Search

Objective: Rare disease. Background: Coronary artery aneurysms in patients with Kawasaki disease may develop acute myocardial infarction. It is challenging to achieve complete revascularization solely through percutaneous coronary intervention in these patients. Therefore, coronary artery bypass grafting is often necessary. Case Report: We present a case of a 68-year-old woman who developed multiple acute myocardial infarctions due to giant aneurysms formed in the right coronary artery (RCA) and the left circumflex artery (LCx). We diagnosed the cause of the aneurysms as Kawasaki disease based on the coronary angiogram, laboratory results, and family history. After the primary balloon angioplasty, we conducted coronary artery bypass grafting, which involved grafting 2 vessels to the LCx and 1 vessel to the RCA. The internal thoracic arteries, which are the standard graft vessels, were occluded, most likely due to Kawasaki disease vasculitis. Instead, we used saphenous vein grafts harvested using the "no-touch" technique, which preserves the perivascular adipose tissue, to improve the long-term patency. In addition, we ligated the LCx aneurysm to prevent occlusion of the grafts and rupture of the aneurysm. Four years after the uneventful discharge, the patient is in good health and coronary computed tomography angiography revealed good patency of all grafts. Conclusions: This report highlights a successful combination of "no-touch" saphenous vein grafting and coronary aneurysm ligation in an adult patient with Kawasaki disease. These techniques may be especially useful for this vasculitic illness which is often associated with occlusion of internal thoracic arteries. [ABSTRACT FROM AUTHOR]

While Kawasaki disease (KD) induced coronary artery aneurysms (KD CAAs) in children are well studied, the features and prognosis of non-KD induced CAAs (non-KD CAAs) in the pediatric population are poorly documented. This case series study is to analyze the etiology and prognosis of non-KD CAAs in children and compare the characteristics of non-KD CAAs and KD CAAs. Non-KD CAA and KD CAA cases at our department from January 2022 to December 2023 were retrospectively collected. Etiologies and prognosis of non-KD CAAs were analyzed. Furthermore, demographic data, biochemical parameters and outcomes between children with Non-KD CAAs and children with KD CAAs were comparatively studied. Fifteen children with non-KD CAAs with a median age of 6 years and 117 children with KD CAAs with a median age of 2.0 years (p = 0.022) were included in this study. The causes of non-KD CAAs include: unknown etiologies (2 cases), coronary artery structural abnormalities (4), Takayasu arteritis (2), virus infection (2), cardiomyopathy (2), aplastic anemia with agranulocytosis (1), ANCA-associated vasculitis (1), and mucopolysaccharidosis (1). In the non-KD CAA group, there were a total of 19 CAAs with 3 being giant, 5 medium, and 11 small; 4 patients had complete CAA regression; an infant with a fistula between the right coronary artery and the coronary sinus complicated with cardiac enlargement died of heart failure. The KD group had significantly higher levels of CRP, white cells counts and ESR with zero mortality. Non-KD CAA cases had a significantly lower regression rate than KD-CAA cases (26.7% vs 66.7%, p = 0.004), and the probability of CAA regression in non-KD patients was 0.341 of that in KD patients (p = 0.006, OR = 0.341, 95% CI: 0.179–0.647). Conclusions: Various etiologies for Non-KD CAAs are identified. Patients with Non-KD CAAs were observed to have lower inflammatory indexes but poorer recovery than patients with KD CAAs. Therapeutic strategies different than those for KD may be needed for non-KD CAAs. What is Known: • Coronary artery aneurysm (CAA) in children is most commonly induced by Kawasaki disease (KD CAA), with a 50 ~ 70% regression rate in 1 to 2 years. • CAA induced by diseases other than KD (non-KD CAA) in children is rare and its prognosis remains largely unknown. What is New: • Most non-KD CAA cases are caused by coronary artery structural malformations. • Non-KD CAA in children has poorer prognosis and lower regression rate compared with KD CAA. • In addition to guideline directed anti-platelet and anti-coagulant therapies, treatments targeting the causal factor are necessary for non-KD CAA. [ABSTRACT FROM AUTHOR]

ObjectiveChildren with Kawasaki disease (KD) and an initial echocardiogram that demonstrates coronary artery aneurysms (CAAs, Z score ≥2.5) are at high risk for severe cardiovascular complications. We sought to determine if primary adjunctive infliximab treatment at a dose of either 5 or 10 mg/kg, compared with intravenous immunoglobulin (IVIG) alone, is associated with a greater likelihood of CAA regression in patients with KD with CAA at the time of diagnosis.Design and settingSingle-centre observational study.PatientsChildren with acute KD and Z score ≥2.5 at baseline.InterventionsPrimary adjunctive infliximab (5 or 10 mg/kg) within 48 hours of initiating IVIG 2 g/kg.Main outcome measuresIncidence of CAA regression to Zmax

