Coronary Aneurysms Research Articles

Increasing incidence of Kawasaki disease and associated coronary aneurysm in Aotearoa New Zealand: a retrospective cohort study

BackgroundKawasaki disease (KD) is a childhood vasculitis which causes coronary artery aneurysms (CAA). There is a paucity of data regarding KD in Aotearoa New Zealand. We aimed to provide up-to-date...

An Update on Kawasaki Disease.

To summarize advances in research on the epidemiology, pathogenesis, diagnosis, and treatment of Kawasaki Disease (KD), a systemic inflammatory illness of unknown etiology that affects children globally. The epidemiology of KD was affected by the COVID-19 pandemic and advances in molecular immunology and machine learning have enabled research into its pathogenesis. There is ongoing research into agents that can be used to intensify initial treatment and accumulating evidence supporting the use of certain rescue regimens for refractory disease over others. There is promise surrounding a new coronary artery aneurysm prediction model. Research into the post-acute morbidity of KD continues. The COVID-19 pandemic temporarily reduced the incidence of KD. The NLRP3 inflammasome plays a key role in KD pathogenesis. Intensified initial treatment benefits high-risk patients, yet no intensification regimen shows superiority over another. Corticosteroids, infliximab, or combination therapy with IVIg plus another agent may be superior rescue regimens compared to IVIg alone for refractory KD. The Son score, developed in North America, predicted coronary artery lesions in Japanese and Italian cohorts. Patients with a history of KD may carry long-term physical and emotional burdens that persist into adulthood yet appear to have typical neurocognitive development. Successful transition to adult healthcare presents a challenge.

Uncommon presentation of left main congenital coronary aneurysm: a rare case report

Coronary aneurysm, a dilated segment of the coronary artery, is a rare condition with a prevalence ranging from 0.02% to 0.2%. According to the current literature, reports of large aneurysms in the left main artery are extremely rare. We present a case of a 43-year-old male patient presenting with cough, wheezing, and dyspnea after a cold. Initial examinations suggested viral myocarditis, but further evaluation revealed a giant aneurysm in the left main coronary artery. Due to the high surgical risk, conservative treatment was chosen. Follow-up assessments showed no significant changes in the coronary aneurysm, with slight improvement in dyspnea. This rare case of a left main congenital coronary aneurysm suggests that treatment should consider the patient's overall condition, thrombosis presence, suitability for anticoagulant therapy, and aneurysm location and size.

Unraveling the gut: the pivotal role of intestinal mechanisms in Kawasaki disease pathogenesis

Kawasaki disease (KD), an acute systemic vasculitis that primarily affects children under 5 years of age, is the leading cause of acquired heart disease in this age group. Recent studies propose a novel perspective on KD’s etiology, emphasizing the gastrointestinal (GI) tract, particularly the role of gut permeability. This review delves into how disruptions in gut barrier function trigger systemic inflammatory responses, exacerbate vascular inflammation, and contribute to coronary artery aneurysms. Evidence suggests that children with KD often exhibit increased gut permeability, leading to an imbalance in gut immunity and subsequent gut barrier damage. These changes impact vascular endothelial cells, promoting platelet aggregation and activation, thereby advancing severe vascular complications, including aneurysms. Additionally, this review highlights the correlation between GI symptoms and increased resistance to standard treatments like intravenous immunoglobulin (IVIG), indicating that GI involvement may predict therapeutic outcomes. Advocating for a new paradigm, this review calls for integrated research across gastroenterology, immunology, and cardiology to examine KD through the lens of GI health. The goal is to develop innovative therapeutic interventions targeting the intestinal barrier, potentially revolutionizing KD management and significantly improving patient outcomes.

