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Background: Coronary Artery Fistulas (CAFs) Patients with aneurysm may face severe complications, necessitating prompt treatment. However, data on the outcomes of transcatheter closure in CAFs patients with aneurysm are notably scarce., Methods: This retrospective study included all consecutive CAFs patients who underwent transcatheter closure at Fuwai Hospital from January 2010 to December 2023. Patients were divided into two groups based on the presence of aneurysm, and baseline characteristics, anatomical features, and transcatheter closure outcomes were further compared., Results: The study ultimately included 104 patients, consisting of 56 in the aneurysm group and 48 in the non-aneurysm group. Patients in the aneurysm group were younger [39.79 (16.35) versus 50.69 (13.31) years, p < 0.001] and more frequently present with heart murmurs (21.43% vs. 6.25%, p = 0.03). Multivariate logistic regression indicated that a larger fistula diameter and the presence of CCFs are independent risk factors for the presence of aneurysm in CAF patients. The procedural success rate (75% vs. 75%, P = 1), fistula recanalization rate (11.11% vs. 16.67%, p = 0.42), and reintervention rate (3.7% vs. 6.25%, p = 0.89) were similar between the aneurysm and non-aneurysm groups., Conclusion: A larger fistula diameters and the presence of coronary-cameral fistulas are independent risk factors for the occurrence of aneurysms in patients with CAFs. The outcomes of transcatheter closure are comparable for CAFs patients with and without aneurysm, though post-closure thrombosis within the fistula appears to be more common in patients with aneurysm., (© 2024 Wiley Periodicals LLC.)

Competing Interests: Declaration of competing interest Ron Waksman – Advisory Board: Abbott Vascular, Boston Scientific, Medtronic, Philips IGT, Pi-Cardia Ltd.; Consultant: Abbott Vascular, Append Medical, Biotronik, Boston Scientific, JC Medical, MedAlliance/Cordis, Medtronic, Philips IGT, Pi-Cardia Ltd., Swiss Interventional/SIS Medical AG, Transmural Systems Inc.; Institutional Grant Support: Biotronik, Medtronic, Philips IGT; Investor: Transmural Systems Inc. All other authors – None.

In the absence of standardized management guidelines, coronary artery aneurysms (CAAs) present therapeutic challenges. Percutaneous coronary intervention (PCI) is rarely explored, especially in giant aneurysms with persistent angina, where surgery might be presumed as a preferred option. We describe the technical aspects and feasibility of PCI using Gore Viabahn expanded polytetrafluoroethylene (ePTFE)-covered nitinol self-expanding stents in a 66-year-old woman with a complex medical history and an enlarging, symptomatic right coronary artery aneurysm. The case was complicated by endoleak after the first stent, but intravascular ultrasound guidance enabled the precise deployment of additional stents, resulting in the successful exclusion of the aneurysm. This case demonstrates steps to successful CAA PCI with Gore Viabahn ePTFE-covered nitinol self-expanding stents and emphasizes that in unsuitable surgical candidates, PCI might be a potential alternative for symptomatic CAAs., (© 2024 Wiley Periodicals LLC.)

BACKGROUND A traumatic coronary artery dissection is a rare but severe complication of chest trauma that can result in blockage of the coronary artery. The clinical symptoms can vary considerably, from asymptomatic arrhythmia to acute myocardial infarction and sudden death. This report describes a young man with coronary artery dissection following blunt chest trauma from a motorcycle accident presenting with ventricular fibrillation due to acute myocardial infarction, which was treated with percutaneous transluminal coronary angioplasty and extracorporeal membrane oxygenation. CASE REPORT We present a 21-year-old man with chest contusion from a motorcycle accident who experienced sudden collapse due to ventricular fibrillation and acute myocardial infarction. The patient was resuscitated with extracorporeal membrane oxygenation, and 12-lead electrocardiogram showed sinus tachycardia with a hyperacute T-wave and ST elevation in leads V2-V6. Percutaneous coronary intervention revealed dissection from the ostial to proximal portion of the left anterior descending artery, and traumatic coronary artery dissection was confirmed. He was successfully treated with percutaneous transluminal coronary angioplasty, in which a drug-eluting stent was inserted to enhance blood flow in the left anterior descending artery, resulting in TIMI 2 flow restoration. After 16 days of intensive care, he was discharged and was well at a 3-month follow-up. CONCLUSIONS This report describes a case with the rare association between blunt chest trauma and coronary artery dissection and highlights that coronary artery dissection can result in ST-elevation myocardial infarction. Extracorporeal membrane oxygenation can protect the patient's circulation for coronary angioplasty. Therefore, early detection and intensive resuscitation can prevent disastrous outcomes.

