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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.

Myocardial infarction (MI) can be caused by many factors. In addition to the typical obstruction or stenosis of the coronary arteries, there is heterogenic MI with non-obstructive coronary arteries (MINOCA). A rare cause of MINOCA is the thrombosis of a coronary artery aneurysm (CAA). This current case report describes a male patient with CAA thrombosis as the cause of MINOCA following surgery for a mucoepidermoid carcinoma. The patient underwent angiography that identified three CAAs that were located as follows: (i) in the proximal part of the left anterior descending artery (5.55 mm); (ii) in the distal part of the circumflex artery (8.05 mm); and (iii) in the distal part of the right coronary artery (6.61 mm). Thrombotic masses were identified within all three structures. The patient received balloon angioplasties without stent implanting and recovered well. The patient was also notable for the presence of two brain artery aneurysms that were the cause of the previous strokes that he had experienced. This case report also reviews the literature in order to: (i) summarize the aetiological factors and clinical manifestations of CAA; (ii) discuss the diagnostic methods for CAA; (iii) describe the medical and surgical management of CAA; and (iv) assess the prognosis of this rare clinical event., Competing Interests: Declaration of conflicting interestThe author declares that there are no conflicts of interest.

Introduction: Kawasaki disease (KD) is a medium vessel vasculitis mainly affecting children below the age of 5. KD is the leading cause of acquired heart disease in developed countries. Diagnosis of KD is clinical, and there are no pathognomonic laboratory tests to confirm the diagnosis. There is a paucity of studies that have utilized proteomic approach for biomarker discovery in KD. Identification of these biomarkers may be helpful for early and more effective diagnosis and may aid in the treatment of KD., Area Covered: The present review focuses on studies that have utilized the proteomic approach in the identification of biomarkers in patients with KD. We have divided these biomarkers into three different categories: the biomarkers used for (a) assessment of risk of KD; (b) assessment of risk of coronary artery aneurysms; and (c) assessment of treatment resistance., Expert Opinion: Efforts to improve the clinical and diagnostic evaluation of KD have focused on general markers of inflammation that are not specific for KD. Identification of a proteomic-based biomarker can reliably and specifically differentiate KD from other diseases and could help in the prompt diagnosis. Comprehensive analysis of the serum proteome of patients with KD may be helpful in identifying candidate protein biomarkers.

Background: Patients with Kawasaki disease (KD) with coronary artery involvement require long-term cardiac care. Although respective evidence-based recommendations are missing, cardiac catheterization is still considered the gold standard for diagnosing detailed coronary pathology. Therefore, to better understand coronary artery pathology development, we conducted a survey to document and evaluate cardiac catheterization data in a European population., Methods and Results: We retrospectively analyzed cardiac catheterization data from KD children from the year 2010 until April 2023. This registry covers basic acute-phase clinical data, and more importantly, detailed information on morphology, distribution, and the development of coronary artery pathologies. A total of 164 mainly White patients (65% boys) were included. A relevant number of patients had no coronary artery aneurysm (CAA) at the cardiac catheterization, indicating that distal CAAs were almost exclusively detected alongside proximal CAAs. Patients with multiple CAAs revealed a significant positive correlation between the number of CAAs and their dimensions in diameter and in length. Location of the CAA within the coronary artery, age at onset of KD, or natal sex did not significantly influence CAA diameters, but CAAs were longer in older children and in boys., Conclusions: That distal CAAs were only present together with proximal ones will hopefully reduce diagnostic CCs in patients with KD without echocardiographically detected proximal CAAs. Furthermore, this study gives valuable insights into dimensional specifics of CAAs in patients with KD. As an ongoing registry, future analyses will further explore long-term outcomes and performed treatments, helping to refine clinical long-term strategies for patients with KD., Registration: URL: https://drks.de/; Unique Identifier: DRKS00031022.