Multisystem Inflammatory Syndrome in Adults (MIS-A) is a rare complication after COVID-19 that mainly occurs in young adults. Patients typically present with unremitting fever, rash, conjunctivitis, neurological signs, shock, gastrointestinal symptoms and thrombocytopenia. Reported cases are scarce. Here we describe three new cases.It is unclear to what extent MIS-A has a genetic basis, or whether MIS-A patients can be safely vaccinated after a case of MIS was reported after vaccination (termed MIS-V). We describe a monozygotic twin who was vaccinated without complications, suggesting no strict genetic basis for MIS-V. Furthermore, we report only the second case of MIS-A-related coronary aneurysm, which fully resolved upon regular treatment.With the majority of young adults living in low resources settings, we suggest more focus on clinical parameters to support a MIS-A diagnosis. We report the first two patients of North-African descent. Currently, MIS-A may be an underappreciated complication of COVID-19 due to the lack of reports in non-Caucasian populations.

Aneurysms of the coronary arteries and abdominal aorta are extremely rare in infancy. Due to the rarity of the anomaly, there are no existing guidelines on management of these aneurysms. We describe the challenges in diagnosis, evaluation and management of an infantile Marfan with this rare presentation. [ABSTRACT FROM AUTHOR]

Extraluminal migration of a drug-eluting intracoronary stent into pericardial space with or without aneurysm formation is a rare complication. It is usually symptomatic although rare asymptomatic cases have been described. Herein, we report a 49-year-old male who presented with atypical chest pain 9 years after intracoronary stent implantation in the right coronary artery and the ramus intermediate branch. Echocardiographic examination showed a 7.4 cm × 3.8 cm large oblong cystic mass along the lateral wall of the left ventricle with inward bulging of the adjacent segments. Computed tomography and invasive coronary angiography showed this mass to be a partly thrombosed giant oblong coronary aneurysm arising from the bifurcation of the left main coronary artery with extraluminal migration of the stent from the ramus branch.

The most significant sequelae of Kawasaki disease (KD) are coronary artery aneurysms, which can lead to risk of future myocardial ischemia. Exercise stress echocardiography allows for non-invasive assessment of myocardial dysfunction. We reviewed our single center experience with exercise stress echocardiography in patients with previous history of KD with coronary aneurysms. We reviewed the records of 53 KD patients who underwent exercise stress echocardiography from 2000 to 2020. Abnormal stress echocardiograms were defined as those showing no increase in biventricular systolic function post-exercise or regional wall motion abnormalities. Computed tomography angiography and cardiac magnetic resonance imaging were reviewed for patients with abnormal stress echocardiograms. Clinical data were reviewed and correlated with stress echocardiogram results. Of the 53 patients, three (5.7%) had an abnormal exercise stress echocardiogram. All three patients were classified as AHA Risk Level 4 or 5 by coronary Z-score (internal dimension normalized for body surface area) and were confirmed to have coronary aneurysms, stenosis, or myocardial tissue perfusion defects on advanced cardiac imaging that could account for the results seen on stress echocardiogram. Exercise stress echocardiography detected signs of myocardial ischemia in a subset of high-risk patients with Kawasaki disease and coronary aneurysms and may be considered as a useful screening tool for this complex patient cohort.

Objectives: To assess the scan time, image quality, and diagnostic performance of self-navigated coronary MR angiography (SN-CMRA) for coronary aneurysm (CAA) detection in Kawasaki disease (KD) patients and compare it with diaphragm-navigated CMRA (DN-CMRA). Materials and methods: SN-CMRA and DN-CMRA were performed on 76 pediatric patients with KD (48 males, 6.75 ± 3.59 years). Thirty-three of whom underwent coronary CT angiography (CCTA)/invasive coronary angiography (ICA). The scan time and qualitative and quantitative image quality assessment were compared between the two sequences. The diagnostic performance for CAA detection by the two approaches using CCTA/ICA as the reference standard was compared on per-patient, per-vessel, and per-segment basis. Results: The scan time of SN-CMRA was significantly shorter than that of DN-CMRA (7.49 ± 2.31 min vs. 10.03 ± 4.47 min, p < 0.001). There was no difference in overall and segmental image quality to reach the clinical diagnostic criteria between the two sequences (all p > 0.05). No significant difference in vessel length of the three main coronary arteries was found between the two approaches (all p > 0.05). Moreover, SN-CMRA showed no difference from DN-CMRA in contrast ratio of blood-myocardium (1.25 (interquartile range [IQR], 1.06 to 1.51) vs. 1.18 (IQR, 0.95 to 1.64), p = 0.706). There was no difference in the diagnostic accuracy of SN-CMRA and DN-CMRA for CAA detection on per-patient, per-vessel, or per-segment basis (all p > 0.05). Conclusion: SN-CMRA at 3T showed reliable diagnostic performance and application value for CAA detection in children with KD. Compared with DN-CMRA, SN-CMRA can simplify the scanning procedure and shorten the scan time, achieving comparable image quality and diagnostic accuracy. Clinical relevance statement: Coronary aneurysm in children with Kawasaki disease (KD) can be detected by self-navigated coronary MR angiography (CMRA) non-invasively and without radiation, achieving comparable image quality and diagnostic performance as diaphragm-navigated CMRA while shortening scanning time. It can provide reference for risk stratification and treatment management of KD. Key Points: • Evaluating the size of coronary aneurysm is important for risk stratification and treatment of Kawasaki disease. • Self-navigated coronary MR angiography (SN-CMRA) shortens scan time and achieves comparable image quality and diagnostic performance compared with diaphragm-navigated coronary MR angiography. • SN-CMRA can evaluate coronary aneurysm non-invasively and without radiation, providing information for risk stratification and treatment. [ABSTRACT FROM AUTHOR]

