COVID-19 associated multisystem inflammatory syndrome in a neonate with atypical coronary artery involvement.

Objective The study aimed to report a COVID-19 associated multisystem inflammatory syndrome in children (MIS-C) in a neonate found to have an atypical diffuse thickening in coronary artery walls, whose diagnosis required a multi-imaging approach. Study Design A neonate presented at birth with multiple organ involvement and coronary artery anomalies. A diagnosis of MIS-C associated to COVID-19 was supported by maternal Sars-CoV-2 infection during pregnancy, and by the presence of both IgG against Sars-CoV-2 and Spike-specific memory B cells response in the neonatal blood. Other plausible causes of the multiple organ involvement were excluded. Result At admission, a severe coronary artery dilatation was identified on echocardiography, supporting the diagnosis of MIS-C Kawasaki-like disease; however, coronary artery internal diameters were found to be normal using cardiac computed tomography angiography. At discharge, comparing the two imaging techniques each other, the correct diagnosis resulted to be an abnormal thickening in coronary arterial walls. These findings suggest that the inflammatory process affecting the coronary arterial wall in MIS-C could result not only in typical coronary artery lesions such as dilatation of the lumen or aneurysms development, but also in abnormal thickening of the coronary artery wall. Conclusion. Our case provides an alert for paediatric cardiologists about the complexity to assess coronary artery involvement in MIS-C, and raises the question of whether an abnormal vascular remodeling, with normal inner diameters, is to be considered like coronary artery dilatation for risk stratification.

The Impact of CK-MB Elevation in Patients With Acute Type A Aortic Dissection With Coronary Artery Involvement

Background Acute coronary artery involvement (ACI) is a lethal complication of acute type A aortic dissection. Although ACI has been reported as one of the prognostic factors of acute type A aortic dissection, it does not always cause coronary ischemia. The extent of myocardial damage varies from case to case. Moreover, since the definition of ACI varies from paper to paper, it is unknown what the difference is between ACI with and without myocardial necrosis. In general, it can be assumed that cases with myocardial infarction have worse results. However, it is unknown how poor ACI is with myocardiac ischemia and how optimistic it is without it. This study compared the surgical results between the two groups of ACI with or without myocardiac ischemia. Methods Among 348 patients who underwent an emergency operation for acute type A aortic dissection, there were 28 (8.0%) patients complicated by ACI and underwent additional coronary artery bypass grafting. We divided 26 of those patients into two groups; the MI group ( with CK-MB elevation) and the NMI group (without CK-MB elevation) and compared both groups. Results Of the 26, sixteen were in the MI group, and ten were in the NMI group. The average CK-MB in the MI group was 225.5 IU/L, and that in the NMI group was 13.5 IU/L. The mean time from onset to surgery was 248 minutes in the MI group, 250 minutes in the NMI group. There was statistical significance in mortality ( 69% vs. 13%, p=0.03). There was no significance in major complications (ICU days, reintubation, reoperation, pneumonia, sepsis). Conclusions Acute coronary artery involvement was associated with 8.0% of patients with ATAAD, and 62% of them had myocardial ischemia with CK-MB elevation. As expected, the MI group had significantly higher mortality than the NMI group. It is crucial for cases with suspected ACI to obtain coronary perfusion as soon as possible to prevent CK-MB from elevationg.

Concurrent coronary artery and subclavian arterial aneurysms in Takayasu arteritis

Background Aneurysmal coronary artery involvement and subclavian artery aneurysm are extremely uncommon in Takayasu arteritis. Case presentation We present a case with concurrent coronary artery and subclavian artery aneurysms. Conclusions This case report stresses multimodality and multisystem imaging in Takayasu arteritis to know the disease load in the patient and to know the possibility of a rare type of involvement (medium and large vessel) in Takayasu arteritis patient.