Long-Term Prognosis of Coronary Aneurysms: Insights of CAAR, an International Registry

BackgroundLimited data are available to guide the management of coronary artery aneurysms (CAAs). ObjectivesThe authors sought to define the clinical characteristics, identify variables that predict outcomes, and provide long-term data on CAAs. MethodsWe describe outcomes from 1,729 consecutive patients with CAAs included in an ambispective international registry (CAAR [Coronary Artery Aneurysm Registry]; NCT02563626) involving 33 hospitals across 9 countries in America and Europe. ResultsPatients were predominantly male (78.6%; 1,359/1,729) with a mean age of 66 years. Classic cardiovascular risk factors were common, as well as coronary artery disease (85.8%; 1,484/1,729), peripheral vascular disease (10.9%; 188/1,729), and chronic kidney disease (8.0%; 138/1,729). The median number of aneurysms per patient was 1.0 (Q1-Q3: 1.0-1.0), with the most affected territory being the left anterior descending artery (49.6%; 857/1,729). The majority underwent any revascularization procedure (68.5%; 1,184/1,729), mainly percutaneous coronary intervention (50.7%; 877/1,729), and were discharged on dual antiplatelet therapy (65.6%; 1,134/1,729). After a median follow-up of 44.8 months (Q1-Q3: 14.9-88.1), 379 died (21.9%), and 641 (37.1%) developed a major adverse cardiovascular event (MACE) (all-cause death, heart failure, unstable angina, and reinfarction). In a multivariable analysis, age (HR: 1.03; 95% CI: 1.02-1.04; P < 0.001), diabetes mellitus (HR: 1.47; 95% CI: 1.23-1.75; P < 0.001), renal insufficiency (HR: 1.53; 95% CI: 1.19-1.96; P = 0.010), peripheral vessel disease (HR: 1.43; 95% CI: 1.13-1.82; P = 0.003), reduced left ventricular ejection fraction (HR: 0.98; 95% CI: 0.98-0.99; P < 0.001), acute indication for the index coronary angiography (HR: 1.30; 95% CI: 1.08-1.55; P = 0.005), and the number of coronary vessels presenting severe stenosis (HR: 1.11; 95% CI: 1.02-1.20; P = 0.015) were independent predictors of MACEs. Remarkably, only 37 patients presented with local aneurysm complications during follow-up. ConclusionsThe long-term prognosis of CAAs is not favorable, with MACEs associated with the underlying risk factor profile for atherosclerotic heart disease.

Clinical characteristics of coronary artery involvement in children with Behcet's syndrome.

To review the clinical features, treatment, and prognosis of coronary involvement in Behcet's syndrome in children. Retrospectively analyzed medical records of BS patients admitted to our institution from 2012 to 2024. Six children with BS with coronary involvement were admitted, including 2 males and 4 females. The mean age was 5.7years and the mean course was 17.2months. All of them had coronary dilatation, including 1 case with aneurysmal dilatation, 2 cases with bilateral coronary dilatation, 4 cases with unilateral dilatation, and 4 cases with valvular disease. No symptom for coronary artery dilation and all of coronary dilatation were detected by echocardiography. Manifestations of extracardiac vascular involvement included arteriovenous wall thickness and thrombosis. Five cases had only arterial involvement and one case had both artery and vein involvement. Pulmonary artery was involved in 1 case. All of them were treated with glucocorticoid and immunosuppressant, 1 child was treated with TNF-α, and 1 received aortic valvuloplasty due to a large number of aortic regurgitations. The patient with coronary aneurysm died suddenly after giving up treatment, and the other 4 cases were all improved to varying degrees. Vascular involvement is an important factor affecting the prognosis of Behcet's syndrome. Patients with coronary involvement are occultic and difficult to diagnose. Both arteries and veins can be involved, and some patients have severe symptoms. Clinicians should attach great importance to early vascular screening, especially coronary artery. Early combined medication may help improve the prognosis. Key Points • Vascular involvement is an important factor affecting the prognosis of Behcet's syndrome. • Coronary involvement in BS is rare in children, which is predominantly coronary dilatation. • The onset of BS with coronary involvement is insidious and easy to be missed, so early coronary artery screening in children with BS should be emphasized. • Early combined medication may help improve the prognosis of BS with coronary involvement.

Accuracy of Machine Learning in Discriminating Kawasaki Disease and Other Febrile Illnesses: Systematic Review and Meta-Analysis.