Competing Interests: Funding Support and Author Disclosures Dr Rogers has served as a consultant for Edwards Lifesciences, Medtronic, and Boston Scientific; has served on the Advisory Boards for Medtronic and Boston Scientific; holds equity in Transmural Systems; and holds intellectual property rights for and was a coinventor on patents assigned to National Institutes of Health. Dr Hashim has served on the Advisory Boards for Abbott Vascular, Boston Scientific, and Philips IGT; and has served as a speaker for Abbott Vascular, Boston Scientific, and Philips IGT. Dr Waksman has served on the Advisory Boards for Abbott Vascular, Boston Scientific, Medtronic, Philips IGT, and Pi-Cardia Ltd; has served as a consultant for Abbott Vascular, Append Medical, Biotronik, Boston Scientific, JC Medical, MedAlliance/Cordis, Medtronic, Philips IGT, Pi-Cardia Ltd, Swiss Interventional/SIS Medical AG, and Transmural Systems Inc; has received institutional grant support from Biotronik, Medtronic, and Philips IGT; and is an investor in Transmural Systems Inc. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

We report three cases of coronary artery aneurysm (CAA) in adults who presented with acute coronary syndrome. Two of these patients did not have traditional coronary artery disease risk factors. Management of CAA poses a significant challenge to interventionalists. We discuss the etiologic mechanisms, risk factors, pathophysiology, and diagnosis using angiography, intravascular ultrasound, and coronary computed tomography. We also highlight management options, including medical therapy and catheter-based interventions such as stenting, coil embolization, stent-assisted coil embolization, and surgical exclusion., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2024 The Author(s).)

Background: Coronary artery aneurysms (CAAs), coronary arteriovenous malformations (CAVMs), and spontaneous coronary artery dissections (SCADs) are rare clinical entities, and much is unknown about their natural history, prognosis, and management., Methods: A systematic search of MEDLINE, Embase, and Cochrane Library databases was performed in March 2023 to identify published papers related to CAAs, CAVMs, and SCADs., Results: CAAs are found in 0.3% to 12% of patients undergoing angiography and are often associated with coronary atherosclerosis. They are usually asymptomatic but can be complicated by thrombosis in up to 4.8% of patients and rarely by rupture (0.2%). CAAs can be managed medically, percutaneously with stents or coil embolization, and surgically. The most common surgical procedure is ligation of the aneurysm, followed by coronary artery bypass grafting. The incidence of CAVMs is 0.1% to 0.2% in patients undergoing angiography, and they are most likely associated with congenital abnormal development of the coronary vessels. The diagnosis of CAVMs is usually incidental. Surgical or percutaneous intervention is indicated for patients with large CAVMs, which carry a potential risk of myocardial infarction. SCADs represent 1% to 4% of all acute coronary syndromes and typically affect young women. SCADs are strongly correlated with pregnancy, suggesting the role of sex hormones in their pathogenesis. Conservative management of SCAD is preferred for stable patients without signs of ischemia as spontaneous resolution is frequently reported. Unstable patients should undergo revascularization either percutaneously or with coronary artery bypass grafting., Conclusions: Further evidence regarding the management of these rare diseases is needed and can ideally be derived from multicenter collaborations., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Children who develop coronary artery aneurysms after Kawasaki disease are at risk for cardiovascular morbidity, requiring health care transition and lifelong follow-up with an adult specialist. Follow-up losses after health care transition have been reported but without outcome and patient experience evaluation., Objective: The Theoretical Domains Framework underpinned our aim to explore the required self-care behaviours and experiences of young adults' post-health care transition., Methods: A qualitative description approach was used for virtual, 1:1 interviews with 11 participants, recruited after health care transition from a regional cardiac centre in Ontario. Directed content analysis was employed., Results: Health, psychosocial, and lifestyle challenges were compounded by a sense of loss. Six themes emerged within the Theoretical Domains Framework categories. Participants offered novel health care transition programme recommendations., Conclusions: The realities of health care transition involve multiple, overlapping stressors for young adults with Kawasaki disease and coronary artery aneurysms. Our findings will inform a renewed health care transition programme and will include outcome evaluation.

