Large Coronary Aneurysm Research Articles

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.

Pharmacogenomics of coronary artery response to intravenous gamma globulin in kawasaki disease

Kawasaki disease (KD) is a multisystem inflammatory illness of infants and young children that can result in acute vasculitis. The mechanism of coronary artery aneurysms (CAA) in KD despite intravenous gamma globulin (IVIG) treatment is not known. We performed a Whole Genome Sequencing (WGS) association analysis in a racially diverse cohort of KD patients treated with IVIG, both using AHA guidelines. We defined coronary aneurysm (CAA) (N = 234) as coronary z ≥ 2.5 and large coronary aneurysm (CAA/L) (N = 92) as z ≥ 5.0. We conducted logistic regression models to examine the association of genetic variants with CAA/L during acute KD and with persistence >6 weeks using an additive model between cases and 238 controls with no CAA. We adjusted for age, gender and three principal components of genetic ancestry. The top significant variants associated with CAA/L were in the intergenic regions (rs62154092 p < 6.32E–08 most significant). Variants in SMAT4, LOC100127, PTPRD, TCAF2 and KLRC2 were the most significant non-intergenic SNPs. Functional mapping and annotation (FUMA) analysis identified 12 genomic risk loci with eQTL or chromatin interactions mapped to 48 genes. Of these NDUFA5 has been implicated in KD CAA and MICU and ZMAT4 has potential functional implications. Genetic risk score using these 12 genomic risk loci yielded an area under the receiver operating characteristic curve (AUC) of 0.86. This pharmacogenomics study provides insights into the pathogenesis of CAA/L in IVIG-treated KD and shows that genomics can help define the cause of CAA/L to guide management and improve risk stratification of KD patients.

Real-world experience with edoxaban for anticoagulation in children at risk for coronary artery thrombosis.

Direct oral anticoagulants have the potential to improve care in children requiring chronic anticoagulation. Edoxaban has favourable pharmacokinetics that could benefit younger patients but data on long-term safety and efficacy for specific paediatric indications are lacking. We present a single-centre experience using edoxaban in children who require chronic anticoagulation for large coronary artery aneurysms secondary to Kawasaki disease. Weight-based dosing of once-daily oral edoxaban was offered as alternative to standard anticoagulation for patients aged 1-18 years. Chart review was performed for a median follow-up period of 49 months on edoxaban. Steady-state pharmacokinetics and pharmacodynamics of edoxaban were also explored. Sixteen patients on chronic therapy with edoxaban were included. No major bleeding events were reported. Two patients experienced coronary artery thrombosis after 23 and 38 months on edoxaban, 7 and 11 years after diagnosed with Kawasaki disease, respectively. This predicts 70% event-free rate at 12 years from diagnosis. Area under the curve estimates over the dosing interval of 24 hours were similar to those reported in adults. Edoxaban use is feasible and well-tolerated for long-term use in paediatric population. We suggest appropriate exposure using weight-based once-daily dosing strategy that may be comparable to standard-of-care anticoagulation in prevention of coronary artery thrombosis. Larger studies are needed to evaluate long-term safety and efficacy of edoxaban in this population.

Coronary artery ectasia associated with IgG4-related disease: a case report and literature review

BackgroundCoronary artery ectasia is defined as a local or diffuse dilatation of the coronary artery more than 1.5 times the diameter of the adjacent normal segment. The etiology of coronary artery ectasia is diverse, and rarely complicated with immunoglobulin G4-related disease (IgG4-related disease). A limited number of cases have been reported, with insidious onset, slow progression but poor prognosis.Case presentationwe report a patient with coronary artery ectasia combined with IgG4-related disease. He has been diagnosed with IgG4-related disease 5 years after his first percutaneous coronary intervention (PCI). Despite routine treatment with steroids, he develops a large coronary aneurysm and eventually died.ConclusionsIt is suggested that a thorough evaluation should be performed when coronary artery ectasia is diagnosed. The factors such as manifestations of coronary artery thickening, typical imaging features, other aortas involvement, increased serum IgG4 level, etc. should be considered for early diagnosis of key etiologies.