Kawasaki disease (KD) is an acute pediatric vasculitis that can lead to coronary artery aneurysms and severe cardiovascular complications, often presenting with obvious fever in the early stages. In current clinical practice, distinguishing KD from other febrile illnesses remains a significant challenge. In recent years, some researchers have explored the potential of machine learning (ML) methods for the differential diagnosis of KD versus other febrile illnesses, as well as for predicting coronary artery lesions (CALs) in people with KD. However, there is still a lack of systematic evidence to validate their effectiveness. Therefore, we have conducted the first systematic review and meta-analysis to evaluate the accuracy of ML in differentiating KD from other febrile illnesses and in predicting CALs in people with KD, so as to provide evidence-based support for the application of ML in the diagnosis and treatment of KD. This study aimed to summarize the accuracy of ML in differentiating KD from other febrile illnesses and predicting CALs in people with KD. PubMed, Cochrane Library, Embase, and Web of Science were systematically searched until September 26, 2023. The risk of bias in the included original studies was appraised using the Prediction Model Risk of Bias Assessment Tool (PROBAST). Stata (version 15.0; StataCorp) was used for the statistical analysis. A total of 29 studies were incorporated. Of them, 20 used ML to differentiate KD from other febrile illnesses. These studies involved a total of 103,882 participants, including 12,541 people with KD. In the validation set, the pooled concordance index, sensitivity, and specificity were 0.898 (95% CI 0.874-0.922), 0.91 (95% CI 0.83-0.95), and 0.86 (95% CI 0.80-0.90), respectively. Meanwhile, 9 studies used ML for early prediction of the risk of CALs in children with KD. These studies involved a total of 6503 people with KD, of whom 986 had CALs. The pooled concordance index in the validation set was 0.787 (95% CI 0.738-0.835). The diagnostic and predictive factors used in the studies we included were primarily derived from common clinical data. The ML models constructed based on these clinical data demonstrated promising effectiveness in differentiating KD from other febrile illnesses and in predicting coronary artery lesions. Therefore, in future research, we can explore the use of ML methods to identify more efficient predictors and develop tools that can be applied on a broader scale for the differentiation of KD and the prediction of CALs.

Coronary artery aneurysms occurrence risk analysis between Kawasaki disease and LRP1B gene in Taiwanese children

Coronary artery aneurysms occurrence risk analysis between Kawasaki disease and LRP1B gene in Taiwanese children

Update on Diagnosis and Management of Kawasaki Disease: A Scientific Statement From the American Heart Association.

Kawasaki disease (KD), an acute self-limited febrile illness that primarily affects children <5 years old, is the leading cause of acquired heart disease in developed countries, with the potential of leading to coronary artery dilation and coronary artery aneurysms in 25% of untreated patients. This update summarizes relevant clinical data published since the 2017 American Heart Association scientific statement on KD related to diagnosis, cardiac imaging in acute KD treatment, and long-term management. Criteria defining North American patients at high risk for developing coronary artery aneurysms who may benefit from more intensive initial treatment have been published. Advances in cardiovascular imaging have improved the ability to identify coronary artery stenosis in patients with KD, yet knowledge gaps remain regarding optimal frequency of serial imaging and the best imaging modality to identify those at risk for inducible myocardial ischemia. Recent data have advanced the understanding of safety and dosing for several anti-inflammatory therapies in KD. New anticoagulation medication, myocardial infarction management, transition of health care for patients with KD, and future directions in research are discussed.

Variation in the management and treatment of children with giant coronary artery aneurysm following Kawasaki disease.

