Kawasaki disease (KD) is an acute childhood disease complicated by coronary artery aneurysms, intima thickening, thrombi, stenosis, lamellar calcifications, and disappearance of the media border. ...Automatic classification of the coronary artery layers (intima, media, and scar features) is important for analyzing optical coherence tomography (OCT) images recorded in pediatric patients. OCT has been known as an intracoronary imaging modality using near-infrared light which has recently been used to image the inner coronary artery tissues of pediatric patients, providing high spatial resolution (ranging from 10 to 20
m). This study aims to develop a robust and fully automated tissue classification method by using the convolutional neural networks (CNNs) as feature extractor and comparing the predictions of three state-of-the-art classifiers, CNN, random forest (RF), and support vector machine (SVM). The results show the robustness of CNN as the feature extractor and random forest as the classifier with classification rate up to 96%, especially to characterize the second layer of coronary arteries (media), which is a very thin layer and it is challenging to be recognized and specified from other tissues.
The aim of this study was to find the best model to obtain valid and normally distributed Z scores for coronary artery (CA) diameters in a large, heterogeneous population of healthy children.
...Echocardiography was performed on 1,033 healthy children. Several regression models were tested with height, weight, body surface area, and aortic valve diameter. The computed Z scores were tested for normal distribution and stability.
CA diameter was best predicted using regression with the square root of body surface area. The weighted least squares method yielded normally distributed and very stable Z-score estimates for all CA segments. In prepubertal children, aortic valve diameter was also a valid predictor of CA diameter.
This study shows two valid methods to estimate Z scores for CA size in children of all ages. Such Z scores are important for risk stratification in patients with Kawasaki disease.
Myocarditis and Kawasaki disease Dionne, Audrey; Dahdah, Nagib
International journal of rheumatic diseases,
January 2018, Letnik:
21, Številka:
1
Journal Article
Recenzirano
Kawasaki disease (KD) is the most common vasculitis of childhood. Coronary artery aneurysms and myocarditis are common cardiovascular complications of KD. While evidence of myocarditis can be found ...in all patients with KD on histology specimens, only a minority of patients are clinically symptomatic. Occasionally children can present with KD shock syndrome and hemodynamic instability as a result of decreased systolic function and vasoplegia. Several children with KD have depressed shortening fraction on echocardiography. Increased end‐systolic and end‐diastolic dimensions, strain abnormalities and diastolic dysfunction are also found in a significant proportion of patients. Echocardiographic signs of myocarditis improve after the acute phase and do so more quickly in patients who have received intravenous immunoglobulins, as opposed to those given only aspirin. Normalization of systolic function is typically observed over long‐term follow‐up; however, more subtle abnormalities (strain, diastolic function) may persist. It is noteworthy that myocarditis associated with KD can occur in absence of coronary artery abnormalities. KD myocarditis can result in long‐term sequelae.
Objectives This study sought to determine the impact of steroid therapy on cardiomyopathy and mortality in patients with Duchenne muscular dystrophy (DMD). Background DMD is a debilitating X-linked ...disease that afflicts as many as 1 in 3,500 boys. Although steroids slow musculoskeletal impairment, the effects on cardiac function and mortality remain unknown. Methods We conducted a cohort study on patients with DMD treated with renin-angiotensin-aldosterone system antagonists with or without steroid therapy. Results Eighty-six patients, 9.1 ± 3.5 years of age, were followed for 11.3 ± 4.1 years. Seven of 63 patients (11%) receiving steroid therapy died compared with 10 of 23 (43%) not receiving steroid therapy (p = 0.0010). Overall survival rates at 5, 10, and 15 years of follow-up were 100%, 98.0%, and 78.6%, respectively, for patients receiving steroid therapy versus 100%, 72.1%, and 27.9%, respectively, for patients not receiving steroid therapy (log-rank p = 0.0005). In multivariate propensity-adjusted analyses, steroid use was associated with a 76% lower mortality rate (hazard ratio: 0.24; 95% confidence interval: 0.07 to 0.91; p = 0.0351). The mortality reduction was driven by fewer heart failure–related deaths (0% vs. 22%, p = 0.0010). In multivariate analyses, steroids were associated with a 62% lower rate of new-onset cardiomyopathy (hazard ratio: 0.38; 95% confidence interval: 0.16 to 0.90; p = 0.0270). Annual rates of decline in left ventricular ejection fraction (−0.43% vs. −1.09%, p = 0.0101) and shortening fraction (−0.32% vs. −0.65%, p = 0.0025) were less steep in steroid-treated patients. Consistently, the increase in left ventricular end-diastolic dimension was of lesser magnitude (+0.47 vs. +0.92 mm per year, p = 0.0105). Conclusions In patients with DMD, steroid therapy is associated with a substantial reduction in all-cause mortality and new-onset and progressive cardiomyopathy.