Coronary periarteritis is a dangerous manifestation of IgG4-related disease, because it forms coronary artery aneurysms, which may cause sudden cardiac death. We report the case of a 78-year-old woman with IgG4-related coronary periarteritis and a coronary aneurysm, which showed progressive enlargement despite maintenance therapy for Type 1 autoimmune pancreatitis. This case was unique, in that coronary periarteritis was the only active lesion that recurred. Low-dose glucocorticoids suppressed the progression of periarterial lesions but led to rapid thinning of the aneurysmal wall and an increase in the size of mural thrombi, which pose a risk of myocardial infarction. Our systematic literature review including 98 cases of 86 articles was performed to examine its treatment strategies and complications. Among the cases in which the effect of immunosuppressive therapy could be followed radiologically, 33 of 37 (89.1%) cases showed improvement in wall thickening/periarterial soft tissue, while 6 of 13 (46.2%) showed worsening increase in the outer diameter of the coronary aneurysms. We propose a draft treatment algorithm and suggest that immunosuppressive therapy for IgG4-related coronary periarteritis with coronary aneurysms should be conducted only after the therapeutic benefit has been determined to outweigh the risks. Because coronary periarteritis can occur without other organ involvement, as in our case, all cases of IgG4-related disease require careful monitoring of coronary artery lesions., (© Japan College of Rheumatology 2023. Published by Oxford University Press.)

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Coronary artery aneurysms (CAA) are uncommon, often incidental findings in patients with acute coronary syndrome (ACS) that represent a management challenge due to as there is a paucity of literature in this area., Methods: We analyzed the National Inpatient Sample database from 2016 to 2020 by including all patients with the admission diagnosis of the ACS who underwent percutaneous coronary intervention (PCI). We sought to evaluate the association of CAA with other relevant systemic conditions and determine the impact of CAA on in-hospital outcomes., Results: Among 1,733,655 hospital admission with ACS who underwent PCI, 2675 had CAA. There was a 2-fold increase in odds of CAA if the patient had coronary artery dissection (OR 2.05 95%CI 1.12-3.75, p = 0.020) or extracoronary aneurysm (OR 2.47 95%CI 1.46-4.16, p = 0.001) and a 3-fold increase in odds if they had a systematic inflammatory disorder (OR 3.24 95%CI 2.08-5.07, p < 0.001). CAA was not associated with increased odds of mortality (OR 1.22 95%CI 0.76-1.95, p = 0.42), bleeding (OR 1.29 95%CI 0.86-1.95, p = 0.22), acute stroke (OR 0.91 95%CI 0.40-2.07, p = 0.83), major adverse cardiac and cerebrovascular events (OR 1.08 95%CI 0.72-1.61, p = 0.71) or cardiac complications (OR 0.85 95%CI 0.49-1.47, p = 0.55). However, it was significantly associated with increased odds of vascular complications (OR 2.17 95%CI 1.47-3.19, p < 0.001)., Conclusions: For patients with ACS who undergo PCI, the presence of CAA is associated with greater odds of vascular complications but after adjustments there was no difference in mortality or other complications. In this population, CAA is more prevalent in patients with coronary dissection, extracoronary aneurysms and systemic inflammatory disorders., Competing Interests: Declaration of competing interest None., (Copyright © 2023. Published by Elsevier Inc.)

Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up., (Copyright © 2023. Published by Elsevier Masson SAS.)

BACKGROUND Kawasaki disease (KD), a systemic vasculitis, affects children aged <5 years and is the leading acquired cardiovascular disease in developed countries. Although intravenous immunoglobulin is an effective treatment for KD and decreases the rate of cardiovascular complications, some patients still develop coronary sequelae, including coronary aneurysms and myocardial infarction. CASE REPORT A 9-year-old boy was diagnosed with KD at the age of 6 years. For coronary sequelae of a giant coronary artery aneurysm (CAA) 8.8 mm in diameter, he was prescribed aspirin and warfarin. At 9 years old, he visited the Emergency Department because of acute chest pain. Electrocardiography revealed an incomplete right bundle branch block and ST-T change over right and inferior leads. Also, troponin I was elevated. Coronary angiography found acute thrombotic occlusion of the right CAA. We performed aspiration thrombectomy with intravenous tirofiban. Coronary angiography and optical coherence tomography (OCT) images later found white thrombi, calcification, destruction of media layer, irregular intimal thickening, and uneven intima edge. We prescribed antiplatelet therapy and warfarin, and he was doing well at a 3-year follow-up. CONCLUSIONS OCT is a promising modality that can impact the clinical care in patients with coronary artery disease. This report presents treatment management and OCT images of KD complicated with a giant CAA and acute myocardial infarction. We used aspiration thrombectomy in combination with medical treatments as the initial intervention strategy. Afterward, the OCT images showed vascular wall abnormalities, which were helpful for defining the future risk and decision making of further coronary interventions and medical treatments.