Background Nail–Patella syndrome (NPS) is an autosomal-dominant pleiotropic condition characterized by pelvic and skeletal abnormalities and most commonly affecting a tetrad of nails, knees, elbows, and iliac horns, the iliac horns being pathognomonic for the condition. The most well-documented extra-skeletal manifestation is renal involvement with alteration in Type III collagen. No documented cases of NPS with anomalous coronary arteries or aneurysms, acute coronary occlusion, or successfully coronary interventions exist in the medical literature. Case summary A 62-year-old female with a medical history significant for NPS diagnosed 50 years ago presented to the emergency department with a chief complaint of chest pain. She recently developed end-stage renal disease managed with peritoneal dialysis within the last year. Angiography revealed 100% right coronary artery occlusion with an anomalous take-off from the left circumflex artery. She demonstrated diffuse coronary aneurysms in the right coronary artery, mid-left anterior descending artery, and other epicardial vessels. Two drug-eluting stents were placed in overlapping fashion. Following careful apposition, the aneurysmal segment was successfully stented without complication. The patient was discharged without complication 2 days later. Discussion Our case shows the first reported case of coronary vascular anomalies and successful coronary revascularization in a patient with NPS in the medical literature. Given the recently reported vascular anomalies and known collagen alterations seen in patients with the genetic disorder, clinicians should suspect further systemic vascular anomalies with their own unique therapeutic challenges when encountering patients with this rare genetic syndrome. [ABSTRACT FROM AUTHOR]

Cardiac tamponade is a rare postoperative complication of esophagectomy, with no previous reports of association with coronary artery aneurysm rupture. We present a case of cardiac tamponade caused by coronary aneurysm rupture following esophageal cancer surgery. A 68-year-old man with no history of heart disease underwent robotic subtotal esophagectomy for esophageal squamous cell carcinoma. He experienced intermittent chest pain on postoperative day (POD) 17. Echocardiography revealed increasing pericardial fluid, and pericardiocentesis on POD 34 revealed bloody pericardial fluid. Contrast-enhanced computed tomography and coronary angiography revealed a ruptured coronary aneurysm causing cardiac tamponade. Emergency surgery with a median sternotomy achieved hemostasis, and the patient recovered successfully. Cardiac tamponade after esophageal surgery, particularly from coronary aneurysm rupture, is rare. Prompt diagnosis and treatment are crucial for patient survival. Despite its risks, median sternotomy was effective in achieving rapid hemostasis and patient recovery in this case. [ABSTRACT FROM AUTHOR]

Cogan syndrome (CS) is a chronic inflammatory disorder primarily affecting young adults. It is characterized by interstitial keratitis, vestibuloauditory dysfunction, and, rarely, systemic vasculitis and aortitis. In this case report, we present a rare instance of a young male with anterior ST-elevation myocardial infarction in the context of aneurysmal coronary vessels and proximal occlusion of the left anterior descending artery. This case underscores the importance of recognizing cardiac risk factors in the setting of CS, as they can be life-threatening. Regular follow up is necessary, especially for patients showing signs of vasculitis or experiencing cardiac symptoms. A multidisciplinary approach to management can help optimize the prognosis for these complex patients. Comprehend Cogan syndrome (CS): Grasp the clinical manifestations and potential cardiovascular complications of CS. Diagnostic approach in CS: Differentiate cardiac symptoms in CS from typical coronary diseases, emphasizing the role of heart catheterization. Multidisciplinary management: Recognize the importance of integrating rheumatologists, cardiologists, and surgeons for optimal CS patient outcomes. [ABSTRACT FROM AUTHOR]

