RESUMO
There have been no published prospective randomized clinical trials that have: (1) established an association between invasive dental and nondental invasive procedures and risk of infective endocarditis; or (2) defined the efficacy and safety of antibiotic prophylaxis administered in the setting of invasive procedures in the prevention of infective endocarditis in high-risk patients. Moreover, previous observational studies that examined the association of nondental invasive procedures with the risk of infective endocarditis have been limited by inadequate sample size. They have typically focused on a few potential at-risk surgical and nonsurgical invasive procedures. However, recent investigations from Sweden and England that used nationwide databases and demonstrated an association between nondental invasive procedures, and the subsequent development of infective endocarditis (in particular, in high-risk patients with infective endocarditis) prompted the development of the current science advisory.
Assuntos
Endocardite Bacteriana , Endocardite , Estados Unidos , Humanos , Estudos Prospectivos , American Heart Association , Endocardite Bacteriana/prevenção & controle , Endocardite/prevenção & controle , AntibioticoprofilaxiaRESUMO
Myocarditis is a rare complication of the COVID-19 mRNA vaccine. We previously reported a case series of 15 adolescents with vaccine-associated myocarditis, 87% of whom had abnormalities on initial cardiac magnetic resonance (CMR), including late gadolinium enhancement (LGE) in 80%. We performed follow-up CMRs to determine the trajectory of myocardial recovery and better understand the natural history of vaccine-associated myocarditis. Case series of patients age < 19 years admitted to Boston Children's Hospital with acute vaccine-associated myocarditis following the BNT162b2 vaccine who had abnormal CMR at the time of initial presentation, and underwent follow-up testing. CMR assessment included left ventricular (LV) ejection fraction, T2-weighted myocardial imaging, LV global native T1, LV global T2, extracellular volume (ECV), and late gadolinium enhancement (LGE). Ten patients (9 male, median age 15 years) with vaccine-associated myocarditis underwent follow-up CMR at a median of 92 days (range 76-119) after hospital discharge. LGE was persistent in 80% of patients, though improved from prior in all cases. Two patients (20%) had abnormal LV global T1 at presentation, which normalized on follow-up. ECV decreased between acute presentation and follow-up in 6/10 patients; it remained elevated at follow-up in 1 patient and borderline in 3 patients. CONCLUSION: CMR performed ~3 months after admission for COVID-19 vaccine-associated myocarditis showed improvement of LGE in all patients, but persistent in the majority. Follow-up CMR 6-12 months after acute episode should be considered to better understand the long-term cardiac risks. WHAT IS KNOWN: ⢠Myocarditis is a rare side effect of COVID-19 mRNA vaccine. â¢Late gadolinium enhancement is present on most cardiac magnetic resonance at the time of acute presentation. WHAT IS NEW: â¢Late gadolinium enhancement improved on all repeat cardiac magnetic resonance at 3-month follow-up. â¢Most patients still had a small amount of late gadolinium enhancement, the clinical significance of which is yet to be determined.
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COVID-19 , Miocardite , Adolescente , Adulto , Vacina BNT162 , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Criança , Meios de Contraste/efeitos adversos , Seguimentos , Gadolínio/efeitos adversos , Humanos , Imageamento por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Masculino , Miocardite/diagnóstico por imagem , Miocardite/etiologia , Miocárdio/patologia , Valor Preditivo dos Testes , Vacinas Sintéticas , Função Ventricular Esquerda , Adulto Jovem , Vacinas de mRNARESUMO
Background Health care transition (HCT) is a period of high vulnerability for patients with chronic childhood diseases, particularly when patients shift from a pediatric to an adult care setting. An increasing number of patients with Kawasaki disease (KD) who develop medium and large coronary artery aneurysms (classified by the American Heart Association according to maximal internal coronary artery diameter Z-scores ≥5 and ≥10, respectively) are becoming adults and thus undergoing an HCT. However, a poor transition to an adult provider represents a risk of loss to follow-up, which can result in increasing morbidity and mortality. Methods and Results This scientific statement provides a summary of available literature and expert opinion pertaining to KD and HCT of children as they reach adulthood. The statement reviews the existing life-long risks for patients with KD, explains current guidelines for long-term care of patients with KD, and offers guidance on assessment and preparation of patients with KD for HCT. The key element to a successful HCT, enabling successful transition outcomes, is having a structured intervention that incorporates the components of planning, transfer, and integration into adult care. This structured intervention can be accomplished by using the Six Core Elements approach that is recommended by the American Academy of Pediatrics, the American Academy of Family Physicians, and the American College of Physicians. Conclusions Formal HCT programs for patients with KD who develop aneurysms should be established to ensure a smooth transition with uninterrupted medical care as these youths become adults.