Large right coronary artery aneurysm with small fistula to coronary sinus: multimodality imaging.

Large right coronary artery aneurysm with small fistula to coronary sinus: multimodality imaging.

Abstract 12428: An Unwelcome House Guest: Coronary Aneurysms and Evolving Stemi

Case Description: 54-year-old man presented to the Emergency Department (ED) three weeks after Covid-19 infection for progressively worsening dyspnea and hypoxemia. Dexamethasone and prophylactic apixaban (2.5mg twice a day) were initiated and he was discharged 48 hours later. A week after discharge he re-presented to the ED requiring 6L of oxygen (O 2 ) despite uninterrupted dexamethasone and apixaban therapy. His past medical history was significant for quiescent IgG4 disease on Rituximab and Type 1 Diabetes. He was afebrile, tachycardic and tachypneic with decreased right lower lobe breath sounds. He had an elevated erythrocyte sedimentation rate and C-reactive protein, no leukocytosis and no pulmonary embolism of CT. He was admitted and vancomycin and cefepime antibiotic therapy for a superimposed bacterial pneumonia was begun. On day 12 of the hospital stay, he experienced new onset chest pain. Evaluation showed an elevated troponin and submillimeter ST segment elevation concerning for an evolving STEMI. Coronary angiography demonstrated an 90% diffuse mid LAD stenosis and two large coronary aneurysms of the left circumflex artery (LCx). The mid-LAD was stented using a 3.0 x 38 mm and 2.75 x 26 mm Onyx drug eluting stents with resolution of his chest pain. IgG4 serum level was normal and imaging did not demonstrate active IgG4 disease. He was discharged on aspirin and clopidogrel. Due to concern for a hypercoagulable state in the setting of Covid 19 infection, IgG4 disease and the large coronary aneurysms for thrombus formation, warfarin anticoagulation was also initiated. On review of his coronary imaging, the largest LCx aneurysm was 9mm on admission and 12mm three weeks later with evidence of diffuse coronary inflammation. CT Fractional Flow Reserve (abnormal ≤ 0.80) demonstrated decreased flow at the distal aneurysm with no focal stenosis to account for flow reduction. Conclusion: 54-year-old man with IgG4 disease presenting with prolonged Covid-19 infection and acute NSTEMI. He was found to have large, flow limiting coronary aneurysms and inflamed coronary arteries all consistent with his IgG4 disease. Management of these aneurysms will be discussed.

An Incidental Discovery of a Left Main Coronary Artery Aneurysm

The left main coronary artery aneurysm is a rare disease caused mainly by atherosclerosis. It is usually an incidental finding during coronary angiography but can manifest itself with ischemic heart disease symptoms. In our paper, we present the case of a polyvascular patient who benefited from a coronary angiography in the context of the etiological workout of his dilated cardiomyopathy, which lead to the discovery of a large left main coronary artery aneurysm associated with two-vessel coronary disease. Through our paper, we aim to discuss the different aspects of this condition and its different treatment options.

Long-term results of large common iliac artery aneurysms caused by Kawasaki disease in four patients.

Among Kawasaki disease patients with systemic artery aneurysms, the brachial and internal iliac arteries are the most commonly affected, and occlusions of both arteries are often found. However, the long-term fate of large common iliac artery aneurysms remains unknown, because their prevalence is very low. The long-term outcomes of common iliac artery aneurysms caused by Kawasaki disease in four patients (three females, one male) were investigated retrospectively based on their medical records and angiograms. Their ages ranged from 30 to 36 years-old. The onset age of Kawasaki disease ranged from 4 to 8 months, and the interval from the onset of Kawasaki disease to the latest angiogram ranged from 17 to 21 years. All patients had bilateral large coronary aneurysms and common iliac artery aneurysms with maximal diameters greater than 10 mm. Although all patients had multi-vessel coronary artery stenotic lesions and systemic artery aneurysms, they were asymptomatic. The three female patients underwent coronary artery bypass grafting, and the male patient underwent replacement of artificial vessels for large bilateral common iliac artery aneurysms at 3 years old of age. Over the long-term, common iliac artery aneurysms greater than 10 mm persisted as calcified aneurysms. However, they had no symptoms due to their common iliac artery aneurysms, and their ankle brachial pressure index was preserved, even if the stenosis of the common iliac artery developed as a late outcome, because the collateral arteries were well developed. The progression of stenosis of the common iliac artery after Kawasaki disease was slower.

