2% (n = 2286); non-propensity matched OR for readmission for PSCAD patients was 1.68 (1.24-2.29, P = 0.001) and propensity matched OR was 3.39 (1.93-5.97, P < 0.001). CONCLUSION: Among hospitalized patient, P-SCAD was associated with similar clinical outcomes and reduced incidence of death when compared with NP-SCAD, though had higher rates of 30-day readmission. Larger-scale observational data will be needed to ascertain the true incidence of cardiovascular complications as it relates to P-SCAD.

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.

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.

Prevalence and characteristics of coronary artery fistulas among 20 259 patients undergoing invasive coronary angiography.

BACKGROUND: Coronary artery fistula (CAF) is a rare coronary anomaly. This study aimed to investigate the prevalence, clinical features, and imaging characteristics of CAF among patients undergoing coronary angiography (CAG). METHOD: This was a retrospective study including 20 259 consecutive patients (12 458 were male) who underwent CAG at our institution from September 2018 to March 2023. Electronic angiography records were reviewed, and a total of 86 (0.42%) CAF patients were enrolled and analyzed. RESULT: Of the 86 CAF patients, 42 (49%) were male. Thus, the prevalence of CAF for males and females was 0.34% and 0.56%, respectively. Arrhythmia, left ventricular (LV) hypertrophy, LV dilation, and LV systolic dysfunction were observed in 38, 25, 10 and 5 cases, respectively. Among the 86 CAF patients, a total of 117 CAFs were detected. 61 (71%) patients had a single CAF, and the remaining 25 (29%) patients had multiple CAFs. Of the 117 CAFs, the most common origins and terminations were the left anterior descending artery (n = 50) and the pulmonary artery (n = 73), respectively. The CAF diameters were greatly varied, ranging from unmeasurable to 7.8 mm, and 22 (18%) CAFs were larger than 3 mm. CONCLUSION: In the present study, the prevalence of CAF was 0.42% with a female predilection. Arrhythmia, LV remodeling and dysfunction were common. Seventy-one percent of patients had a single CAF. The left anterior descending artery and the pulmonary artery were the most common origin and termination of CAFs, respectively. Most CAFs were small, and 18% of CAFs were larger than 3 mm.

Anomalous Coronary Arteries: A State-of-the-Art Approach.

Congenital coronary anomalies are not an infrequent occurrence and their clinical presentation typically occurs during early years, though may be manifested only in adulthood. In the setting of anomalous aortic origin of a coronary artery, this is particularly concerning as it inflicts sudden loss of healthy young lives. Risk stratification remains a challenge and so does the best management decision-making in these patients, particularly if asymptomatic. Standardized approach to evaluation and management, with careful data collection and collaboration among centers, will likely impact future outcomes in this patient population, thus allowing for exercise participation and healthier lives.

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.

Computed tomographic characteristics of congenital coronary artery fistulas in an adult population.

BACKGROUND: Coronary artery fistulas (CAFs) are usually congenital coronary artery anomalies of termination. AIMS: This study aimed to assess the prevalence, anatomic characteristics, and clinical significance of CAFs detected by computed tomography (CT) in an adult population. METHODS: We performed 45 817 CT examinations in 39 066 subjects between 2008 and 2020. The electronic database was manually checked using specific keywords to identify patients with CAFs. The CT characteristics of CAFs were evaluated. CAF was defined as clinically significant if it was the most plausible cause of myocardial infarction, infective endocarditis, heart failure, death during follow-up, hospitalization, or if it required either percutaneous or surgical intervention. RESULTS: Of 39 066 patients, 56 CAFs were detected in 42 subjects (20 men, 47.6%) with a prevalence of 0.11%. Most CAFs originated from the right coronary artery (RCA) (48.2%) and drained into the pulmonary artery (PA) (58.9%). CAFs terminating in the PA were more frequently multiple (P <0.001) and tortuous (P <0.001) as compared to CAFs without PA drainage. Clinically significant CAFs, identified in 7 of 42 patients, were more common in younger (P = 0.03) and male (P = 0.04) subjects and had larger lumen area and diameter at the site of origin (P = 0.03, P = 0.03, respectively). CONCLUSIONS: In the unselected adult population undergoing coronary CT angiography, the RCA and the PA are the most common sites of origin and termination of CAFs, respectively. CAFs draining into the PA are more often multiple and tortuous. Clinically meaningful CAFs are larger and most frequently detected in younger and male patients.

The Patient Experience After Spontaneous Coronary Artery Dissection.