Polyarteritis nodosa (PAN) is a rarely seen vasculitis that often affects small-sized and medium-sized arteries. PAN can present with diverse clinical features depending on the organs involved, including potential cardiac involvement. Typical cardiac manifestations of PAN in the paediatric age group include pericarditis, valvular heart disease and coronary artery aneurysms. In contrast to PAN, Kawasaki disease (KD) is often associated with coronary artery abnormalities. Herein, we report a case of a young boy presented with KD-like features, including a coronary artery aneurysm confirmed by an echocardiogram (ECHO) and eventually diagnosed as PAN. The patient was treated with steroids, methotrexate and tocilizumab with improvement of the symptoms. Repeated ECHO revealed the resolution of the coronary artery aneurysms. Our paper highlights a rare presentation of PAN mimicking KD presentation. Physicians should consider PAN in chronic or severe courses of KD or coronary artery aneurysms., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Kawasaki disease is a paediatric vasculitis that presents with fever, rash, conjunctivitis, mucositis, lymphadenopathy, and extremity changes, and primarily affects children younger than 5 years. Coronary artery aneurysms are observed in approximately 20% of patients without treatment. Giant coronary artery aneurysms are rare but can result in substantial morbidity and mortality due to the risk of thrombosis, stenosis, and myocardial infarction. Infants younger than 6 months and children with coronary artery abnormalities are at highest risk for the development of large or giant coronary artery aneurysms, necessitating swift identification and aggressive treatment. The children at high risk for coronary artery aneurysms warrant primary intensification therapy; however, what the most optimal adjunct therapy might be to reduce their risk is unclear and large-scale international trials are needed. Kawasaki disease is a clinical diagnosis that shares many features with other common febrile illnesses, including multisystem inflammatory syndrome in children. Identifying biomarkers that can distinguish Kawasaki disease from similar conditions and predict coronary artery aneurysm risk are needed to aid timely diagnosis, guide management, and improve patient outcomes., Competing Interests: Declaration of interests We declare no competing interests., (Copyright © 2024 Elsevier Ltd. All rights reserved, including those for text and data mining, AI training, and similar technologies.)

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., (© 2024. The Author(s).)

Objective: To elucidate the relationship between leukocyte-associated Ig-like receptor-1 (LAIR-1) and the pathogenesis of Kawasaki disease (KD) and coronary artery aneurysms(CAA)., Methods: The 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., Results: sLAIR-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., Conclusion: sLAIR-1 may serve as a potential biomarker for KD and CAAs, while LAIR-1 might be implicated in KD pathogenesis and CAA., Competing Interests: Declaration of competing interest The author has no relevant financial or non-financial interests to disclose., (Copyright © 2024 European Federation of Immunological Societies. Published by Elsevier B.V. All rights reserved.)

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., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Competing Interests: None.

Competing Interests: Conflict of interests All authors declare that there is no conflict of interest.

We conducted an autopsy on a 3-month-old boy in whom Kawasaki disease (KD) was strongly suspected based on the autopsy findings. The infant had a fever and was brought to a nearby clinic, where he was prescribed antipyretics and kept under observation. However, 15 days after onset of the fever, he suddenly died in bed. He exhibited no obvious redness of the lips, tongue, or conjunctiva. Membranous desquamation was present on his distal fingers. Vasculitis was observed in the coronary arteries, renal artery, splenic artery, and pulmonary vein. In addition, coronary artery aneurysms were present in the right coronary artery and left anterior descending artery. Thrombotic occlusion was observed in one aneurysm in the right coronary artery, resulting in acute myocardial infarction. The coronary artery wall showed infiltration of numerous macrophages and neutrophils. This case was classified as incomplete KD because the coronary artery aneurysm could not be demonstrated before death and was only recognized at autopsy. Pathologists and forensic scientists need to be aware that there are cases in which KD goes undiagnosed and untreated, leading to coronary artery aneurysm formation and sudden death., (© 2024 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.)

In this case report, we describe the surgical treatment of a right coronary sinus aneurysm. A 69-year-old male patient was screened because of palpitations. He was finally diagnosed with an aneurysm of the sinus of Valsalva of the right coronary cusp. According to current aortic guidelines, surgical reconstruction was proposed. The patient underwent a cardiac operation through a median sternotomy under routine cardiopulmonary bypass. After aortic cross-clamping, the aorta was opened and the connection between the aorta and the aneurysm was clearly visualized, underneath the ostium of the right coronary artery. After excision of the right coronary button and the remaining right coronary sinus wall, this sinus was reconstructed with a Dacron graft, with subsequent coronary reimplantation. The postoperative course was uneventful. The patient was discharged on postoperative day 7. A complete sinus reconstruction was preferred over local patching of the defect because of the proximity of the aneurysm sac to the right coronary artery and the fragile, thin aortic tissue just underneath the coronary ostium., (© The Author 2024. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

A man in his 60s with paroxysmal atrial fibrillation was scheduled for a catheter ablation but was admitted to our department after contrast-enhanced CT showed a large homogeneous right atrial mass (52×52 mm) as well as a dilated right coronary artery (RCA). Coronary artery angiography showed a large fistula from the RCA to the mass in the right atrium. A giant coronary artery aneurysm was suspected and a surgical resection was performed. The mass was attached to the atrial septal wall and was palpated in the right atrium with a feeding artery from the RCA. The final diagnosis was an extremely rare case of giant coronary artery aneurysm originating from the RCA. The surgery was successful, and the patient was discharged 30 days later., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

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

A 77-year-old man presented with an acute inferior ST-segment elevation myocardial infarction.