Background Although the technology of bioresorbable vascular scaffold (BVS) aroused the peak of interest a few years ago and currently remains available only as part of experimental research, patients who have had BVS implanted should be still carefully monitored to detect possible long-term complications. Case summary We present the case of a 47-year-old man who had received BVS implantation for ST-segment elevation myocardial infarction. Six years later, computed tomography coronary angiography (CTCA) demonstrated in-segment restenosis in between two newly formed coronary aneurysms at the site of the implanted BVS. The patient received successful optical coherence tomography–guided percutaneous intervention with a new metallic drug-eluting stent implantation. Discussion Our case demonstrates that coronary aneurysms can be well characterized with CTCA and are often incidentally discovered as they cause no symptoms. The incidence of coronary aneurysm at the site of a previously implanted BVS is not defined, and little is known about the pathophysiology and evolution of these lesions. Therefore, the decision to proceed with conservative management or intervention must be tailored to the clinical conditions of the patient, the anatomy, the rapidity of growth, and the possible thrombotic burden. [ABSTRACT FROM AUTHOR]

Cardiac involvement (CI) is rare in Behçet syndrome (BS), but the important point is that CI may be the first manifestation of the disease. The presence of CI worsens the prognosis of BS, so early diagnosis and early initiation of immunosuppressive treatment (IST) are vital. Coronary aneurysm may develop spontaneously in these patients, or any vascular intervention may cause aneurysm with a pathergy‐like reaction. The risk of restenosis is high after percutaneous coronary intervention or coronary artery bypass surgery applied without IST. Therefore, it should be kept in mind that IST constitutes the main step of treatment. Herein, we present a young male diagnosed with BS after acute coronary syndrome caused by coronary artery aneurysms and thrombosis. [ABSTRACT FROM AUTHOR]

Polyarteritis nodosa (PAN) is a medium-vessel vasculitis presenting with cutaneous and multisystem involvement with considerable morbidity. The necrotizing vasculitis in PAN typically involves renal, celiac, and mesenteric vascular beds. Coronary artery involvement is a characteristic feature of Kawasaki disease, another medium-vessel vasculitis; however, it has been rarely reported with PAN. Here, we present 2 cases with PAN involving coronaries mimicking Kawasaki disease. A 3.5-year-old boy with classical features of Kawasaki disease with giant coronary aneurysm refractory to IVIg, methylprednisolone, infliximab presented with persistent rise in inflammatory markers and gastrointestinal bleeding. Digital subtraction angiography (DSA) revealed celiac artery branches stenosis and beading suggestive of PAN. Another 2-year-old girl presented with persistent fever, abdominal pain, and distension. She had hypertension, hepatomegaly, and splenomegaly on examination. Echocardiography revealed multiple coronary aneurysms and DSA revealed numerous renal artery aneurysms. Coronary aneurysm although is a rare presentation of childhood PAN, and can mimic Kawasaki disease. Although both are medium-vessel vasculitis differentiation between these two entities is pivotal, as there are differences in treatment modalities, duration of immunomodulatory therapy, and the outcome. This manuscript describes the salient differences which can help differentiate PAN masquerading as Kawasaki disease at initial presentation. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Kawasaki disease (KD) is an acute, self-limited, systemic vasculitis of unknown etiology. The diagnosis is essentially clinical and the treatment is based on the use of intravenous human immunoglobulin (IVIG) and acetylsalicylic acid (ASA) in anti-inflammatory dose, being ideally initiated by the tenth day of the onset of symptoms in order to avoid the complications associated with this pathology. METODOLOGY: This is a case report of a 9-year-old child with Kawasaki syndrome with atypical initial presentation. DISCUSSION: Previously healthy child, presenting febrile jaundice hemorrhagic syndrome and influenza syndrome, associated with radiological changes that could be correlated with SARS-COV-2 infection, without initially ruling out the hypothesis of Pediatric Multisystemic Inflammatory Syndrome (SIM-P). After negative serologies and cultures, there was a progressive remission of symptoms, except for the fever. Criteria for KD were met on the 16th day of evolution, when monotherapy with ASA without IVIG was initiated, with resolution of the fever. CONCLUSION: It is essential to be aware of the possible variations in the clinical presentation of KD and correlate it with the epidemiological and social context of the patient, due to the morbidity related to this vasculitis.

Background: Coronary aneurysms are an infrequent finding in diagnostic angiography, with a reported incidence of 0.35–0.7% in the largest contemporary registries. At least half of them have an atherosclerotic etiology and as such they are often diagnosed in the setting of acute coronary syndrome. The wiring of a thrombosed aneurysm is a difficult task and after successful recanalization, the operator has to decide on the optimal method of aneurysm exclusion. Covered stents are commonly deployed, but their use involves the risk of delivery failure as well as device dislodgement and loss due to their stiffness and size. Moreover, proper stent sizing and apposition is difficult in the case of thrombosed aneurysms. Case presentation: We present a case of coronary aneurysm recanalization and exclusion with a covered stent, with a postdilation-induced stent foreshortening. Due to the subsequent stent migration into the aneurysm sac, its repositioning was attempted. The pitfalls of coronary aneurysm stenting and neurointerventional techniques of prolapsed device realignment are discussed. Conclusions: An adequate landing zone is of the utmost importance in aneurysm exclusion with covered stents. In the case of a short stent anchoring in the normal vessel, another covered or conventional stent should be deployed to mitigate the risk of the device migration.