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Síndrome de Linfonodos Mucocutâneos , Transição para Assistência do Adulto , Adolescente , Adulto , American Heart Association , Criança , Humanos , Síndrome de Linfonodos Mucocutâneos/terapia , Transição para Assistência do Adulto/organização & administração , Estados UnidosRESUMO
Importance: The BNT162b2 (Pfizer-BioNTech) messenger RNA COVID-19 vaccine was authorized on May 10, 2021, for emergency use in children aged 12 years and older. Initial reports showed that the vaccine was well tolerated without serious adverse events; however, cases of myocarditis have been reported since approval. Objective: To review results of comprehensive cardiac imaging in children with myocarditis after COVID-19 vaccine. Design, Setting, and Participants: This study was a case series of children younger than 19 years hospitalized with myocarditis within 30 days of BNT162b2 messenger RNA COVID-19 vaccine. The setting was a single-center pediatric referral facility, and admissions occurred between May 1 and July 15, 2021. Main Outcomes and Measures: All patients underwent cardiac evaluation including an electrocardiogram, echocardiogram, and cardiac magnetic resonance imaging. Results: Fifteen patients (14 male patients [93%]; median age, 15 years [range, 12-18 years]) were hospitalized for management of myocarditis after receiving the BNT162b2 (Pfizer) vaccine. Symptoms started 1 to 6 days after receipt of the vaccine and included chest pain in 15 patients (100%), fever in 10 patients (67%), myalgia in 8 patients (53%), and headache in 6 patients (40%). Troponin levels were elevated in all patients at admission (median, 0.25 ng/mL [range, 0.08-3.15 ng/mL]) and peaked 0.1 to 2.3 days after admission. By echocardiographic examination, decreased left ventricular (LV) ejection fraction (EF) was present in 3 patients (20%), and abnormal global longitudinal or circumferential strain was present in 5 patients (33%). No patient had a pericardial effusion. Cardiac magnetic resonance imaging findings were consistent with myocarditis in 13 patients (87%) including late gadolinium enhancement in 12 patients (80%), regional hyperintensity on T2-weighted imaging in 2 patients (13%), elevated extracellular volume fraction in 3 patients (20%), and elevated LV global native T1 in 2 patients (20%). No patient required intensive care unit admission, and median hospital length of stay was 2 days (range 1-5). At follow-up 1 to 13 days after hospital discharge, 11 patients (73%) had resolution of symptoms. One patient (7%) had persistent borderline low LV systolic function on echocardiogram (EF 54%). Troponin levels remained mildly elevated in 3 patients (20%). One patient (7%) had nonsustained ventricular tachycardia on ambulatory monitor. Conclusions and Relevance: In this small case series study, myocarditis was diagnosed in children after COVID-19 vaccination, most commonly in boys after the second dose. In this case series, in short-term follow-up, patients were mildly affected. The long-term risks associated with postvaccination myocarditis remain unknown. Larger studies with longer follow-up are needed to inform recommendations for COVID-19 vaccination in this population.