The mRNA vaccines against COVID-19 infection have been effective in reducing the number of symptomatic cases worldwide. With widespread uptake, case series of vaccine-related myocarditis/pericarditis ...have been reported, particularly in adolescents and young adults. Men tend to be affected with greater frequency, and symptom onset is usually within 1 week after vaccination. Clinical course appears to be mild in most cases. On the basis of the available evidence, we highlight a clinical framework to guide providers on how to assess, investigate, diagnose, and report suspected and confirmed cases. In any patient with highly suggestive symptoms temporally related to COVID-19 mRNA vaccination, standardized workup includes serum troponin measurement and polymerase chain reaction testing for COVID-19 infection, routine additional lab work, and a 12-lead electrocardiogram. Echocardiography is recommended as the imaging modality of choice for patients with unexplained troponin elevation and/or pathologic electrocardiogram changes. Cardiovascular specialist consultation and hospitalization should be considered on the basis of the results of standard investigations. Treatment is largely supportive, and myocarditis/pericarditis that is diagnosed according to defined clinical criteria should be reported to public health authorities in every jurisdiction. Finally, we recommend COVID-19 vaccination in all individuals in accordance with the Health Canada and National Advisory Committee on Immunization guidelines. In patients with suspected myocarditis/pericarditis after the first dose of an mRNA vaccine, deferral of a second dose is recommended until additional reports become available.
Les vaccins à ARNm contre la COVID-19 ont permis de réduire efficacement le nombre de cas symptomatiques de cette infection dans le monde entier. Par suite de l'usage généralisé du vaccin, une série de cas de myocardite ou de péricardite liées au vaccin a été signalée, en particulier chez les adolescents et les jeunes adultes. Le phénomène tend à toucher plus fréquemment les sujets de sexe masculin, et les symptômes apparaissent généralement au cours de la semaine suivant la vaccination. L’évolution clinique semble bénigne dans la très grande majorité des cas. À partir des données disponibles, nous dégageons un cadre de référence clinique auquel les fournisseurs pourront se reporter au moment d’évaluer, d'examiner, de diagnostiquer et de signaler les cas suspects et confirmés. Chez tout patient qui a des symptômes fortement évocateurs et présentant un lien temporel avec l'administration du vaccin à ARNm contre la COVID-19, le bilan diagnostique systématique comprend le dosage de la troponine sérique et le dépistage de la COVID-19 par PCR, d'autres analyses de laboratoire courantes et un électrocardiogramme (ECG) à 12 dérivations. L’échocardiographie est la technique d'imagerie recommandée en première intention chez les patients présentant une hausse inexpliquée du taux de troponine et/ou des modifications pathologiques du tracé de l'ECG. La consultation d'un spécialiste en soins cardiovasculaires et l'hospitalisation devraient être envisagées en fonction des résultats des examens standard. Le traitement est en grande partie axé sur les soins de soutien, et les cas de myocardite ou de péricardite diagnostiqués selon des critères cliniques définis devraient être signalés aux autorités de santé publique locales partout au pays. Enfin, nous recommandons la vaccination de chaque personne contre la COVID-19, conformément aux lignes directrices de Santé Canada et du Comité consultatif national de l'immunisation. En ce qui concerne les patients chez qui une myocardite ou une péricardite est soupçonnée après l'administration de la première dose d'un vaccin à ARNm, il est recommandé de reporter l'administration de la seconde dose jusqu’à ce que des données supplémentaires soient disponibles.