A practical nomogram for predicting coronary thrombosis for Kawasaki disease patients with medium or large coronary artery aneurysm.

Kawasaki disease (KD) is the main cause of acquired heart disease in children. Coronary thrombosis is a serious cardiovascular complication of KD, which affects the long-term treatment effect. The purpose was to develop and validate a model for predicting coronary thrombosis in KD with medium or large coronary artery aneurysm (CAA). A total of 358 consecutive KD patients with medium or large CAA from Chongqing Children's Hospital were enrolled retrospectively. The demographic data, clinical characteristics, laboratory features before intravenous immunoglobulin (IVIG) treatment, and all radiological features during hospitalization and follow-up were collected. Eligible patients follow-up for > 2years. Follow-up was weekly for the first 1month, monthly for the next 11months, and every 3-6months after 1year. The main examinations included echocardiogram and electrocardiogram. The primary endpoint was defined as coronary thrombosis during the follow-up. Coronary thrombosis was assessed by echocardiographic assessment of the presence of echoes in the lumen of the right coronary artery, left main coronary artery, left anterior descending artery, or left circumflex artery by echocardiologists. The independent risk factors were identified using univariate analyses and multivariate logistic regression analyses, and the nomogram was constructed for predicting coronary thrombosis. Tenfold cross-validation was used to perform internal validation. The area under the ROC curve (AUC), calibration curve, and decision curve analysis were used to evaluate the discrimination, calibration, and clinical utility of the nomogram, respectively. Multivariate logistic regression analysis revealed that male (odds ratio [OR] 3.491; 95% confidence interval [CI] 1.570-7.765), large CAA (OR 3.725; 95% CI 1.388-9.999), no use high-dose aspirin prior to IVIG (OR 3.114; 95% CI 1.291-7.510), two-vessel coronary artery involvement (OR 4.433; 95% CI 1.732-11.344), three-vessel coronary artery involvement (OR 5.417; 95% CI 2.048-14.328), four-vessel coronary artery involvement (OR 13.183; 95% CI 3.408-50.997), serum fibrinogen level > 5.325g/L (OR 14.233; 95% CI 5.479-36.921), serum thrombin time level ≤ 15.15s (OR 3.576; 95% CI 1.756-7.284) were significantly associated with coronary thrombosis. The nomogram was established based on these variables. The AUC of the nomogram were 0.920, and tenfold cross-validation (repeated 100 times) showed that the average AUC was 0.902. Moreover, the nomogram had a well-fitted calibration curve and also exhibited good clinical usage. The nomogram is based on six ready-made clinical variables, is easy to use, has excellent diagnostic performance, and can help clinicians make better clinical decisions on the management and treatment of KD patients with medium or large CAA.