Male Patients with Takayasu Arteritis and Coronary Artery Involvement are Prone to have Serious Coronary Stenosis and High Mortality

Background: Takayasu arteritis (TAK) is a rare autoimmune vasculitis that predominantly affects the aorta and its major branches, including the coronary arteries. Limited information is available regarding sex-specific differences in coronary artery involvement among TAK patients. Objective: To assess the differences in coronary angiographic findings and the long-term outcomes between male and female TAK patients having coronary artery involvement. Methods: This retrospective cohort study included 87 TAK patients, grouped by sex, who underwent coronary angiography. General characteristics, clinical features, coronary angiographic findings, and therapeutic strategies were obtained from medical records. Major Adverse Cardiac Events (MACE), including death from any cause; myocardial infarction; repeated coronary artery revascularization; and rehospitalization due to unstable or progressive angina, or heart failure occurring during follow-up, were also recorded. Results: A total of 207 coronary lesions with stenosis were found in 87 TAK patients. The prevalence of ostial coronary lesions was lower in men than in women (9.1 vs. 23.9%, p=0.031). We observed less moderate stenosis (6.8 vs 22.7%, p=0.018) and more severe stenosis or occlusion (70.5 vs 46.0%, p=0.004) in the coronary lesions of male TAK patients. During the up to 7-year follow-up, death from any cause was greater in men than in women (21.4 vs. 1.5%, p=0.003); no other significant differences in MACE occurrence were observed between sexes. Conclusions: Among TAK patients with coronary artery involvement, males tend to have more severe coronary stenosis and a higher risk for long-term mortality than females.

Association Between rs12037447, rs146732504, rs151078858, rs55723436, and rs6094136 Polymorphisms and Kawasaki Disease in the Population of Polish Children

Background: Kawasaki disease (KD) is an acute self-limited febrile vasculitis that mainly affects young children. Coronary artery involvement is the most serious complication in children with KD. It is currently the leading cause of acquired cardiac disease in children from developed countries. Literature data indicate a significant role of genetic susceptibility to KD.Objective: The aim of this study was to perform the first Genome-Wide Association Study (GWAS) in a population of Polish children with KD and identify susceptible genes involved in the pathogenesis of KD.Materials and Methods: The blood samples of Kawasaki disease patients (n = 119) were collected between 2016 and 2020, isolated and stored at the Department of Pediatrics, Nutrition and Metabolic Diseases, Children's Memorial Health Institute in Warsaw. The control group was based on Polish donors (n = 6,071) registered as the POPULOUS collection at the Biobank Lab of The Department of Molecular Biophysics in University of Lodz. DNA samples were genotyped for 558,231 Single Nucleotide Polymorphisms (SNPs) using the 24 × 1 Infinium HTS Human Core Exome microarrays according to the protocol provided by the manufacturer. In order to discover and verify genetic risk-factors for KD, association analysis was carried out using PLINK 1.9.Results: Of all 164,395 variants, 5 were shown to occur statistically (padjusted < 0.05) more frequent in Kawasaki disease patients than in controls. Those are: rs12037447 in non-coding sequence (padjusted = 8.329 × 10−4, OR = 8.697, 95% CI; 3.629–20.84) and rs146732504 in KIF25 (padjusted = 0.007354, OR = 11.42, 95% CI; 3.79–34.43), rs151078858 in PTPRJ (padjusted = 0.04513, OR = 8.116, 95% CI; 3.134–21.01), rs55723436 in SPECC1L (padjusted = 0.04596, OR = 5.596, 95% CI; 2.669–11.74), rs6094136 in RPN2 (padjusted = 0.04755, OR = 10.08, 95% CI; 3.385–30.01) genes.Conclusion: Polymorphisms of genes KIF25, PTRPJ, SPECC1L, RNP2 may be linked with the incidence of Kawasaki disease in Polish children.

Irreversible coronary aneurysm presenting as acute coronary syndrome in a child with hypereosinophilic syndrome: a case report

Hypereosinophilic syndrome is defined as persistent eosinophilia in the blood for more than 6 months, without any identifiable cause and with end-organ involvement evidence. Cardiac manifestations of HES include heart failure due to restrictive cardiomyopathy, arrhythmia, intraventricular thrombosis, and coronary artery involvement occurs frequently. In rare instances, coronary ectasia, aneurysms, or dissection can occur and cause morbidity and mortality in these patients. A coronary aneurysm occurs rarely in adult patients with HES but to our knowledge, this is the first report of this association in a 14-year-old boy who was presented to us as coronary aneurysm due to hypereosinophilic syndrome.