Kawasaki disease (KD) is a paediatric vasculitis associated with coronary artery aneurysms (CAA). Genetic variants influencing susceptibility to KD have been previously identified, but no risk alleles have been validated that influence CAA formation. We conducted a genome-wide association study (GWAS) for CAA in KD patients of European descent with 200 cases and 276 controls. A second GWAS for susceptibility pooled KD cases with healthy paediatric controls from vaccine trials in the UK (n = 1609). Logistic regression mixed models were used for both GWASs. The susceptibility GWAS was meta-analysed with 400 KD cases and 6101 controls from a previous European GWAS, these results were further meta-analysed with Japanese GWASs at two putative loci. The CAA GWAS identified an intergenic region of chromosome 20q13 with multiple SNVs showing genome-wide significance. The risk allele of the most associated SNV (rs6017006) was present in 13% of cases and 4% of controls; in East Asian 1000 Genomes data, the allele was absent or rare. Susceptibility GWAS with meta-analysis with previously published European data identified two previously associated loci (ITPKC and FCGR2A). Further meta-analysis with Japanese GWAS summary data from the CASP3 and FAM167A genomic regions validated these loci in Europeans showing consistent effects of the top SNVs in both populations. We identified a novel locus for CAA in KD patients of European descent. The results suggest that different genes determine susceptibility to KD and development of CAA and future work should focus on the function of the intergenic region on chromosome 20q13.

A coronary artery aneurysm is an uncommon vascular disorder, and it can be a life-threatening disease when associated with rupture or an embolism. A 52-year-old man was found to have a 50-mm coronary artery aneurysm at the right coronary artery, and the aneurysm was completely occluded by a thrombus. He had no symptoms after arriving at our hospital, and his hemodynamics was stable. Therefore, initially, we administered anticoagulation therapy involving heparin. After therapy, the distal coronary artery was detected when the thrombus dissolved, and elective surgery was planned. Coronary artery bypass grafting, ligation of the inflow and outflow vessels, and resection of the aneurysm were performed. Early anticoagulation therapy and surgical aneurysm resection were effective for treating the completely occluded coronary artery aneurysm. We herein report this rare case of a giant coronary artery aneurysm occluded completely by a thrombus and treated successfully by anticoagulation therapy and surgical aneurysm resection. [ABSTRACT FROM AUTHOR]

Abstract Background Studies on Multisystem Inflammatory Syndrome in Children (MIS-C) and Kawasaki Disease (KD) have yielded inconsistent results and are lacking in Asian and African countries. This study aimed to compare the laboratory and clinical features, short-term outcomes, and one-year follow-ups of a large cohort of MIS-C and KD patients. Methods Data from 176 MIS-C and 56 KD patients admitted to Tehran Children's Medical Center between January 2021 and January 2022 were collected. Patients were followed up until January 2023. Results While lymphopenia and thrombocytopenia were more prevalent in MIS-C (73.2% vs. 20% in KD, p

Abstract Background Coronary artery ectasia is a rare angiographic finding and results from a disease process that compromises the integrity of the vessel wall. Its prevalence ranges between 0.3% and 5% of patients undergoing coronary angiography (Swaye et al. in Circulation 67:134–138, 1983). Coronary artery ectasia in patients with ST-elevation myocardial infarction is associated with an increased risk of cardiovascular events and death after percutaneous coronary intervention. Case presentation We report the case of a 50-year-old male Caucasian patient, admitted for ventricular tachycardia at 200 beats per minute hemodynamically not tolerated that was reduced by external electric shock. Electrocardiogram after cardioversion showed a sinus rhythm with anterior ST-elevation myocardial infarction. Thrombolytic therapy was chosen after exposure to dual antiplatelet therapy and heparin since the expected time to percutaneous coronary intervention was greater than 120 minutes from first medical contact and the patient presented within 12 hours of onset of ischemic symptoms. The electrocardiogram after thrombolysis showed the resolution of the ST segment. The echocardiogram showed a dilated left ventricle with severe dysfunction with left ventricle ejection fraction at 30%. Coronary angiography revealed non-obstructive giant ecstatic coronaries without any thrombus. A check-up to look for possible etiologies for coronary artery ectasia was carried out and returned normal. Since no etiology for coronary artery ectasia was found at the limit of available exams in our center, the patient was discharged with antiplatelet therapy (aspirin 100 mg once a day) and heart failure treatment with an indication for an implantable cardiac defibrillator. Conclusions Coronary artery ectasia in the context of acute myocardial infarction is a rare condition that may have dangerous complications, especially when an optimal treatment for ecstatic culprit vessels is still controversial.