If making patients aware of their antecedent KD diagnosis as a potential cardiovascular risk factor deeply affects their psyche, then physicians must also refrain from discussing obesity, for ...example, when counseling primary or secondary prevention of cardiovascular diseases.
Patients with Kawasaki disease can develop life-altering coronary arterial abnormalities, particularly in those resistant to intravenous immunoglobulin (IVIg) therapy. We tested the tumor necrosis ...factor α receptor antagonist etanercept for reducing both IVIg resistance and coronary artery (CA) disease progression.
In a double-blind multicenter trial, patients with Kawasaki disease received either etanercept (0.8 mg/kg;
= 100) or placebo (
= 101) subcutaneously starting immediately after IVIg infusion. IVIg resistance was the primary outcome with prespecified subgroup analyses according to age, sex, and race. Secondary outcomes included echocardiographic CA measures within subgroups defined by coronary dilation (
score >2.5) at baseline. We used generalized estimating equations to analyze
score change and a prespecified algorithm for change in absolute diameters.
IVIg resistance occurred in 22% (placebo) and 13% (etanercept) of patients (
= .10). Etanercept reduced IVIg resistance in patients >1 year of age (
= .03). In the entire population, 46 (23%) had a coronary
score >2.5 at baseline. Etanercept reduced coronary
score change in those with and without baseline dilation (
= .04 and
= .001); no improvement occurred in the analogous placebo groups. Etanercept (
= 22) reduced dilation progression compared with placebo (
= 24) by algorithm in those with baseline dilation (
= .03). No difference in the safety profile occurred between etanercept and placebo.
Etanercept showed no significant benefit in IVIg resistance in the entire population. However, preplanned analyses showed benefit in patients >1 year. Importantly, etanercept appeared to ameliorate CA dilation, particularly in patients with baseline abnormalities.
Kawasaki disease (KD) is an inflammatory febrile illness of early childhood and the primary cause of acquired heart disease during childhood. Coronary artery aneurysms (CAA) are a serious ...complication of KD, leading to ischemic heart disease, myocardial infarction, and sudden cardiac death. Timely diagnosis in the first ten days of fever is crucial to reduce the risk of coronary artery complications. Nitrogen-terminal B-type natriuretic peptide (NT-proBNP), originally used for the management of adults with heart disease, was shown to be useful in the diagnosis and management of patients with KD. NT-proBNP is released by cardiomyocytes in response to mechanical factors such as the dilation of cardiac chambers, and to pro-inflammatory cytokines. The utility of NT-proBNP as a biological marker in KD is based on the universal myocardial inflammatory component early in the course of the disease. Patients with KD have higher NT-proBNP at the time of diagnosis than febrile controls, with a pooled sensitivity of 89% (95% confidence interval 78⁻95), and a specificity of 72% (95% confidence interval 58⁻82). The positive likelihood ratio is 3.2:1 (95% confidence interval 2.1⁻4.8). Moreover, patients with resistance to intravenous immunoglobulin treatment and CAA were found to have higher levels of NT-proBNP, suggesting a prognostic role. Nevertheless, the non-specificity of NT-proBNP to KD limits its use as a stand-alone test. In this light, a tentative associative retrospective diagnostic algorithm was highly reliable for including all cases at risk of CAA, which warrants further prospective studies for a better diagnostic index of suspicion and risk stratification of patients.
PDF