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Coronary artery aneurysms are well-described in Kawasaki disease and the Multisystem Inflammatory Syndrome in Children and are graded using Z scores. Three Z score systems (Boston, Montreal, and DC) are widely used in North America. The recent Pediatric Heart Network Z score system is derived from the largest diverse sample to-date. The impact of Z score system on the rate of coronary dilation and management was assessed in a large real-world dataset., Methods: Using a combined dataset of patients with acute Kawasaki disease from the Children's Hospital at Montefiore and the National Heart, Lung, and Blood Institute Kawasaki Disease Study, coronary Z scores and the rate of coronary lesions (Z ≥ 2.0) and aneurysms (Z ≥ 2.5) were determined using four Z score systems. Agreement among Z scores and the effect on Kawasaki management were assessed., Results: Of 333 patients analysed, 136 were from Montefiore and 197 from the Kawasaki Disease Study. Age, sex, body surface area, and rate of coronary lesions did not differ between the samples. Among the four Z score systems, the rate of acute coronary lesions varied from 24 to 55%. The mean left anterior descending Z scores from Pediatric Heart Network and Boston had a large uniform discrepancy of 1.3. Differences in Z scores among the four systems may change anticoagulation management in up to 22% of a Kawasaki population., Conclusions: Choice of Z score system alone may impact Kawasaki disease diagnosis and management. Further research is necessary to determine the ideal coronary Z score system.

Background: Coronary artery aneurysms (CAA) are reported in up to 5% of patients undergoing coronary angiography. Treatment of CAAs with covered stents has been reported in several case reports, however there is limited evidence available on the effectiveness and safety of this interventional practice., Purpose: To evaluate the current practice and outcomes of elective treatment of coronary artery aneurysms with covered stents., Methods: We conducted a systematic review of published case reports and case series of patients presenting with CAA that have been treated with covered stents in a non-emergency setting., Results: A total of 63 case reports and 3 case series were included in the final analysis comprising data from 81 patients. The treated CAA was situated in a native coronary artery in 92.6%, and in a saphenous vein graft in 7.4%. Procedural success was achieved in 95.1%. The types of stents used were mainly polytetrafluoroethylene (75.3%) and Papyrus (11.1%). In 11.0% of cases additional abluminal drug eluting stents (DES) and in 6.8% additional adluminal DES were implanted. After a mean follow up of 13.4 months overall major adverse cardiovascular events (MACE), mortality, myocardial infarction, stroke, stent thrombosis and target lesion revascularization were reported in 26.2, 0.0, 7.6, 0.0, 4.6 and 18.5% of cases, respectively., Conclusions: The use of covered stents for elective treatment of CAA appears to be effective and reasonably safe. Nevertheless, it is associated with higher MACE rate, driven mainly by higher target lesion revascularization. Further studies, particularly in form of randomized trials and controlled registries are warranted to identify patients who might profit the most from this procedure., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Kawasaki disease (KD) is a common form of vasculitis in children that can be complicated by coronary artery aneurysms (CAAs). Data of long-term outcomes and major adverse cardiac events (MACE) in children with CAAs following KD in developing country are limited. Our aims were to determine the rates of MACE and identify risk factors associated with MACE in children with KD and CAAs in Thailand. We performed a retrospective analysis of data from 170 children diagnosed with KD and CAAs in two tertiary hospitals between 1994 and 2019. During a median (range) follow-up of 5.4 years (22 days to 23 years), 19 patients (11.2%) experienced MACE, that included 12 coronary artery bypass grafting, 2 percutaneous coronary intervention and 5 children with evidence of myocardial ischemia and coronary occlusion. Coronary interventions were performed at a median time of 4 years (0.01 to 9.5 years) after KD diagnosis. Forty-nine patients (28.8%) had giant CAAs. No MACE was reported in children with small CAAs. Independent risks of MACE were from the absence of intravenous immunoglobulin treatment (HR 7.22; 95% CI 2.21 to 23.59; p = 0.001), the presence of giant aneurysms (HR 13.59; 95% CI 2.43 to 76.09; p = 0.003), and CAAs that involved bilateral branches of coronary arteries (HR 6.19; 95% CI 1.24 to 30.92; p = 0.026). Among children with giant CAAs, the intervention-free rate was 93.8%, 78.7% and 52.2%, at 1, 5 and 10 years, respectively. Of note, 81% of the small CAAs regressed to a normal size, and for medium CAAs, 50% regressed to normal size. Overall, ~10% of children with CAAs following KD experienced MACE in this cohort. Timely IVIG treatment in children with KD following symptom onset will reduce the risk of MACE. Cautious surveillance to identify cardiac complications should be recommended for children once medium or giant CAAs develop. Trial registration: TCTR20190125004., Competing Interests: The authors have declared that no competing interest exist.