Giant coronary artery aneurysms are rare but potentially fatal complications of Kawasaki disease. The lack of evidence-based recommendations on their management and treatment cause guidelines and practices to differ. We aimed to assess these variations. An anonymous online survey regarding surveillance, imaging, pharmacological management, and interventional practices was distributed among 134 physicians attending to Kawasaki disease patients worldwide. A p-value of <0.05 was deemed significant. The majority (60%) of respondents were general paediatric cardiologists, and 29% interventional specialists. The average years in practice was 15 ± 9.6. Physicians from Asia had the most experience with giant coronary artery aneurysms. American practitioners preferred combining anticoagulants with aspirin. Beta-blockers and statins were more likely used in teenagers versus younger children. Cardiac catheterisation was most (52%) chosen for coronary surveillance in patients with echocardiogram anomalies, followed by Coronary CT-angiography. The indications for coronary intervention were split among respondents, regardless of geographic region or experience. The preferred treatment of coronary stenosis was percutaneous intervention (69%) versus bypass surgery. For thrombosis, thrombolytics (50%) were preferred over percutaneous (39%) and surgical (11%) interventions. Most (92%) preferred intervening in young children in a paediatric facility but were split between a paediatric and adult facility for older children. Most chose combined management by adult and paediatric specialists for either age-scenarios (70, 82%). As identified by our study, the lack of large studies and evidence-based recommendations cause uncertainty and ambivalence towards certain treatments. International collaborative efforts are needed to provide more robust evidence in the management of these patients.

Identification of clinical risk factors for coronary artery lesions in children with Kawasaki disease: a retrospective cohort study.

Coronary artery lesions are the most severe complications of Kawasaki disease. Despite recent advances, evidence of the association between risk factors and coronary artery lesion is lacking. In this study, we demonstrated the potential clinical indicators that could assist to evaluate the prevalence of coronary artery lesion among paediatric patients with Kawasaki disease. We retrospectively enrolled 260 paediatric patients with Kawasaki disease. Patients with coronary dilation, coronary aneurysm, and intimal thickening of coronary arteries were included in this study. Medical records of each patient were collected. Logistic regression analysis was performed to explore risk factors and the occurrence of coronary artery lesion in patients with Kawasaki disease. Respectively, 64 (24.6%), 39 (15%), and 56 patients (21.5%) of the participants had coronary dilation, coronary aneurysm, and intimal thickening of coronary arteries. Univariate analysis revealed that age, gender, duration of fever, time of initial use of intravenous immunoglobulin, erythrocyte sedimentation rate, white blood cell counts, time of platelet increase, the maximum value of platelet, albumin, and immunoglobulin G level was associated with coronary artery lesion. In multivariable logistic analysis, those younger and mainly males were associated with all three outcomes of coronary artery lesion, lower serum albumin levels, and later initial use of intravenous immunoglobulin were linked to a higher risk of coronary dilation and coronary aneurysm. The potential risk factors that could be used to estimate the occurrence of coronary artery lesion in Kawasaki disease patients are young age, male, lower serum albumin lever, and later initial use of intravenous immunoglobulin. However, long-term follow-up and multi-centre studies are required to verify our findings in the future.

An infant case of coronary artery aneurysms with no systemic symptoms after treatment for Kawasaki disease.

This case is a 4-month-old patient with Kawasaki disease who showed coronary artery lesions after a disease relapse. Following the guidelines from the Randomised Controlled Trial to Assess Immunoglobulin plus Steroid Efficacy, the patient initially saw an improvement in symptoms. However, a relapse occurred despite low levels of C-reactive protein, leading to significant coronary artery growth. Treatment with a third dose of intravenous immunoglobulin and cyclosporine led to a slow reduction in the size of the coronary artery abnormalities. This case highlights the difficulty in monitoring Kawasaki disease progression through clinical symptoms and C-reactive protein levels alone. We also emphasise the necessity of echocardiographic monitoring in patients receiving anti-inflammatory treatments, including steroids, because coronary artery problems can arise or continue without the usual signs of Kawasaki disease or increased C-reactive protein levels.

Clinical and Angiographic Features of Coronary Artery Aneurysm: A Case Series from Tertiary Hospital

Clinical and Angiographic Features of Coronary Artery Aneurysm: A Case Series from Tertiary Hospital

Surgery for a giant atherosclerotic left main trifurcation saccular coronary artery aneurysm.