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disease characterized by elevated serum IgG4 levels. It has the potential to affect multiple organs. Despite the diverse manifestations of IgG4-RD, the association with coronary artery disease (CAD) remains poorly understood due to limited evidence. We report the case of a 52-year-old male patient who exhibited typical angina upon exertion, accompanied by elevated serum IgG4 levels. Coronary computed tomographic angiography (CCTA) revealed the presence of pseudotumor formations surrounding and aneurysm changes affecting all coronary arteries, consistent with IgG4-RD. The patient was treated with prednisolone and azathioprine, with the possibility of additional rituximab therapy if symptomatology failed to improve. This case sheds light on the rare occurrence of IgG4-RD with coronary artery involvement and underscores the importance of recognizing this unique clinical entity for appropriate management and further research.

Background Aneurysmal dilatation of saphenous vein grafts used for coronary artery bypass grafting is a rare complication. These aneurysms are often large in calibre and pose a risk of rupture with significant haemorrhage. Case summary We describe a case whereby a large saphenous vein graft aneurysm is closed percutaneously using a vascular plug to cease flow and promote thrombosis of the aneurysm whilst reconstructing the occluded native artery to negate ischaemia. Conclusion Saphenous vein graft aneurysms following coronary artery bypass graft are rare and late complications. The preferred modality of closure is via percutaneous approach that requires meticulous planning to achieve a good outcome. [ABSTRACT FROM AUTHOR]

We describe a 16-year-old asymptomatic male who presented with coronary artery dilation (z score + 2.3) identified on echo performed solely for presence of COVID-19 antibodies. This case raises the question of whether cardiac screening should be considered for all patients with a history of COVID-19.

Introduction. Coronary artery aneurysms (CAAs) are rare disorders, especially the left main CAA. In the literature, there are several reported cases with CAAs, various localization, size, clinical presentation, and way of treatment. There is no unique consensus about the most adequate treatment for these patients; however, surgery is still preferable, although there are some new experiences of percutaneous treatment. The decision is made for each patient individually. We report a case of giant left main CAA, with acute coronary syndrome and heart failure presentation, surgically treated. Case report. A 66-year-old female patient was admitted to the emergency department of our clinic due to chest pain and dyspnea. Acute non-STelevation segment myocardial infarction (STEMI) of anterolateral localization was diagnosed (creatine kinase max 1,111 U/L, troponin T 3.754 ng/mL), complicated with acute heart failure. Heart catheterization and coronary angiography revealed a giant saccular, 3.5 × 3.5 mm left main CAA full with thrombi, compressing the proximal segments of the left anterior descending and circumflex artery. Conclusion. Giant left main CAAs are rare pathologic findings, and there are no established principles for treatment. Although the percutaneous way of treatment is now available in selected cases, the surgical approach is still preferred for these patients. [ABSTRACT FROM AUTHOR]

Background: Studies on Multisystem Inflammatory Syndrome in Children (MIS-C) and Kawasaki Disease (KD) have yielded inconsistent results and are lacking in Asian and African countries. This study aimed to compare the laboratory and clinical features, short-term outcomes, and one-year follow-ups of a large cohort of MIS-C and KD patients. Methods: Data from 176 MIS-C and 56 KD patients admitted to Tehran Children's Medical Center between January 2021 and January 2022 were collected. Patients were followed up until January 2023. Results: While lymphopenia and thrombocytopenia were more prevalent in MIS-C (73.2% vs. 20% in KD, p < 0.001), KD patients exhibited a higher median white blood cell count and prevalence of anemia, along with higher fibrinogen and erythrocyte sedimentation rate levels (p < 0.001, p < 0.001, p = 0.005, p < 0.001, respectively). MIS-C patients also exhibited lower ejection fraction, a greater occurrence of pericardial effusion, and a higher incidence of coronary aneurysms and ectasia, and ascites. Echocardiography after seven days of treatment showed a reduction in pathologies for both groups, but it was significant only for MIS-C. After one year, coronary artery abnormalities remained in only six cases. Conclusions: In conclusion, this study highlights differences between MIS-C and KD, including laboratory indices as well as echocardiographic and abdominal ultrasound findings. These findings contribute valuable data on Iranian patients to the existing literature on this topic and have significant implications for accurate diagnosis and improved management of pediatric patients presenting with these conditions. [ABSTRACT FROM AUTHOR]