Mycotic coronary aneurysms are rare, with potentially fatal complications. The treatment of choice is surgical intervention. We present a case of a mycotic coronary aneurysm secondary to a catheter-related bloodstream infection, failed surgical treatment, and eventual treatment with percutaneous coil embolization., (©Copyright 2021 by University Health Partners of Hawai‘i (UHP Hawai‘i).)

Noonan syndrome (NS) is a genetic disorder characterized by facial dysmorphism, congenital heart disease, and short stature. In very rare cases, patients with this syndrome have coronary disease. Their management and prognosis are currently unclear. We have described 4 cases of coronary aneurysms/coronary ectasia and 1 case of a single coronary artery in patients with adult Noonan syndrome, followed in a medical and surgical center of adult congenital heart disease. The average age was 49.4years old. The majority of them had both pulmonary stenosis and interauricular communication. None had symptoms of angina, at rest, or with stress. Only one patient who had any structural heart disease, had a thrombotic complication with chronic occlusion of the right coronary and anterior inter ventricular artery, fortuitous finding, with no ischemic signs to functional tests, treated only with anti-vitamin K. Finally, any deaths have also been reported in our series. Coronary artery diseases essentially coronary aneurysm/ectasia remain a rare condition in adult patients with NS. Evolution is unknown and complications such as coronary artery thrombosis do not necessarily require surgical treatment., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Patients with congenital heart disease frequently have aneurysms or coronary artery fistulae that necessitate treatment. Metal vascular coils have been a mainstay of treatment for these lesions. In 2002, coils coated or filled with expandable hydrogel were introduced to treat cerebral aneurysms; however, the literature on their use in patients with congenital heart disease is limited. We present 5 cases in which large vascular lesions in children or adolescents with congenital heart disease were successfully occluded with hydrogel coils., (© 2021 by the Texas Heart® Institute, Houston.)

Saphenous vein graft aneurysms (SVGAs) following coronary artery bypass grafting (CABG) surgery were first described in 1975. Although rare, in the absence of a prompt diagnosis, SVGAs can be responsible for serious complications and adverse outcomes. The clinical presentation of SVGAs described in the literature can vary from an asymptomatic patient with an incidental radiological finding to a profoundly shocked patient with life-threatening hemorrhage secondary to SVGA rupture. Improvements in diagnostic tools within the last decade, such as multislice computed tomographic scanning, has enabled early detection of SVGAs, and therefore, an expansion of the current management options. In this review, the current data and knowledge about clinical presentation, diagnosis, natural history, and treatment of SVGAs are updated, with a specific emphasis on the evolution of management strategies of this rare complication over the last 45 years. Finally, a clinical algorithm to guide decision-making and management is proposed., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