OBJECTIVE: There is considerable burden of anxiety, depression, and post-traumatic stress disorder in patients with spontaneous coronary artery dissection (SCAD), yet research is limited on the experience and impact of SCAD from the patient perspective. This literature review sought to describe the current state of the literature on the patient experience of SCAD and consequences for patients following a SCAD event from the patient perspective. To better understand how people's experiences of SCAD affect their wellbeing, quality of life, lifestyles, and identity, and what would be useful from the patient perspective, an integrative review was performed. METHODS: An integrative literature review was conducted to understand the experience of SCAD and the post-event implications. Five databases were searched. Search terms included 'spontaneous coronary artery dissection', 'SCAD', 'patient', 'experience', 'perspective', and 'opinion'. English-language, peer-reviewed primary research in people with a diagnosis of SCAD that reflected the patient experience was included. Data indicating the SCAD experience including distress prevalence were extracted into an Excel spreadsheet, and narrative synthesis of included studies followed. FINDINGS: From 325 identified studies, five were included for review, yielding a combined sample of 447 participants. Patients with SCAD reported a lack of information about SCAD and the recovery process, and use of the internet for obtaining information. Patients with SCAD reported challenges in recovery including anxiety associated with fear of recurrence and uncertainty, and a need for greater support. A wide range of negative emotions was reported during and after the SCAD event. Participants reported participating in support groups, with mixed reviews of their appropriateness and effectiveness.

[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%).

Spontaneous coronary artery dissection: an overview.

The prevalence of spontaneous coronary artery dissection (SCAD) has increased over the last decades in young adults presenting with acute coronary syndrome. Although the diagnostic tools, including intracoronary imaging, have permitted a more accurate diagnosis of SCAD, the prognosis and overall outcomes remain dismal. Furthermore, the disproportionate sex distribution affecting more women and the underdiagnosis in many parts of the world render this pathology a persistent clinical challenge, particularly since the management remains largely supportive with a limited and controversial role for percutaneous or surgical interventions. The purpose of this review is to summarize the available literature on SCAD and to provide insights into the gaps in knowledge and areas requiring further investigation.

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.

Spontaneous coronary artery dissection in women with acute myocardial infarction: is there a new role for autoimmunity?

AIMS: Spontaneous coronary artery dissection (SCAD) is an uncommon cause of acute myocardial infarction in women and has an unclear pathophysiology. Autoantibodies (AAs) targeting angiotensin-II receptor type 1 (AT1R) and endothelin-1 receptor type A (ETAR) have known detrimental effects on endothelial function. We investigated the prevalence of these AAs in SCAD-affected female patients. METHODS AND RESULTS: Female patients diagnosed at coronary angiography with myocardial infarction and SCAD were consecutively enrolled. Autoantibodies targeting angiotensin-II receptor type 1 and ETAR-AA titres and seropositivity prevalence were compared between SCAD patients, ST-elevation myocardial infarction (STEMI) patients, and healthy women. Ten women with SCAD and 20 age-matched controls (10 women with STEMI and 10 healthy women) were included. Six out of 10 (60%) women with myocardial infarction and SCAD were seropositive for AT1R-AAs and ETAR-AAs. In contrast, only one (10%) healthy woman and one (10%) STEMI patient were seropositive for AT1R-AAs (P = 0.03 and P = 0.03, respectively). One STEMI patient was seropositive for ETAR-AAs, while none of the healthy women was found to be seropositive (P = 0.03 and P = 0.01, respectively). The median AA titre was significantly higher in SCAD patients than in healthy women (P = 0.01 for AT1R-AAs; P = 0.02 for ETAR-AAs) and STEMI patients (P < 0.001 for AT1R-AAs; P = 0.002 for ETAR-AAs). CONCLUSION: Autoantibodies targeting angiotensin-II receptor type 1 and ETAR-AA seropositivity is significantly higher in SCAD women with myocardial infarction than in healthy women or female patients with STEMI. Our findings, corroborated by previous data in the literature and biological plausibility, suggest a possible role for AT1R-AAs and ETAR-AAs in the pathophysiology of SCAD in women with acute myocardial infarction and should warrant further studies with larger sample sizes.

Derivation and Validation of Spontaneous Coronary Artery Dissection Prediction Score in Patients With Myocardial Infarction.

Spontaneous coronary artery dissection (SCAD) is an important cause of acute myocardial infarction (AMI); however, the prevalence of SCAD and its predictors in AMI are unknown. We sought to derive and validate a simple score that can help predict SCAD in patients with AMI. We analyzed the Nationwide Readmissions Database and created a risk score for SCAD in patients with an index hospitalization for AMI. We used a multivariate logistic regression analysis to determine the independent predictors of SCAD, and each was assigned points proportional to its regression coefficient. Among 1,155,164 patients with AMI, 8,630 (0.75%) had SCAD. Based on the derivation cohort, the independent predictors of SCAD were fibromuscular dysplasia (odds ratio [OR] 67.0, 95% confidence interval [CI] 42.0 to 107.9, p <0.01), Marfan or Ehlers-Danlos syndrome (OR 4.7, 95% CI 1.7 to 12.5, p <0.01), polycystic ovarian syndrome (OR 5.4, 95% CI 3.0 to 9.8, p <0.01), female gender (OR 1.99, 95% CI 1.9 to 2.1, p <0.01), and aortic aneurysm (OR 1.41, 95% CI 1.1 to 1.7, p <0.01). The SCAD risk score included fibromuscular dysplasia (5 points), Marfan or Ehlers-Danlos syndrome (2 points), polycystic ovarian syndrome (2 points), female gender (1 point), and aortic aneurysm (1 point). The C-statistics of the score were 0.58 (derivation cohort) and 0.61 (validation cohort). In conclusion, the SCAD score is an easy bedside clinical tool that can help clinicians identify patients with AMI who are at risk for SCAD.