Identifying children with Kawasaki disease at high risk for coronary aneurysms

Kawasaki disease, a childhood vasculitis manifested by symptoms of high fever, mucocutaneous inflammation, and unilateral cervical adenopathy, can cause lasting damage to the coronary arteries, and more rarely, other medium-sized muscular arteries. Most children respond well to prompt treatment with high-dose intravenous immunoglobulin (IVIG). However, coronary artery aneurysms, defined by internal lumen diameter at least 2.5 SDs above the population mean for body surface area (Z≥2.5), occur in ∼25% of affected children and are the principal cause of long-term morbidity and mortality in Kawasaki disease. Several risk scores have attempted to predict which children are likely to develop coronary aneurysms. In previous studies in North America, baseline risk factors for coronary artery aneurysms on echocardiography performed 2-8 weeks after Kawasaki disease onset have included higher coronary artery dimensions on baseline echocardiography, IVIG resistance (the persistence or recrudescence of fever after a single dose of IVIG), young age (<12 months or <6 months depending upon the risk score), Asian race, and late diagnosis and treatment with IVIG. Japanese risk scores based upon clinical and laboratory variables have been highly accurate in predicting IVIG resistance, one of the strongest risk factors for coronary artery aneurysms. In Japanese children predicted to have high risk of IVIG resistance, primary adjunctive therapy with corticosteroids or cyclosporine has been shown in randomized clinical trials to reduce the incidence of coronary artery aneurysms. However, Japanese children predicted to be at low risk for aneurysms on the basis of laboratory measures may still develop coronary abnormalities. Understanding the risk factors for aneurysm development thus requires further refinement. In this volume of The Journal, Iio et al analyze independent risk factors for development of coronary artery aneurysms in 1632 Japanese children with Kawasaki disease who were predicted to be at low risk for resistance to primary IVIG treatment. In this low-risk group, 5.5% of patients developed any coronary artery aneurysms and 1%, medium or large coronary aneurysms. In multivariable analysis, baseline coronary artery Z score >2.5, age <12 months at fever onset, and IVIG resistance were independent risk factors for diagnosis of coronary artery aneurysms on echocardiography one month after disease onset. Among these risk factors, coronary artery Z score ≥2.5 was most strongly associated with aneurysm development. Taken together with previous literature, these data suggest that enlarged coronary Z scores measured on baseline echocardiography can be used to select children at high risk for subsequent coronary artery aneurysms and thus who may benefit from adjunctive therapy. The study of Iio et al adds weight to the concept that a high-risk cohort for randomized clinical trials of primary adjunctive therapy in Kawasaki disease may be most simply selected through findings on baseline echocardiography. The ability to select such a high-risk cohort is critical to the design of trials to answer remaining questions in Kawasaki disease therapy: In children of all races and ethnicities, who should receive treatment with adjunctive therapies? How do antiinflammatory therapies compare with each other, and what is the cost/benefit ratio for these therapies? Article page 158 Risk Factors of Coronary Artery Aneurysms in Kawasaki Disease with a Low Risk of Intravenous Immunoglobulin Resistance: An Analysis of Post RAISEThe Journal of PediatricsVol. 240PreviewTo detect risk factors of coronary artery aneurysm (CAA) development in patients with Kawasaki disease determined to have a low risk for resistance to primary intravenous immunoglobulin (IVIG) treatment based on the Kobayashi score. Full-Text PDF

Thromboembolic ST elevation myocardial infarction due to a large coronary aneurysm: Role of apixaban.

Thromboembolic ST elevation myocardial infarction due to a large coronary aneurysm: Role of apixaban.

Falling Through the Cracks: The Current Gap in the Health Care Transition of Patients With Kawasaki Disease: A Scientific Statement From the American Heart Association.

BackgroundHealth care transition (HCT) is a period of high vulnerability for patients with chronic childhood diseases, particularly when patients shift from a pediatric to an adult care setting. An increasing number of patients with Kawasaki disease (KD) who develop medium and large coronary artery aneurysms (classified by the American Heart Association according to maximal internal coronary artery diameter Z‐scores ≥5 and ≥10, respectively) are becoming adults and thus undergoing an HCT. However, a poor transition to an adult provider represents a risk of loss to follow‐up, which can result in increasing morbidity and mortality.Methods and ResultsThis scientific statement provides a summary of available literature and expert opinion pertaining to KD and HCT of children as they reach adulthood. The statement reviews the existing life‐long risks for patients with KD, explains current guidelines for long‐term care of patients with KD, and offers guidance on assessment and preparation of patients with KD for HCT. The key element to a successful HCT, enabling successful transition outcomes, is having a structured intervention that incorporates the components of planning, transfer, and integration into adult care. This structured intervention can be accomplished by using the Six Core Elements approach that is recommended by the American Academy of Pediatrics, the American Academy of Family Physicians, and the American College of Physicians.ConclusionsFormal HCT programs for patients with KD who develop aneurysms should be established to ensure a smooth transition with uninterrupted medical care as these youths become adults.

Effectiveness and safety of dual antiplatelet therapy in coronary aneurysms caused by Kawasaki disease in children: study protocol for a multicenter randomized clinical trial.