Aneurysms of the left main coronary artery are rare clinical entities with an incidence of 0.1%. We herein report a case of left main trifurcation aneurysm in a 57-year-old woman whose coronary angiography demonstrated a giant saccular aneurysm of trifurcation of the left main coronary artery, measuring 32 × 21mm with tight post-aneurysmal stenosis in the intermediate artery. The patient underwent coronary artery bypass grafting following the resection of CAA and reconstruction with saphenous vein roof-plasty, which ensured the preservation of the native coronary vasculature. At the 6-month follow-up, she remained asymptomatic and preserved preoperative left ventricular function.

STEMI in an Adolescent: A Critical Case of Kawasaki Disease.

Acute coronary syndrome (ACS) is a rare cause of chest pain in adolescents and young adults. Kawasaki disease (KD) is an uncommon illness, but coronary artery aneurysms secondary to KD are implicated in approximately 5% of cases of ACS in young adults. Patients with KD may present with catastrophic coronary disease at a young age and thus early recognition of this disease process and lifelong follow-up is essential. Here we discuss a 19-year-old active duty male with no previously recognized medical history who presented with crushing chest pain while deployed. He was diagnosed with an ST elevation myocardial infarction and was found to have a giant aneurysm of the left main coronary artery with obstructive thrombus treated with emergent thrombectomy and balloon angioplasty. He developed cardiogenic shock requiring mechanical circulatory and ventilatory support. After a prolonged recovery and more in-depth history-taking, he was discovered to have been diagnosed with KD as a child without follow-up. Diagnosing ACS in young adults can be challenging, but a history of KD should increase suspicion for coronary disease. This case demonstrates the importance of screening after a childhood diagnosis of KD, specifically when caring for the tactical athlete. It also highlights the impact that social determinants of health can have on patient outcomes. Finally, it demonstrates the importance of communication and coordination between military and civilian medical centers across the globe driving the care of our deployed injured and ill active-duty military personnel.

Successful Surgical Management of Multiple Giant Coronary Artery Aneurysms with Coronary Artery Fistulas.

Successful Surgical Management of Multiple Giant Coronary Artery Aneurysms with Coronary Artery Fistulas.

Role of leukocyte-associated Ig-like receptor-1 in the pathogenesis of Kawasaki disease and coronary artery aneurysms

ObjectiveTo elucidate the relationship between leukocyte-associated Ig-like receptor-1 (LAIR-1) and the pathogenesis of Kawasaki disease (KD) and coronary artery aneurysms(CAA). MethodsThe study cohort comprises children who were diagnosed with KD and were categorized into two groups: KD patients with CAA (KD-CAA) and KD without CAA (KD-NCAA), with healthy children serving as control group (HC). LAIR-1 on leukocytes was examined via flow cytometry, while serum LAIR-1 (sLAIR-1) was quantified using ELISA. IL-1β, IL-2, IL-4, IL-5, IL-6, IL-8, IL-10, IL-12p70, IL-17A, IFN-α, IFN-γ and TNF-α were examined by Immunofluorescence assay. ResultssLAIR-1 levels were elevated in the KD and KD-CAA groups compared with those in the HC and KD-NCAA groups (P < 0.05). sLAIR-1 exhibited an area under the curve value of 0.858 for predicting KD (P < 0.001) and 0.628 for predicting CAA (P = 0.055, borderline significance). LAIR-1 was increased on the neutrophils in KD group, whereas it was lower in KD-CAA than that in KD-NCAA, and decreased in KD after IVIG treatment. In contrast, LAIR-1 was reduced on CD4+ and CD8+ T lymphocyte in KD group, and increased in KD after IVIG treatment (all P < 0.05). LAIR-1 on neutrophils showed a positive correlation with IL-5, while on CD4+ T cells, it was negatively correlated with IL-2, IL-6, IL-10, IFN-γ, and IL-8. On CD8+ T cells, LAIR-1 was negatively correlated with IL-2 and IFN-γ. ConclusionsLAIR-1 may serve as a potential biomarker for KD and CAAs, while LAIR-1 might be implicated in KD pathogenesis and CAA.

Coronary Artery Vasculitis and Encasement: Multimodality Imaging Findings and Mimics.