Precipitating factors in patients with spontaneous coronary artery dissection: Clinical, laboratoristic and prognostic implications.

BACKGROUND: Spontaneous coronary artery dissection (SCAD) often presents with acute coronary syndrome and underlying pathophysiology involves the interplay between predisposing factors and precipitating stressors, such as emotional and physical triggers. In our study we sought to compare clinical, angiographic and prognostic features in a cohort of patients with SCAD according to the presence and type of precipitating stressors. METHODS: Consecutive patients with angiographic evidence of SCAD were divided into three groups: patients with emotional stressors, patients with physical stressors and those without any stressor. Clinical, laboratoristic and angiographic features were collected for each patient. The incidence of major adverse cardiovascular events, recurrent SCAD and recurrent angina was assessed at follow-up. RESULTS: Among the total population (64 subjects), 41 [64.0%] patients presented with precipitating stressors, including emotional triggers (31 [48.4%] subjects) and physical efforts (10 [15.6%] subjects). As compared with the other groups, patients with emotional triggers were more frequently female (p = 0.009), had a lower prevalence of hypertension (p = 0.039] and dyslipidemia (p = 0.039), were more likely to suffer from chronic stress (p = 0.022) and presented with higher levels of C-reactive protein (p = 0.037) and circulating eosinophils cells (p = 0.012). At a median follow-up of 21 [7; 44] months, patients with emotional stressors experienced higher prevalence of recurrent angina (p = 0.025), as compared to the other groups. CONCLUSIONS: Our study shows that emotional stressors leading to SCAD may identify a SCAD subtype with specific features and a trend towards a worse clinical outcome.

Coronary Artery Dissection and Myocardial Infarction With Nonobstructed Coronary Arteries: Insights From a UK Nationwide Autopsy-Based Registry-Brief Report.

BACKGROUND: Spontaneous coronary artery dissection (SCAD) and myocardial infarction with nonobstructed coronary arteries (MINOCA) are increasingly recognized causes of acute coronary syndrome and potentially of sudden cardiac death (SCD). SCAD has been correlated to coronary fibromuscular dysplasia (FMD), but the prevalence of SCAD and FMD among SCD victims is unclear. Therefore, we sought to assess characteristics of decedents with SCAD found at autopsy and to compare their clinical and pathological profile with MINOCA victims. METHODS: We reviewed a database of 5325 consecutive cases of SCDs referred to our cardiac pathology center between 1994 and 2017. RESULTS: We identified 18 (0.3%) cases with SCAD and 37 (0.7%) with MINOCA. No signs of coronary FMD were found among SCAD and MINOCA victims. Compared to MINOCA, SCAD decedents were mostly females (78% versus 38%, P=0.006) and SCD occurred during peripartum more frequently in SCAD rather than MINOCA female victims (28% versus 3%, P=0.012) Infarcted myocardium was identified in all cases of MINOCA but only in 5 (28%) of SCAD decedents (P<0.001). Premortem cardiac symptoms were present in 100% of SCAD and 49% of MINOCA victims (P<0.001); substances use or abuse was reported in none of SCAD versus 43% of MINOCA decedents (P=0.001). CONCLUSIONS: SCAD and MINOCA are rare causes of SCD. At autopsy, coronary FMD is not present among SCAD victims. Compared to MINOCA, SCAD victims are more frequently females, are linked to pregnancy, and always experienced premortem cardiac symptoms. Among MINOCA victims' substance use or abuse is common.

Anomalous Coronary Arteries: A State-of-the-Art Approach.

Congenital coronary anomalies are not an infrequent occurrence and their clinical presentation typically occurs during early years, though may be manifested only in adulthood. In the setting of anomalous aortic origin of a coronary artery, this is particularly concerning as it inflicts sudden loss of healthy young lives. Risk stratification remains a challenge and so does the best management decision-making in these patients, particularly if asymptomatic. Standardized approach to evaluation and management, with careful data collection and collaboration among centers, will likely impact future outcomes in this patient population, thus allowing for exercise participation and healthier lives.