In the 2017 American Heart Association (AHA) Kawasaki disease (KD) guidelines, risk levels (RLs) for long-term management are defined by both maximal and current coronary artery (CA) dimensions normalized as z-scores. We sought to determine the degree to which current recommended practice differs from past actual practice, highlighting areas for knowledge translation efforts. The International KD Registry (IKDR) included 1651 patients with CA aneurysms (z-score > 2.5) from 1999 to 2016. Patients were classified by AHA RL using maximum CA z-score (RL 3 = small, RL 4 = medium, RL 5 = large/giant) and subcategorized based on decreases over time. Medical management provided was compared to recommendations. Low-dose acetylsalicylic acid (ASA) use ranged from 86 (RL 3.1) to 95% (RL 5.1) for RLs where use was "indicated." Dual antiplatelet therapy (ASA + clopidogrel) use ranged from 16% for RL 5.2 to 9% for RL 5.4. Recommended anticoagulation (warfarin or low molecular weight heparin) use was 65% for RL 5.1, while 12% were on triple therapy (anticoagulation + dual antiplatelet). Optional statin use ranged from 2 to 8% depending on RL. Optional beta-blocker use was 2-25% for RL 5, and 0-5% for RLs 3 and 4 where it is not recommended. Generally, past practice was consistent with the latest AHA guidelines, taking into account the flexible wording of recommendations based on the limited evidence, as well as unmeasured patient-specific factors. In addition to strengthening the overall evidence base, knowledge translation efforts may be needed to address variation in thromboprophylaxis management.

Background: Polyarteritis nodosa (PAN) is a relatively rare systemic necrotizing vasculitis that typically affects medium-sized arteries. Although myocardial ischemia may occur due to involvement of the coronary arteries, overt myocardial infarction is uncommon. Case presentation A 22-year-old Chinese man experiencing chest pain for 7 months was admitted to our hospital. Consistently, the pain tended to last for a few minutes and then spontaneously subside. He had 7-year history of "stable" PAN. Coronary angiography revealed slight plaque infiltration of the left main coronary artery; however occlusion of all the three major coronary arteries with multiple aneurysms. A stent was implanted into the obtuse margin branch artery which was 95% stenosis, and then the chest pain was alleviated. Considering that the occlusion of coronary arteries was due to insidious vasculitis, prednisone 50 mg/day and methotrexate 15 mg/week were reinitiated, in combination with anti-angina medications., Conclusions: We report a young patient with insidious occlusion of three main coronary arteries under the circumstance of stable PAN for 7 years, suggesting the necessity of assessing the heart, in spite of normal acute phase reactants. The appropriate screening strategy needs to be studied.

Background: Coronary artery aneurysms (CAAs) are increasingly diagnosed on coronary angiography; however, controversies persist regarding their optimal management. In the present study, we analysed the long-term outcomes of patients with CAAs following three different management strategies., Methods: We performed a retrospective review of patient records with documented CAA diagnosis between 2000 and 2005. Patients were divided into three groups: medical management versus percutaneous coronary intervention (PCI) versus coronary artery bypass grafting (CABG). We analysed the rate of major cardiovascular and cerebrovascular events (MACCEs) over a period of 10 years., Results: We identified 458 patients with CAAs (mean age 78±10.5 years, 74.5% men) who received medical therapy (N=230) or underwent PCI (N=52) or CABG (N=176). The incidence of CAAs was 0.7% of the total catheterisation reports. The left anterior descending was the most common coronary artery involved (38%). The median follow-up time was 62 months. The total number of MACCE during follow-up was 155 (33.8%); 91 (39.6%) in the medical management group vs 46 (26.1%) in the CABG group vs 18 (34.6%) in the PCI group (p=0.02). Kaplan-Meier survival analysis showed that CABG was associated with better MACCE-free survival (p log-rank=0.03) than medical management. These results were confirmed on univariate Cox regression, but not multivariate regression (OR 0.773 (0.526 to 1.136); p=0.19). Both Kaplan-Meier survival and regression analyses showed that dual antiplatelet therapy (DAPT) and anticoagulation were not associated with significant improvement in MACCE rates., Conclusion: Our analysis showed similar long-term MACCE risks in patients with CAA undergoing medical, percutaneous and surgical management. Further, DAPT and anticoagulation were not associated with significant benefits in terms of MACCE rates. These results should be interpreted with caution considering the small size and potential for selection bias and should be confirmed in large, randomised trials., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

BACKGROUND Coronary artery aneurysm (CAA) is uncommon angiographic finding with unclear pathophysiology. Atherosclerosis is the main contributing risk factor in adults. To date, there are no standardized recommendations for the management of CAA. Therefore, this case report describes the effectiveness of PCI as therapeutic approach for giant CAA. CASE REPORT We present the case of a 69-year-old male smoker brought to the Emergency Department (ED) due to the crescendo angina. Coronary angiography showed a giant saccular proximal left anterior descending coronary artery aneurysm, which was successfully treated with covered stent implantation, leading to good outcome. CONCLUSIONS The management of CAA is individualized and depends on several parameters such as aneurysm characteristics, technical challenges, and clinical situation. Future clinical trials investigating the role of PCI are needed.