Background: Coronary artery aneurysms have been considered the most serious complication of Kawasaki disease. However, some coronary artery aneurysms do regress. Therefore, the ability to predict the expected time of coronary artery aneurysm regression is critical. Herein, we have created a nomogram prediction system to determine the early regression (<1 month) among patients with small to medium coronary artery aneurysms., Methods: Seventy-six Kawasaki disease patients identified with coronary artery aneurysms during the acute or subacute phase were included. All the patients who met inclusion criteria demonstrated regression of coronary artery aneurysms within the first-year post Kawasaki disease diagnosis. The clinical and laboratory parameters were compared between the groups of coronary artery aneurysms regression duration within and beyond 1 month. Multivariate logistic regression analysis was used to identify the independent parameters for early regression based on the results from the univariable analysis. Then nomogram prediction systems were established with associated receiver operating characteristic curves., Results: Among the 76 included patients, 40 cases recovered within 1 month. Haemoglobin, globulin, activated partial thromboplastin time, the number of lesions, location of the aneurysm, and coronary artery aneurysm size were identified as independent factors for early regression of coronary artery aneurysms in Kawasaki disease patients. The predictive nomogram models revealed a high efficacy in predicting early regression of coronary artery aneurysms., Conclusion: The size of coronary artery aneurysms, the number of lesions, and the location of aneurysms presented better predictive value for predicting coronary artery aneurysms regression. The nomogram system created from the identified risk factors successfully predicted early coronary artery aneurysm regression.

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

Association of Pulmonary Artery Aneurysm with Ischemic Heart Disease is uncommon, and its surgical management has been rarely described in the literature. Surgical intervention should be individualised according to the coexisting diseases and comorbidities to achieve optimal outcome. We report a case of a 76-year-old man with background history of coronary artery stenting due to ischaemic heart disease. The patient presented with features of coronary compression due to giant pulmonary artery aneurysm. He was operated with replacement of aneurysmal pulmonary trunk with 25 mm Hancock conduit.

Objective: Children 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 Setting: Single-centre observational study., Patients: Children with acute KD and Z score ≥2.5 at baseline., Interventions: Primary adjunctive infliximab (5 or 10 mg/kg) within 48 hours of initiating IVIG 2 g/kg., Main Outcome Measures: Incidence of CAA regression to Zmax <2 within 2 months of disease onset., Results: Of the 168 patients with KD, 111 received IVIG alone and 57 received primary adjunctive infliximab therapy: 39 received 5 mg/kg and 18 received 10 mg/kg. Incidence of CAA regression to Zmax <2 within 2 months was statistically significant at 52%, 62% and 83% in the IVIG alone, IVIG+infliximab 5 mg/kg and IVIG+infliximab 10 mg/kg, respectively. The multivariable logistic regression model adjusting for age, sex, baseline Zmax and bilateral CAA at baseline showed that IVIG plus 10 mg/kg infliximab was significantly associated with a greater likelihood of CAA regression (adjusted OR: 4.45, 95% CI 1.17 to 16.89, p=0.028) compared with IVIG alone. The difference between IVIG+infliximab 5 mg/kg and IVIG alone was not significant., Conclusions: Primary adjunctive high-dose 10 mg/kg infliximab treatment was associated with a greater likelihood of CAA regression in patients with CAA at the time of diagnosis., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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

Background: Kawasaki Disease (KD) is the leading cause of acquired heart disease in children in developed countries with a variable incidence worldwide. Previous studies reported an unexpectedly high incidence of KD in the Canadian Atlantic Provinces. The goals of our study were to validate this finding in the province of Nova Scotia and to carefully review patients' characteristics and disease outcomes., Methods: This was a retrospective review of all children < 16 years old from Nova Scotia diagnosed with KD between 2007-2018. Cases were identified using a combination of administrative and clinical databases. Clinical information was collected retrospectively by health record review using a standardized form., Results: Between 2007-2018, 220 patients were diagnosed with KD; 61.4% and 23.2% met the criteria for complete and incomplete disease, respectively. The annual incidence was 29.6 per 100,000 children < 5 years. The male to female ratio was 1.3:1 and the median age was 3.6 years. All patients diagnosed with KD in the acute phase received intravenous immunoglobulin (IVIG); 23 (12%) were refractory to the first dose. Coronary artery aneurysms were found in 13 (6%) patients and one patient died with multiple giant aneurysms., Conclusion: We have confirmed an incidence of KD in our population which is higher than that reported in Europe and other regions of North America despite our small Asian population. The comprehensive method to capture patients may have contributed to the detection of the higher incidence. The role of local environmental and genetic factors also deserves further study. Increased attention to regional differences in the epidemiology of KD may improve our understanding of this important childhood vasculitis., (© 2023. The Author(s).)