Background/aim:The aim of our study was to evaluate the long-term impacts of Kawasaki disease on our patients regarding coronary involvement demographic characteristics, treatment regimens, and clinical course. Materials and methods: Our study included 104 patients diagnosed and hospitalized with Kawasaki disease in our center, from January 2004 to January 2019. In our study, patients were divided into three groups according to coronary artery involvement. Patients in group 1 had no echocardiographic findings, while the ones in group 2 had coronary artery dilatation and ones in group 3 had coronary artery aneurysm (CAA). Results:Among 104 patients, the median age was 9.15 (3.0-22.0) years, and 61 of the patients were male while 43 of the patients were female. With a wide range of 1.50-16.50 years of follow-up time, the median diagnosis age of our patients was 31 months (3.0-164.0). Fever duration (median day 10 (5-21), p = 0.025) was statistically significantly higher in group 3. Blood C-reactive protein (CRP) levels, white blood cell (WBC) counts, and neutrophil counts were significantly higher in group 3. There was a statistically significant difference between patients in group 3 and group 2 in which the lowest strain deformation values were in the patients of group 3. In contrast to group 1, the time for initiation of IVIG therapy is significantly prolonged both in group 2 (median: 9.5 days, p = 0.028) and group 3 (median: 10 days, p = 0.036). Conclusion: In our study, serum CRP levels, WBC count, and neutrophil count were higher in patients with coronary artery abnormalities, in agreement with the previous studies. In the light of our results, we consider that the most important determining factor for the development of coronary artery aneurysm is the time of intravenous immunoglobulin (IVIG) administration. [ABSTRACT FROM AUTHOR]

Epicardial fat may play an important role in the pathogenesis of coronary artery disease (CAD). We investigated the relationship between coronary artery ectasia (CAE) and epicardial fat volume (EFV). This retrospective study included 506 patients with CAE (group 1), 500 with CAD (group 2), and 500 patients with normal coronaries as controls (group 3). The pericardium was traced manually from the edge of the pulmonary trunk to the last measured by computed tomography slice containing images of the heart to obtain a region of interest. EFV was significantly higher in patients with CAD than in those with CAE (87.94 ± 22.18 vs 61.33 ± 12.75 mL; P <.001). Patients with normal coronaries had EFV of 56.62 ± 9.82 mL. Multivariate logistic regression analysis showed that male gender [Odds ratio (OR) (95% confidence interval (CI)): 1.220 (1.015–1.682), P =.042], diabetes [OR (95% CI): 1.036 (1.008–1.057); P =.002], and smoking [OR (95% CI): 3.043 (1.022–9.462); P =.005] were significantly associated with CAE. The receiver operating characteristic (ROC) curve showed that EFV had strongest diagnostic value for detecting CAD rather than CAE [AUC.502 P =.074 (95% CI:.311–.784)]. This study demonstrated that EFV is an independent predictor for CAE and CAD. However, sensitivity and specificity for detecting CAE is low when compared with CAD. [ABSTRACT FROM AUTHOR]

Objective: Unusual clinical course 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. [ABSTRACT FROM AUTHOR]

Background: Coronary artery ectasia is a rare angiographic finding and results from a disease process that compromises the integrity of the vessel wall. Its prevalence ranges between 0.3% and 5% of patients undergoing coronary angiography (Swaye et al. in Circulation 67:134–138, 1983). Coronary artery ectasia in patients with ST-elevation myocardial infarction is associated with an increased risk of cardiovascular events and death after percutaneous coronary intervention. Case presentation: We report the case of a 50-year-old male Caucasian patient, admitted for ventricular tachycardia at 200 beats per minute hemodynamically not tolerated that was reduced by external electric shock. Electrocardiogram after cardioversion showed a sinus rhythm with anterior ST-elevation myocardial infarction. Thrombolytic therapy was chosen after exposure to dual antiplatelet therapy and heparin since the expected time to percutaneous coronary intervention was greater than 120 minutes from first medical contact and the patient presented within 12 hours of onset of ischemic symptoms. The electrocardiogram after thrombolysis showed the resolution of the ST segment. The echocardiogram showed a dilated left ventricle with severe dysfunction with left ventricle ejection fraction at 30%. Coronary angiography revealed non-obstructive giant ecstatic coronaries without any thrombus. A check-up to look for possible etiologies for coronary artery ectasia was carried out and returned normal. Since no etiology for coronary artery ectasia was found at the limit of available exams in our center, the patient was discharged with antiplatelet therapy (aspirin 100 mg once a day) and heart failure treatment with an indication for an implantable cardiac defibrillator. Conclusions: Coronary artery ectasia in the context of acute myocardial infarction is a rare condition that may have dangerous complications, especially when an optimal treatment for ecstatic culprit vessels is still controversial. [ABSTRACT FROM AUTHOR]