We evaluated the safety and efficacy of second-generation drug-eluting stents (DES) fully covering a coronary artery aneurysm (CAA) and stenosis lesion. Patients (n = 33) with CAA and stenosis lesion (>60%) were enrolled between January 2014 and December 2017. Baseline characteristics and biochemical variables were recorded during hospital admission. Changes in CAA resolution (the reduction on CAA size), minimal lumen diameter (MLD), and diameter stenosis (DS) were determined before, just after, and 1 year after percutaneous coronary intervention (PCI). After DES implantation, MLD and DS after PCI were improved compared with those before PCI ( P < .01). Also, thrombolysis in myocardial infarction blood flow was significantly enhanced after PCI ( P < .01). One year after PCI, maximal CAA diameter in patients with CAA and stenosis lesion was significantly reduced compared with those just after PCI ( P < .01). Meanwhile, CAA resolution ratio in these patients were more than those just after PCI ( P < .01). Furthermore, there was a significant reduction about CAA length in these patients ( P < .01). Last, there were no clinical events (including cardiac death, myocardial infarction, and revascularization) in the study. Second-generation DES implantation fully covering CAA and stenosis lesion was safe and effective.

To identify the risk factors of progression and persistence of small- and medium-sized coronary artery aneurysm (CAA) in a contemporary cohort of patients with Kawasaki disease (KD) and to determine the relationship between CAA progression and persistence. A total of 89 KD patients with small- and medium-sized CAA were prospectively enrolled. All patients were followed up at least for 2 years by serial echocardiography. Multivariate logistic regression analysis was conducted to evaluate independent risk factors for CAA progression and persistence. A total of 46 (51.7%) and 73 (82.0%) patients showed echocardiographic CAA regression by 1 month and 24 months of follow-up, respectively. CAA progression was documented in 12 (13.5%) patients during follow-up. The initial aneurysm size according to CAA classification (OR 0.089, 95% CI 0.013-0.634, P = 0.016) and CAA progression (OR 42.618, 95% CI 3.740-485.6, P = 0.003) were independently associated with CAA persistence. The number of involved coronary arteries (OR 0.223, 95% CI 0.065-0.767, P = 0.015) and lymphocyte proportion (OR 1.327, 95% CI 1.019-1.727, P = 0.040) were independently associated with CAA progression.Conclusion: Patients with KD and greater initial aneurysm size, CAA progression, more involved coronary arteries, and lower lymphocyte proportion may require intensive cardiac monitoring and adjuvant therapies.What is Known:• Long-term outcomes of patients with KD and CAA are primarily driven by the consequences of CAA regression and progression.• Regression and progression occurs more frequently in patients with small- and medium-sized CAAs, and less frequently for giant CAAs.What is New:• The CAA size at diagnosis, NCAI, and the proportion of lymphocytes are presumably associated with the small- and medium-sized CAA persistence or CAA progression.• The CAA progression was associated with CAA persistence.

We report two females with coronary artery occlusion caused by presumed Kawasaki disease that delivered children without any special treatment. After a 58-year-old female had ventricular tachycardia, a giant coronary artery aneurysm with calcification at the bifurcation of the left coronary artery and segmental stenosis of the right coronary artery were pointed out by CT angiography. She had an episode of sepsis when 3 years old. Further, she remembered chest pain during sleep after that episode. She had delivered twice without any complication during her 20s. Her diagnosis was undiagnosed coronary artery lesions caused by presumed Kawasaki disease and a previous myocardial infarction, and she underwent radiofrequency catheter ablation and implantable cardioverter defibrillator implantation. The other 48-year-old female was accidentally discovered to have coronary artery calcification on CT, while experiencing pneumonia. Her CT angiograms revealed a right coronary artery occlusion and coronary artery calcification at segments 1, 6, and 11. She had a history of "scarlet fever" before 12 months. Premature ventricular contractions were detected, while delivering her first child when 31 years old. However, she was not diagnosed as ischaemic heart disease and delivered twice by a vaginal delivery without any complication. Current guidelines recommend systemic anti-coagulation and anti-platelet therapy for all patients with giant aneurysms resulting from Kawasaki disease in childhood. The two women reported here were fortunate not to have had complications during pregnancy and delivery despite their severe coronary artery aneurysms, which were unrecognised clinically until later in life. They were lucky cases.