BackgroundMedium or large coronary artery aneurysm (CAA) is a serious complication of Kawasaki disease (KD) and may cause thrombosis, coronary artery stenosis, and even myocardial infarction at different stages of the disease. Dual antiplatelet therapy (aspirin plus clopidogrel) is considered for prophylaxis of coronary thrombosis in KD presenting with more than medium CAAs based on adult coronary artery disease experience and expert consensus. This paper describes the study protocol for a randomized controlled trial that measures the clinical effectiveness and safety of dual antiplatelet therapy in the thromboprophylactic treatment of KD.MethodsThe present study is a multicenter, open-label, randomized controlled trial (RCT) with a 12-month follow-up. KD patients with medium or large CAAs (Z-value ≥5), evaluated by echocardiogram and clinicians before treatment, are randomized in a 1:1 ratio and assigned to aspirin therapy alone (Control group), or dual antiplatelet (aspirin plus clopidogrel) therapy (Experimental group). Antiplatelet therapy is given to the KD patients from the time of diagnosis until the coronary artery returns to normal. Weekly or monthly follow-up visits are conducted to record compliance, recovery, and biochemical indicators and continue for one year. The primary outcome is the incidence of thrombus throughout the disease and the effective of dual antiplatelet. The secondary outcomes are the safety of dual antiplatelet drugs, platelet-related indicators, inflammatory indicators, biochemical indexes and drug-related indicators during the study period. Patients who do not meet the inclusion criteria of the RCT trial or those unwilling to provide informed consent enter the registration trial.DiscussionThis is the first study to evaluate the effectiveness and safety of dual antiplatelet therapy in coronary aneurysms caused by Kawasaki disease in children. It is hoped that this study will play an important and significant role in improving the prognosis and long-term quality of life for children with KD complicated by CAAs.Trial registrationChinese Clinical Trial Registry, ChiCTR1800019181. Registered on Oct 30, 2018.

Coronary artery changes in congenital coronary-cameral fistulas evaluated by computed tomographic angiography.

The study sought to determine coronary artery diameter in congenital coronary-cameral fistula (cCCF), factors associated with coronary artery aneurysm, coronary artery changes after fistula closure, and computed tomographic (CT) findings after treatment. We retrospectively reviewed CT findings of the cCCF for origins, terminations, fistula length, complexities, and Sakakibara classification. Coronary artery diameter was expressed as coronary artery Z score. Fistula features associated with coronary artery aneurysm were analyzed. Post-fistula closures were analyzed for coronary artery dilatation, coronary thrombosis, complete fistula closure, and fistula thrombosis. Twenty-five patients (median age 33months, interquartile range, IQR 25-48) were included. Coronary feeders and terminations were frequently right coronary artery (48%) and right ventricle (56%), respectively. Fistula aneurysm occurred in 52% of cases. Mean coronary artery Z score was 13.03 ± 6.36 with a high incidence of giant coronary artery aneurysm (68%). We found no statistically significant risk factors associated with coronary artery aneurysm (p value range 0.075-0.370). Median duration of the follow-up CT after closure of the fistulas was 6.4months (IQR 5.0-8.7). Coronary artery Z score significantly decreased by 0.82 (IQR 0.28-1.35), p = 0.006 and coronary thrombosis occurred in 23% of cases during follow-up. Large coronary aneurysm is common in cCCF. No characteristic feature of the fistula influencing coronary artery aneurysm is identified. There is a diminution in coronary artery Z score after fistula closure. Coronary thrombosis is a major complication after treatment.

Giant coronary artery aneurysms involving more than one coronary artery: case report