Assuntos
Vacina BNT162/efeitos adversos , COVID-19/prevenção & controle , Hospitalização/estatística & dados numéricos , Miocardite/etiologia , Adolescente , COVID-19/diagnóstico , COVID-19/epidemiologia , COVID-19/imunologia , Vacinas contra COVID-19/administração & dosagem , Técnicas de Imagem Cardíaca/métodos , Criança , Ecocardiografia/métodos , Eletrocardiografia/métodos , Feminino , Seguimentos , Coração/diagnóstico por imagem , Coração/fisiopatologia , Humanos , Tempo de Internação/estatística & dados numéricos , Imageamento por Ressonância Magnética/métodos , Masculino , Miocardite/diagnóstico , SARS-CoV-2/genética , SARS-CoV-2/imunologia , Volume Sistólico/fisiologia , Troponina/sangue , Função Ventricular Esquerda/fisiologiaRESUMO
BACKGROUND: Despite timely administration of IVIG, some patients with Kawasaki disease (KD) develop rapidly progressive or giant coronary artery aneurysms (CAA). CASE PRESENTATION: We describe our experience using cyclophosphamide (CYC) for the treatment of such cases as well as a review of the literature on the use of CYC in KD. Through a retrospective chart review of our KD population, we identified ten children treated for KD with intravenous CYC (10 mg/kg/dose) for one or two doses. Seven patients were male, the median age was 2.0 years (range 4 months - 5 years). All patients received initial IVIG between day 4-10 of illness. Other anti-inflammatory treatments administered before CYC included second IVIG (n = 9), corticosteroids (n = 10), infliximab (n = 4), cyclosporine (n = 2), and anakinra (n = 1). Median illness day at administration of the first CYC dose was 22.5 days (range:10-36 days). The primary indication for treatment with CYC for all patients was large or giant CAA and/or rapid progression of CAA. Three patients received a second dose of CYC (10 mg/kg) for progressively enlarging CAA. CAA did not progress after final CYC treatment. One patient with a history of neutropenia in infancy developed severe neutropenia 9 days after treatment with CYC, which recovered without intervention or complications. No patient developed infections or other serious toxicity from CYC. CONCLUSION: In KD patients with severe and progressive enlargement of CAA despite anti-inflammatory therapy, CYC seemed to arrest further dilation and was well-tolerated. Future multicenter studies are needed to confirm our findings in this subgroup of KD patients.
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Aneurisma Coronário/tratamento farmacológico , Aneurisma Coronário/etiologia , Ciclofosfamida/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/complicações , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
OBJECTIVES: To characterize the socioeconomic and racial and/or ethnic disparities impacting the diagnosis and outcomes of multisystem inflammatory syndrome in children (MIS-C). METHODS: This multicenter retrospective case-control study was conducted at 3 academic centers from January 1 to September 1, 2020. Children with MIS-C were compared with 5 control groups: children with coronavirus disease 2019, children evaluated for MIS-C who did not meet case patient criteria, children hospitalized with febrile illness, children with Kawasaki disease, and children in Massachusetts based on US census data. Neighborhood socioeconomic status (SES) and social vulnerability index (SVI) were measured via a census-based scoring system. Multivariable logistic regression was used to examine associations between SES, SVI, race and ethnicity, and MIS-C diagnosis and clinical severity as outcomes. RESULTS: Among 43 patients with MIS-C, 19 (44%) were Hispanic, 11 (26%) were Black, and 12 (28%) were white; 22 (51%) were in the lowest quartile SES, and 23 (53%) were in the highest quartile SVI. SES and SVI were similar between patients with MIS-C and coronavirus disease 2019. In multivariable analysis, lowest SES quartile (odds ratio 2.2 [95% confidence interval 1.1-4.4]), highest SVI quartile (odds ratio 2.8 [95% confidence interval 1.5-5.1]), and racial and/or ethnic minority background were associated with MIS-C diagnosis. Neither SES, SVI, race, nor ethnicity were associated with disease severity. CONCLUSIONS: Lower SES or higher SVI, Hispanic ethnicity, and Black race independently increased risk for MIS-C. Additional studies are required to target interventions to improve health equity for children.