Background: This study evaluated the risk of hypertension, major adverse cardiac events (MACE), and all-cause mortality in Kawasaki disease (KD) patients up to young adulthood., Methods: An inception cohort of 1169 KD patients between 1991 and 2008 from a tertiary-level hospital in Ontario, Canada was linked with health administrative data to ascertain outcomes up to 28 years of follow-up. Their risk was compared with 11,690 matched population comparators. The primary outcome was hypertension and secondary outcomes were MACE and death., Results: After a median follow-up of 20 years [IQR: 8.3], the cumulative incidence of hypertension and MACE in the KD group was 3.8% (95% CI: 2.5-5.5) and 1.2% (95% CI: 0.6-2.4%), respectively. The overall survival probability in the KD group was 98.6% (95% CI: 97.2-99.3%). Relative to comparators, KD patients were at an increased risk for hypertension [aHR: 2.2 (95% CI: 1.5-3.4)], death [aHR: 2.5 (95% CI: 1.3-5.0)], and MACE [aHR: 10.7 (95% CI: 6.4-17.9)]. For hypertension and MACE, the aHR was the highest following diagnosis and then the excess risk diminished after 16 and 13 years of follow-up, respectively. MACE occurred largely in KD patients with coronary aneurysms [cumulative incidence: 12.8%]., Conclusions: KD patients demonstrated a reassuring cardiac prognosis up to young adulthood with low events and excellent survival. KD patients were at increased risk for hypertension, but this excess risk occurred early and declined with time., Impact: With the current standard of care, KD patients demonstrated favorable cardiac prognosis, with low events of hypertension, MACE, and excellent survival. Hypertension and MACE risk appear to be highest around the time of KD diagnosis. MACE occurred primarily in KD patients with coronary aneurysms. Our findings are reassuring to KD patients, families, and their providers. Our study demonstrated an association between KD exposure and hypertension. This association is relatively novel. Previous studies have remained conflicting if KD contributes to long-term atherosclerotic risk., (© 2022. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Coronary aneurysm secondary to coronary fistula is a rare condition, with no existing report on its pathological examination. We report the case of a patient diagnosed with a right coronary artery fistula with coronary aneurysm during the fetal period. During follow-up after delivery, the aneurysm became larger, even though the shunt size decreased. We were afraid the aneurysm would rupture and therefore, planned elective catheter embolization. At the age of 4 years, the patient underwent surgery, which involved closing the fistula and making the lumen of the aneurysm smaller. However, the surgery was not catheter embolization as planned because segment 3 branched off from the largest aneurysm where we planned to embolize. Pathologically, the structure of the coronary artery differed from that of a healthy one, with thickened intima and media, fewer scattered smooth muscle cells, widely distributed elastic fibers, and mucoid degeneration in the media. The structure of the coronary artery suggested that the vessel wall was weak and that the aneurysm would rupture if not treated. Postoperative coronary angiography showed that segment 2 was obstructed, while the collaterals from the left coronary artery perfused the area. We could have treated the fistula with a catheter as scheduled.

Background: Kawasaki disease (KD) is a systemic vasculitis; systemic arteries other than the coronary arteries should therefore also be evaluated. This study investigated the feasibility of evaluating coronary aneurysms, systemic artery aneurysms (SAAs), and cerebrovascular diseases in patients with KD using non-contrast magnetic resonance angiography (NC-MRA)., Methods: Coronary artery protocols, including coronary magnetic resonance angiography (MRA) and vessel wall imaging, were performed in 57 examinations of 28 patients. Systemic artery protocol, including SAA scans and head MRA, along with coronary artery protocol, were performed in 42 examinations of 42 patients. The image quality of the SAAs was evaluated on a 4-point scale. Examination time and sedation dosage were compared between the protocols. The presence of SAAs and cerebrovascular disease was also evaluated., Results: The image quality score of SAAs was 4 (interquartile range [IQR]: 4-4) for the aorta, 4 (IQR: 3-4) for the subclavian artery, 4 (IQR: 3-4) for the renal artery, and 3 (IQR: 3-4) for the iliac artery. No differences were found between examination time (47.0 [IQR: 43.0-61.0] min vs. 51.0 [IQR: 45.0-60.0] min, p = 0.48) and sedative dose (4.63 [IQR: 3.93-5.79] mg/kg vs. 4.21 [IQR: 3.56-5.71] mg/kg, p = 0.37) between the protocols. Systemic artery protocol detected SAAs in three patients (7.1%), and cerebrovascular disease was not detected., Conclusions: Evaluating the coronary and systemic arteries in patients with KD using NC-MRA on a single examination was possible without compromising examination time or sedation dose. The systemic artery protocol was useful in finding SAAs., (© 2023 Japan Pediatric Society.)