BackgroundCoronary artery aneurysms are rare findings in patients undergoing coronary angiography. The presence of multiple coronary artery aneurysms located in more than one coronary artery is even more uncommon. The pathophysiology of such aneurysms is unknown, but the majority are often due to atherosclerosis, congenital heart disease, or vasculitis.Case presentationWe present a rare case of a 78-year-old female patient who presented with unstable angina and non-ST segment elevation myocardial infarction. On coronary angiography, she was found to have three separate 1 cm saccular aneurysms involving the proximal left anterior descending coronary artery. The right coronary artery could not be visualized. Computed chest tomography revealed a 6.6 × 6.3 cm saccular aneurysm of the right coronary artery, and a 4.4 cm fusiform aneurysm of the ascending aorta. The patient gave no history of percutaneous coronary intervention or cardiac surgical procedures. She had a previous history of endovascular stenting of an abdominal aortic aneurysm. The sizable right coronary artery aneurysm showed extrinsic compression of both the right atrium and ventricle with right ventricular hypokinesis. Serological studies for vasculitis were all negative. Pathology of the aneurysm wall revealed calcific atherosclerosis without evidence of vasculitis. The patient underwent subtotal resection of the right coronary aneurysm with ligation of the proximal and distal ends of the right coronary artery and double bypass surgery to the left anterior descending and right posterior descending coronary arteries.ConclusionThe presence of multiple, large coronary artery aneurysms is very rare. Treatment can be challenging and should be individualized. Surgical treatment is recommended for giant coronary artery aneurysms to prevent potential complications.

276 Management of Giant Coronary Artery Aneurysms as a Coronary Sequelae of Kawasaki Disease – Surgical or Conservative Management?

Abstract Introduction Kawasaki Disease (KD) is the commonest childhood acquired heart disease in developed countries, predominantly affecting children younger than 5 years of age. These patients are at high risks of developing coronary arterial aneurysms (CAA) and CAA with absolute internal luminal diameters measuring ≥8mm or Z-score ≥10 is classified as giant coronary aneurysms (GCA). Case Report A 19-year-old girl with previous history of Kawasaki disease was diagnosed with giant coronary aneurysms on echocardiograms. Her subsequent CT coronary angiography showed large aneurysms in the right coronary artery (29mm x 25mm) and left anterior descending artery (10mm x 10mm) with signs of calcification. However, surprisingly, despite the evidence of large coronary aneurysms, she was completely asymptomatic and was physically active. Her cardiac MRI scan showed right coronary perfusion deficit in the distal right coronary territory but with no evidence of myocardial infarction. After having a detailed discussion in the MDT and with colleagues from San Diego, USA, a conservative approach was decided to be in the patient’s best interest. She has been advised to avoid competitive or strenuous exercise due to the associated risks. She is fit-and-well and is currently on Warfarin variable dose and Aspirin 75mg OD, with annual cardiac MRI surveillance.

Coronary aneurysm in a young patient with Turner syndrome.

A young woman with Turner syndrome was found to have a large coronary aneurysm in the left anterior descending coronary artery upon CT angiogram screening for aortic pathology.

P76 Management of Giant Coronary Artery Aneurysms as a Coronary Sequelae of Kawasaki Disease – Surgical or Conservative Management?