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Negro ou Afro-Americano/estatística & dados numéricos , COVID-19/etnologia , Hispânico ou Latino/estatística & dados numéricos , Fatores Socioeconômicos , Síndrome de Resposta Inflamatória Sistêmica/etnologia , População Branca/estatística & dados numéricos , COVID-19/epidemiologia , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Massachusetts/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Determinantes Sociais da Saúde , Síndrome de Resposta Inflamatória Sistêmica/epidemiologiaRESUMO
OBJECTIVES: To determine predictors of >1 emergency department (ED) visit for a Kawasaki disease diagnosis in a quaternary care pediatric hospital and compare outcomes between patients with 1 vs >1 visit for Kawasaki disease diagnosis. STUDY DESIGN: Medical records of patients evaluated for Kawasaki disease between January 2006 and August 2018 at Boston Children's Hospital were abstracted for demographic and clinical data. Predictors of >1 visit were explored using logistic regression and classification and regression tree analysis. RESULTS: Of 530 patients diagnosed with Kawasaki disease, 117 (22%) required multiple ED visits for Kawasaki disease diagnosis. Multivariable regression and classification and regression tree analysis identified ≤2 Kawasaki disease criteria (OR 33.9; 95% CI 18.1-63.6), <3 days of fever at the first visit (OR 3.47; 95% CI 1.77-6.84), and non-White race (OR 2.15; 95% CI 1.18-3.95) as predictors of >1 visit. There were no significant differences in duration of hospitalization, day of illness at initial Kawasaki disease treatment, intravenous immunoglobulin resistance, need for adjunctive therapies, or coronary artery outcomes between patients diagnosed with Kawasaki disease at initial visit vs subsequent visits. CONCLUSIONS: Incomplete Kawasaki disease criteria, fewer days of fever, and non-White race were significant predictors of multiple ED visits for Kawasaki disease diagnosis in this single institution study. Our findings underscore the importance of maintaining a high index of suspicion for Kawasaki disease in patients with <4 Kawasaki disease criteria. Further research is needed to determine causes for increased healthcare use in non-White patients to receive a Kawasaki disease diagnosis.
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Diagnóstico Tardio/estatística & dados numéricos , Serviço Hospitalar de Emergência , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Adolescente , Boston/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Modelos Logísticos , Masculino , Síndrome de Linfonodos Mucocutâneos/etnologia , Síndrome de Linfonodos Mucocutâneos/terapia , Prognóstico , Estudos RetrospectivosRESUMO
BACKGROUND: Children are at risk for multisystem inflammatory syndrome in children (MIS-C) after infection with severe acute respiratory syndrome coronavirus 2. Cardiovascular complications, including ventricular dysfunction and coronary dilation, are frequent, but there are limited data on arrhythmic complications. METHODS: Retrospective cohort study of children and young adults aged ≤21 years admitted with MIS-C. Demographic characteristics, electrocardiogram (ECG) and echocardiogram findings, and hospital course were described. RESULTS: Among 25 patients admitted with MIS-C (60% male; median age 9.7 [interquartile range 2.7-15.0] years), ECG anomalies were found in 14 (56%). First-degree atrioventricular block (AVB) was seen in 5 (20%) patients a median of 6 (interquartile range 5-8) days after onset of fever and progressed to second- or third-degree AVB in 4 patients. No patient required intervention for AVB. All patients with AVB were admitted to the ICU (before onset of AVB) and had ventricular dysfunction on echocardiograms. All patients with second- or third-degree AVB had elevated brain natriuretic peptide levels, whereas the patient with first-degree AVB had a normal brain natriuretic peptide level. No patient with AVB had an elevated troponin level. QTc prolongation was seen in 7 patients (28%), and nonspecific ST segment changes were seen in 14 patients (56%). Ectopic atrial tachycardia was observed in 1 patient, and none developed ventricular arrhythmias. CONCLUSIONS: Children with MIS-C are at risk for atrioventricular conduction disease, especially those who require ICU admission and have ventricular dysfunction. ECGs should be monitored for evidence of PR prolongation. Continuous telemetry may be required in patients with evidence of first-degree AVB because of risk of progression to high-grade AVB.