A coronary aneurysm is a rare type of cardiovascular disease. We report a case of a 53-year-old male patient who presented to our hospital with a giant left circumflex coronary fistula aneurysm (LCCA) (75 mm × 70 mm). Since coronary angiography and coronary computed tomography angiography failed to detect the fistula of the coronary aneurysm, interventional occlusion surgery could not be performed. We discovered the fistula in the right atrium by anterograde perfusion with blood-containing myocardial protective fluid after switching to intraoperative exploration during cardiac surgery. The coronary aneurysm's fistula and inlet were then sutured, and the aneurysm was resected. The patient recovered successfully after the operation. This case was instructive in managing LCCA, especially with an unidentified fistula.

Left main coronary artery aneurysm is an unusual complication of infective endocarditis. Although this type of aneurysm is often asymptomatic, rupture and thrombus formation that result in myocardial infarction are known complications; therefore, prompt recognition and surgical intervention are warranted. This report describes a patient who presented with a giant left main coronary artery aneurysm 3.5 years after being treated for 4-valve endocarditis. The management and technical aspects of this challenging case are discussed here., (© 2023 by the Texas Heart® Institute, Houston.)

Background and Aim of the Study: The widespread use of noninvasive/invasive coronary imaging increased the probability of recognition of coronary aneurysms. Left main coronary aneurysms (LMCA), though rare, are potentially life-threatening but in the absence of controlled studies, guidelines do not provide any specific recommendation for their management. We, therefore, aimed to investigate the epidemiology, clinical presentation, therapeutic strategies, and prognostic implication of LMCA., Methods: A systematic review of the literature was performed to retrieve all the reported cases of LMCA as of December 2021, which were summarized and classified according to their etiology, clinical presentation, and therapeutic management., Results: Out of 1997 works retrieved, 180 studies were analyzed, describing 209 LMCA cases (aged 51 ± 19 years, 68% males). Atherosclerosis was the most common etiology (40%), followed by inflammatory (12%), congenital (9%), or degenerative (6%) conditions. Stable angina (43%) and acute coronary syndromes (32%) were more often the first clinical manifestations, while 29 (14%) LMCA were incidental findings. Most cases were treated surgically (53%), while percutaneous intervention was rarely adopted (7%). Data about antithrombotic therapies were scarce and heterogeneous. Finally, when longitudinal data were reported (n = 81), LMCA resulted associated with a severe prognosis, with a 15% mortality over an 8-month median follow-up., Conclusions: LMCA are most frequently, but not exclusively, caused by advanced atherosclerosis. Irrespective of their etiology and clinical presentation, LMCA may be associated with high short-term mortality. In absence of controlled studies, a careful evaluation of each case is warranted to optimize therapeutic strategies., (© 2022 Wiley Periodicals LLC.)

Congenital fistula between the first diagonal branch of the coronary artery and left ventricle with a giant coronary artery aneurysm is extremely rare. We present the case of a 50-year-old asymptomatic male patient with such a condition that was diagnosed by transthoracic echocardiography, coronary computed tomography angiography, and coronary angiography. The patient was treated by surgical fistula closure under cardiopulmonary bypass. The postoperative coronary computed tomography angiography showed the patient got a complete cure, and the patient remains asymptomatic after 5-year follow up.

Giant aneurysm of the left main coronary artery is exceedingly rare and accounts for less than 2% of patients undergoing coronary angiography. The etiology varies depending on the patient's age and geographic area, but half are of atherosclerotic origin. In most cases, coronary aneurysms are asymptomatic, however, symptomatic patients present with symptoms characteristic of coronary artery disease such as chest pain (angina pectoris), myocardial infarction, congestive heart failure, and even sudden death. Coronary angiography is considered the gold standard tool to determine the presence or absence of coronary artery disease, and if present, its size and location. Herein, we report a case of giant aneurysm of the left main coronary artery presenting as syncope and documented nonsustained ventricular tachycardia., (© 2022 by the Texas Heart® Institute, Houston.)

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