Immunoglobulin G4-related disease (IgG4-RD) of the cardiovascular system is rare. Multiple approaches for the management of IgG4-RD have been reported, including surgical resection of affected tissues and treatment with systemic glucocorticoids being generally used. Therefore, the results of surgical resection alone are unknown. A 79-year-old male underwent total aortic arch replacement 5 years previously. Subsequently, a coronary aneurysm of the left circumflex artery (LCx) enlarged with pericardial effusion was removed 2 years after the first surgery. He was diagnosed with a confirmed IgG4-related coronary aneurysm. Serum IgG4 level was 331 mg/dL, and the aneurysm at the distal portion of the LCx was residual. However, he did not receive any corticosteroid treatment. Follow-up transthoracic echocardiography (TTE) revealed an abnormal echo-free cavity structure at 5 o'clock position of the short-axis view. This case describes the progression of residual IgG4-related coronary aneurysm, without corticosteroid therapy. A case with thoracic aortic disease combined with coronary aneurysm may be IgG4-RD. The possibility of progression is high in cases where serum IgG4 remains at a high level, especially without steroid treatment, and follow-up examinations, such as TTE and computed tomography, are more important. Therefore, we reaffirm that corticosteroid therapy may have been important. Immunoglobulin G4-related disease (IgG4-RD) of the cardiovascular system is rare. Multiple approaches for the management of IgG4-RD have been reported, including surgical resection of affected tissues and treatment with systemic glucocorticoids being generally used. Therefore, the results of surgical resection alone, to avoid steroid-related complications, are unknown. Our case showed thoracic aortic disease combined with coronary aneurysm may be IgG4-RD. The importance of corticosteroid treatment was reaffirmed because the residual coronary aneurysm progressed without corticosteroid treatment. [ABSTRACT FROM AUTHOR]

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA Z score ≥2.5) was used. Time-to-event analyses were performed using the Kaplan-Meier method and Cox proportional hazard models for risk factor analysis. In patients with CAA Z scores ≥10, the cumulative incidence of luminal narrowing (>50% of lumen diameter), coronary artery thrombosis, and composite major adverse cardiovascular complications at 10 years was 20±3%, 18±2%, and 14±2%, respectively. No complications were observed in patients with a CAA Z score

Background: Kawasaki disease (KD) is a vasculitis of early childhood mimicking several infectious diseases. Differentiation between KD and infectious diseases is essential as KD's most important complication-the development of coronary artery aneurysms (CAA)-can be largely avoided by timely treatment with intravenous immunoglobulins (IVIG). Currently, KD diagnosis is only based on clinical criteria. The aim of this study was to evaluate whether routine C-reactive protein (CRP) and additional inflammatory parameters myeloid-related protein 8/14 (MRP8/14 or S100A8/9) and human neutrophil-derived elastase (HNE) could distinguish KD from infectious diseases. Methods and Results: The cross-sectional study included KD patients and children with proven infections as well as febrile controls. Patients were recruited between July 2006 and December 2018 in Europe and USA. MRP8/14, CRP, and HNE were assessed for their discriminatory ability by multiple logistic regression analysis with backward selection and receiver operator characteristic (ROC) curves. In the discovery cohort, the combination of MRP8/14+CRP discriminated KD patients (n = 48) from patients with infection (n = 105), with area under the ROC curve (AUC) of 0.88. The HNE values did not improve discrimination. The first validation cohort confirmed the predictive value of MRP8/14+CRP to discriminate acute KD patients (n = 26) from those with infections (n = 150), with an AUC of 0.78. The second validation cohort of acute KD patients (n = 25) and febrile controls (n = 50) showed an AUC of 0.72, which improved to 0.84 when HNE was included. Conclusion: When used in combination, the plasma markers MRP8/14, CRP, and HNE may assist in the discrimination of KD from both proven and suspected infection.

Introduction. Coronary artery aneurysms (CAAs) are rare disorders, especially the left main CAA. In the literature, there are several reported cases with CAAs, various localization, size, clinical presentation, and way of treatment. There is no unique consensus about the most adequate treatment for these patients; however, surgery is still preferable, although there are some new experiences of percutaneous treatment. The decision is made for each patient individually. We report a case of giant left main CAA, with acute coronary syndrome and heart failure presentation, surgically treated. Case report. A 66-year-old female patient was admitted to the emergency department of our clinic due to chest pain and dyspnea. Acute non-ST-elevation segment myocardial infarction (STEMI) of anterolateral localization was diagnosed (creatine kinase max 1,111 U/L, troponin T 3.754 ng/mL), complicated with acute heart failure. Heart catheterization and coronary angiography revealed a giant saccular, 3.5 × 3.5 mm left main CAA full with thrombi, compressing the proximal segments of the left anterior descending and circumflex artery. Conclusion. Giant left main CAAs are rare pathologic findings, and there are no established principles for treatment. Although the percutaneous way of treatment is now available in selected cases, the surgical approach is still preferred for these patients.