Assuntos
Bloqueio Atrioventricular/etiologia , Betacoronavirus , Infecções por Coronavirus/complicações , Pneumonia Viral/complicações , Síndrome de Resposta Inflamatória Sistêmica/complicações , Adolescente , Bloqueio Atrioventricular/diagnóstico , Bloqueio Atrioventricular/epidemiologia , COVID-19 , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Pandemias , Estudos Retrospectivos , Fatores de Risco , SARS-CoV-2 , Adulto JovemRESUMO
BACKGROUNDPediatric SARS-CoV-2 infection can be complicated by a dangerous hyperinflammatory condition termed multisystem inflammatory syndrome in children (MIS-C). The clinical and immunologic spectrum of MIS-C and its relationship to other inflammatory conditions of childhood have not been studied in detail.METHODSWe retrospectively studied confirmed cases of MIS-C at our institution from March to June 2020. The clinical characteristics, laboratory studies, and treatment response were collected. Data were compared with historic cohorts of Kawasaki disease (KD) and macrophage activation syndrome (MAS).RESULTSTwenty-eight patients fulfilled the case definition of MIS-C. Median age at presentation was 9 years (range: 1 month to 17 years); 50% of patients had preexisting conditions. All patients had laboratory confirmation of SARS-CoV-2 infection. Seventeen patients (61%) required intensive care, including 7 patients (25%) who required inotrope support. Seven patients (25%) met criteria for complete or incomplete KD, and coronary abnormalities were found in 6 cases. Lymphopenia, thrombocytopenia, and elevation in inflammatory markers, D-dimer, B-type natriuretic peptide, IL-6, and IL-10 levels were common but not ubiquitous. Cytopenias distinguished MIS-C from KD and the degree of hyperferritinemia and pattern of cytokine production differed between MIS-C and MAS. Immunomodulatory therapy given to patients with MIS-C included intravenous immune globulin (IVIG) (71%), corticosteroids (61%), and anakinra (18%). Clinical and laboratory improvement were observed in all cases, including 6 cases that did not require immunomodulatory therapy. No mortality was recorded in this cohort.CONCLUSIONMIS-C encompasses a broad phenotypic spectrum with clinical and laboratory features distinct from KD and MAS.FUNDINGThis work was supported by the National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases; the National Institute of Allergy and Infectious Diseases; Rheumatology Research Foundation Investigator Awards and Medical Education Award; Boston Children's Hospital Faculty Career Development Awards; the McCance Family Foundation; and the Samara Jan Turkel Center.
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Corticosteroides/administração & dosagem , Betacoronavirus/metabolismo , Imunoglobulinas Intravenosas/administração & dosagem , Imunomodulação , Proteína Antagonista do Receptor de Interleucina 1/administração & dosagem , Síndrome de Resposta Inflamatória Sistêmica , Adolescente , Biomarcadores/sangue , COVID-19 , Criança , Pré-Escolar , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/metabolismo , Humanos , Lactente , Interleucina-10/sangue , Interleucina-6/sangue , Síndrome de Ativação Macrofágica/sangue , Síndrome de Ativação Macrofágica/diagnóstico , Síndrome de Ativação Macrofágica/tratamento farmacológico , Síndrome de Ativação Macrofágica/imunologia , Masculino , Síndrome de Linfonodos Mucocutâneos/sangue , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Síndrome de Linfonodos Mucocutâneos/imunologia , Peptídeo Natriurético Encefálico/sangue , Estudos Retrospectivos , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica/sangue , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/tratamento farmacológico , Síndrome de Resposta Inflamatória Sistêmica/imunologiaRESUMO
OBJECTIVE: To evaluate the association of neighborhood socioeconomic status (SES) with time to intravenous immunoglobulin treatment, length of stay (LOS), and coronary artery aneurysms (CAAs) in patients with Kawasaki disease. STUDY DESIGN: We examined the relationship of SES in 915 patients treated at a large academic center between 2000 and 2017. Neighborhood SES was measured using a US census-based score derived from 6 measures related to income, education, and occupation. Linear and logistic regression were used to examine the association of SES with number of days of fever at time of treatment, LOS, and CAA. RESULTS: Patients in the lowest SES quartile were treated later than patients with greater SES (7 [IQR 5, 9] vs 6 [IQR 5, 8] days, P = .01). Patients in the lowest SES quartile were more likely to be treated after 10 days of illness, with an OR 1.9 (95% CI 1.3-2.8). In multivariable analysis, SES remained an independent predictor of the number of days of fever at time of treatment (P = .01). Patients in the lowest SES quartile had longer LOS than patients with greater SES (3 [IQR 2, 5] vs 3 [IQR 2, 4], P = .007). In subgroup analysis of white children, those in the lowest SES quartile vs quartiles 2-4 were more likely to develop large/giant CAA 17 (12%) vs 30 (6%), P = .03. CONCLUSIONS: Lower SES is associated with delayed treatment, prolonged LOS, and increased risk of large/giant CAA. Novel approaches to diagnosis and education are needed for children living in low-SES neighborhoods.