Abstract Introduction Kawasaki Disease (KD) is the commonest childhood acquired heart disease in developed countries, predominantly affecting children younger than 5 years’ of age. These patients are at high risks of developing coronary arterial aneurysms (CAA) and CAA with absolute internal luminal diameters measuring ≥8mm or Z-score ≥10 are classified as giant coronary aneurysms (GCA). Case Report A 19-year-old girl with previous history of Kawasaki disease was diagnosed with giant coronary aneurysms on echocardiograms. Her subsequent CT coronary angiography showed large aneurysms in the right coronary artery (29mm x 25mm) and left anterior descending artery (10mm x 10mm) with signs of calcification. However, surprisingly, despite the evidence of large coronary aneurysms, she was completely asymptomatic and was physically active, participating in competitive sports. Her cardiac MRI scan 17 years after KD onset showed right coronary perfusion deficit in the distal right coronary territory but with no evidence of myocardial infarction. A repeated cardiac CT was performed and showed delayed filling of the distal right coronary artery. However, it was uncertain at this point if the aneurysm was causing true ischaemia or simply delayed perfusion. An ECG stress test was subsequently performed for further investigation which showed no significant abnormality. After having a detailed discussion in the MDT and with colleagues from San Diego, USA, a conservative approach was decided to be in the patient’s best interest. She has been advised to avoid competitive or strenuous exercise due to the associated risks. She is fit-and-well, and is currently on Warfarin variable dose and Aspirin 75mg OD, with annual cardiac MRI surveillance. Discussion 20% of KD patients develop CAA and approximately 5% of KD patients have evidence of GCA. Unlike small and medium CAA, GCA regress in a much slower and more constant rate, and they never achieve complete resolution. A Japanese retrospective study showed the 10-year coronary event-free survival rates in small, medium and giant CAA as 100%, 96% and 61% respectively (p&amp;lt;.001). Patients with GCA are at significantly increased risks of coronary complications and major cardiac events (48%). Despite maximal medical treatment, patients with GCA have high risks of aneurysm rupture which is associated with high mortality rates. These patients are also at lifelong risks of atherosclerosis and stenosis which may lead to myocardial ischaemia and even death. Therefore, it is absolutely essential to control for cardiovascular risk factors and adhere to strict treatment regime. These patients also require lifelong, uninterrupted cardiology follow-up in a specialist KD clinic. Catheter and surgical interventions may be indicated in some patients. There have been recent attempts in combining CABG with downsizing reconstruction for GCA in an attempt to decrease GCA diameter and improve the coronary flow rate to prevent thrombosis which has shown quite promising results. However, the decisions whether to undergo a surgical intervention should be tailored to each individual, taking into consideration all aspects of a patient, including their disease status and social life. Conclusion With an increasing prevalence of KD in UK, it is essential to always consider the diagnosis of KD in a febrile child with raised inflammatory markers, especially in patients presenting with prolonged fever (longer than 4-5 days) as a delay in commencing treatment poses a significantly increased risk of developing coronary complications which are associated with high morbidity and mortality rates.

Large Multi-Vessel Coronary Artery Aneurysms: An Unusual Cause of Chest Pain

Coronary artery aneurysms are an uncommon finding in adulthood and are often incidentally diagnosed. We present a case of large multi-vessel coronary artery aneurysms likely due to childhood Kawasaki disease.

Low-Molecular-Weight Heparin vs Warfarin for Thromboprophylaxis in Children With Coronary Artery Aneurysms After Kawasaki Disease: A Pragmatic Registry Trial

BackgroundThe substantial risk of thrombosis in large coronary artery aneurysms (CAAs) (maximum z-score ≥ 10) after Kawasaki disease (KD) mandates effective thromboprophylaxis. We sought to determine the effectiveness of anticoagulation (low-molecular-weight heparin [LMWH] or warfarin) for thromboprophylaxis in large CAAs. MethodsData from 383 patients enrolled in the International KD Registry (IKDR) were used. Time-to-event analysis was used to account for differences in treatment duration and follow-up. ResultsFrom diagnosis onward (96% received acetylsalicylic acid concomitantly), 114 patients received LMWH (median duration 6.2 months, interquartile range [IQR] 2.5-12.7), 80 warfarin (median duration 2.2 years, IQR 0.9-7.1), and 189 no anticoagulation. Cumulative incidence of coronary artery thrombosis with LMWH was 5.7 ± 3.0%, with warfarin 6.7 ± 3.7%, and with no anticoagulation 20.6 ± 3.0% (P < 0.001) at 2.5 years after the start of thromboprophylaxis (LMWH vs warfarin HR 1.5, 95% confidence interval [CI] 0.4-5.1; P = 0.56). A total of 51/63 patients with coronary artery thrombosis received secondary thromboprophylaxis (ie, thromboprophylaxis after a previous thrombus): 27 LMWH, 24 warfarin. There were no differences in incidence of further coronary artery thrombosis between strategies (HR 2.9, 95% CI 0.6-13.5; P = 0.19). Severe bleeding complications were generally rare (1.6 events per 100 patient-years) and were noted equally for patients on LMWH and warfarin (HR 2.3, 95% CI 0.6-8.9; P = 0.25). ConclusionsLMWH and warfarin appear to have equivalent effectiveness for preventing thrombosis in large CAAs after KD, although event rates for secondary thromboprophylaxis and safety outcomes were low. Based on our findings, all patients with CAA z-score ≥ 10 should receive anticoagulation, but the choice of agent might be informed by secondary risk factors and patient preferences.