Coronary artery vasculitis (CAV) and coronary artery encasement are rarely diagnosed conditions that are important diagnostic considerations, particularly in patients with acute coronary syndrome without traditional cardiovascular risk factors or systemic illness. Vasculitis refers to inflammation of the blood vessel walls, which can be primary or secondary. This process should be distinguished from neoplastic involvement of the coronary arteries, termed coronary artery encasement. Prospective diagnosis of these diseases is challenging, often requiring multidisciplinary workup with careful attention to clinical presentation and multiorgan findings. While CAV and coronary artery encasement can be indistinguishable at coronary CT angiography, certain imaging features help order the differential diagnosis. CAV should be considered when there is smooth wall thickening that is circumferential and/or continuous. A diagnosis of coronary artery encasement is favored when there is irregular or nodular wall thickening that is eccentric to the vessel lumen. Epicardial fat stranding may also appear more extensive compared with CAV. Potential mimics of CAV include atherosclerosis, acute plaque rupture, coronary artery aneurysm, and spontaneous coronary artery dissection. Detection and diagnosis of CAV may help avoid complications related to accelerated atherosclerosis and infarction. Radiologists should be familiar with the range of pathologic conditions that can affect the coronary arteries beyond atherosclerosis as they may be the first to raise such diagnostic possibilities, guiding next steps in patient workup and management. ©RSNA, 2024 Supplemental material is available for this article.

Factors Associated with Shock at Presentation in Kawasaki Disease Versus Multisystem Inflammatory Syndrome in Children Associated with COVID-19

While clinical overlap between Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) has been evident, information regarding those presenting with shock has been limited. We sought to determine associations with shock within and between diagnosis groups. The International KD Registry enrolled contemporaneous patients with either KD or MIS-C from 39 sites in 7 countries from 01/2020 to 01/2023. Demographics, clinical features and presentation, management, laboratory values, and outcomes were compared between the diagnosis/shock groups. Shock at presentation was noted for 19 of 672 (2.8%) KD patients and 653 of 1472 (44%; p<0.001) MIS-C patients. Within both groups, patients with shock were significantly more likely to be admitted to the intensive care unit, receive inotropes and to have greater laboratory abnormalities indicative of hyperinflammation and organ dysfunction, including abnormal cardiac biomarkers. Patients with KD and shock had a greater maximum coronary artery Z score (median +2.62) versus KD patients without shock (+1.36; p<0.001) and MIS-C patients with shock (+1.45; versus +1.32 for those without shock; p<0.001). They were also more likely to have large coronary artery aneurysms. In contrast, MIS-C patients with shock had lower left ventricular ejection fraction (mean 51.6%) versus MIS-C patients without shock (56.6%; p<0.001) and KD patients with shock (56.7%; versus 62.8% for those without shock; p=0.04). While patients with KD presenting with shock are clinically similar to patients with MIS-C, especially those with shock, they have more severe coronary artery involvement, while MIS-C patients with shock have lower left ventricular ejection fraction.

Severity of Pediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2 Diminished during Successive Waves of the COVID-19 Pandemic: Data from a Nationwide German Survey

To elucidate how the clinical presentation of Pediatric Inflammatory Multisystem Syndrome temporally associated with SARS-CoV-2 (PIMS-TS) was influenced by the successive variants of concern (VOC) and patient age. A nationwide PIMS-TS registry was established in Germany in May 2020, shortly after the first cases were described in the US and United Kingdom. The registry captured information on patient characteristics, clinical course, laboratory findings, imaging and outcome. All pediatric hospitals in Germany, along with one in Austria, were invited to participate. Between March 18, 2020, and April 30, 2023, 920 cases were reported. By examining a combination of data on clinical features, laboratory findings, treatment, imaging results, and outcomes, our analysis demonstrated disease severity to have continuously declined over the course of the Wildtype, Alpha, Delta and Omicron waves. Based on clinical symptoms, laboratory and diagnostic findings, and ICU admission rates, older children, irrespective of the related VOC, were shown to experience more severe, acute PIMS-TS; however, they had lower rates of coronary aneurysm. During the course of COVID-19 pandemic, as each new VOC emerged, PIMS-TS lessened in severity. In parallel, older children came to experience more debilitating disease.