Background: We aimed to investigate the clinical implications of unresponsiveness to single or repeated courses intravenous immunoglobulin (IVIG) and Kawasaki disease (KD) shock syndrome in patients with KD in an era of a single brand of IVIG., Methods: Data were collected from National Health Insurance database 2010-2013. Characteristics of the KD patients were analyzed, including age, gender, shock, and associated coronary aneurysms., Results: There were 3043 KD patients (male: 1872) identified. Among them, 46 (1.51%) had KDSS, 261 patients (8.5%) had IVIG unresponsiveness, and 225 patients (7.4%) developed coronary aneurysms. Moreover, 51 patients did not respond to the second course IVIG therapy, i.e., re-IVIG unresponsiveness. KDSS was associated with the occurrence of IVIG unresponsiveness (P < 10 -4 ) and re-IVIG unresponsiveness (P = 0.02). In addition to male gender and KD shock syndrome, IVIG unresponsiveness (OR: 2.18, 95% CI: 1.48-3.22, P = 0.001) and re-IVIG unresponsiveness (OR: 2.87, 95% CI: 1.40-5.89, P = 0.004) were both independent risk factors for coronary aneurysms., Conclusions: In a nationwide KD cohort, both IVIG unresponsiveness and re-IVIG unresponsiveness increase the risk of coronary aneurysms. Such observation addresses the importance of refining the treatment for IVIG unresponsiveness, at least in those with KD shock syndrome.

Objective: Determine coronary artery ectasia (CAE) prevalence and clinical outcome in a large cohort of patients underwent coronary angiography., Methods: In an 11-year period, between 2006 and 2017, 20 455 coronary angiography studies were performed at a large university centre. Patients diagnosed with CAE based on procedure report were included in the final analysis., Results: CAE was diagnosed in 174 out of 20 455 studies (0.85% per total angiograms, 161 patients). Patients' average age was 59.6±11.2 years old with male predominance (90.7%). Diffuse ectasia morphology was most common (78.9%), followed by fusiform (16.1%) and saccular (5%). Mixed CAE and atherosclerotic heart disease (ASHD) was present in 75.2% of the patients and isolated CAE in 24.8%. The most common coronary artery involved was the right coronary artery (RCA) (79%). Following index angiography, all the isolated CAE group was managed conservatively, while 67% of the mixed CAE-ASHD group underwent coronary intervention. In an average follow-up of 6±3.6 years, adverse clinical event (a composite endpoint of any death, cerebrovascular accident, myocardial infarction, thromboembolic event, bleeding and stent thrombosis) occurred in 48.8% of the mixed CAE-ASHD group compared with 25% in the isolated CAE group (p<0.05)., Conclusions: CAE is a rare phenomenon. The most common artery involved was the RCA, and the diffused type of CAE was the most frequent. Most patients with CAE have also concomitant ASHD, and those patients have higher mortality and complications rate, compared with isolated CAE disease., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Coronary Aneurysms are a focal dilatation of an artery segment >1.5-fold the normal size of adjacent segments. Although some series have suggested a prevalence of 0.3-12%, data are lacking. In addition, they are not mentioned in practice guidelines. Our aim was investigate its prevalence, management and long-term outcomes., Methods and Results: The coronary artery aneurysm registry (CAAR) involved 32 hospitals across 9 countries in America and Europe. We reviewed 436,467 consecutive angiograms performed over the period 2004-2016. Finally, 1565 patients were recruited. Aneurysm global prevalence was 0.35%. Most patients were male (78.5%) with a mean age of 65 years and frequent cardiovascular risk factors. The main indication for angiogram was an acute coronary syndrome, 966 cases. The number of aneurisms was ≤2 per patient in 95.8% of the cases, mostly saccular, most frequently found in the left anterior descending and with numbers proportional with coronary stenosis. Aortopathies were related with more aneurysms too. Most patients received any revascularization procedure (69%), commonly percutaneous (53%). After a median follow-up of 37.2 months, 485 suffered a combined event (MACE) and 240 died. Without major differences comparing CABG vs PCI, MACE and death were more frequent in patients who received bare metal stents., Conclusions: Coronary artery aneurysms are not uncommon. Usually, they are associated with coronary stenosis and high cardiovascular risk. Antiplatelet therapy seems reasonable and a percutaneous approach is safe and effective., (Copyright © 2019 Elsevier B.V. All rights reserved.)