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Disparidades nos Níveis de Saúde , Tempo de Internação/estatística & dados numéricos , Síndrome de Linfonodos Mucocutâneos/terapia , Classe Social , Tempo para o Tratamento , Pré-Escolar , Aneurisma Coronário/etiologia , Feminino , Humanos , Lactente , Modelos Lineares , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Características de Residência/estatística & dados numéricos , Fatores de RiscoRESUMO
BACKGROUND: Coronary artery aneurysms (CAA) are a serious complication of Kawasaki disease. Treatment with intravenous immunoglobulin (IVIg) within 10 days of fever onset reduces the risk of CAA from 25% to <5%. Corticosteroids and infliximab are often used in high-risk patients or those with CAA at diagnosis, but there are no data on their longer-term impact on CAA. METHODS: Retrospective multicenter study including children who had CAA with a z score ≥2.5 and <10 at time of diagnosis and who received primary therapy with IVIg alone or in combination with either corticosteroids or infliximab within 10 days of onset of fever. RESULTS: Of 121 children, with a median age of 2.8 (range 0.1-15.5) years, 30 (25%) received primary therapy with corticosteroids and IVIg, 58 (48%) received primary therapy with infliximab and IVIg, and 33 (27%) received primary therapy with IVIg only. Median coronary z scores at the time of diagnosis did not differ among treatment groups (P = .39). Primary treatment intensification with either corticosteroids or infliximab were independent protective factors against progression of coronary size on follow-up (coefficient: -1.31 [95% confidence interval: -2.33 to -0.29]; coefficient: -1.07 [95% confidence interval: -1.95 to -0.19], respectively). CONCLUSIONS: Among a high-risk group of patients with Kawasaki disease with CAA on baseline echocardiography, those treated with corticosteroids or infliximab in addition to IVIg had less progression in CAA size compared with those treated with IVIg alone. Prospective randomized trials are needed to determine the best adjunctive treatment of patients who present with CAA.
Assuntos
Aneurisma Coronário/tratamento farmacológico , Aneurisma Coronário/epidemiologia , Imunoglobulinas Intravenosas/administração & dosagem , Infliximab/administração & dosagem , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Adolescente , Antirreumáticos/administração & dosagem , Criança , Pré-Escolar , Aneurisma Coronário/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
Background Accurate prediction of coronary artery aneurysms ( CAAs ) in patients with Kawasaki disease remains challenging in North American cohorts. We sought to develop and validate a risk model for CAA prediction. Methods and Results A binary outcome of CAA was defined as left anterior descending or right coronary artery Z score ≥2.5 at 2 to 8 weeks after fever onset in a development cohort (n=903) and a validation cohort (n=185) of patients with Kawasaki disease. Associations of baseline clinical, laboratory, and echocardiographic variables with later CAA were assessed in the development cohort using logistic regression. Discrimination (c statistic) and calibration (Hosmer-Lemeshow) of the final model were evaluated. A practical risk score assigning points to each variable in the final model was created based on model coefficients from the development cohort. Predictors of CAAs at 2 to 8 weeks were baseline Z score of left anterior descending or right coronary artery ≥2.0, age <6 months, Asian race, and C-reactive protein ≥13 mg/ dL (c=0.82 in the development cohort, c=0.93 in the validation cohort). The CAA risk score assigned 2 points for baseline Z score of left anterior descending or right coronary artery ≥2.0 and 1 point for each of the other variables, with creation of low- (0-1), moderate- (2), and high- (3-5) risk groups. The odds of CAA s were 16-fold greater in the high- versus the low-risk groups in the development cohort (odds ratio, 16.4; 95% CI , 9.71-27.7 [ P<0.001]), and >40-fold greater in the validation cohort (odds ratio, 44.0; 95% CI, 10.8-180 [ P<0.001]). Conclusions Our risk model for CAA in Kawasaki disease consisting of baseline demographic, laboratory, and echocardiographic variables had excellent predictive utility and should undergo prospective testing.
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Aneurisma Coronário/etiologia , Síndrome de Linfonodos Mucocutâneos/complicações , Adolescente , Fatores Etários , Asiático , Biomarcadores/sangue , Boston , Proteína C-Reativa/análise , California , Criança , Pré-Escolar , Aneurisma Coronário/diagnóstico por imagem , Aneurisma Coronário/etnologia , Progressão da Doença , Ecocardiografia , Feminino , Humanos , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/etnologia , Valor Preditivo dos Testes , Estudos Prospectivos , Ensaios Clínicos Controlados Aleatórios como Assunto , Reprodutibilidade dos Testes , Estudos Retrospectivos , Medição de Risco , Fatores de RiscoRESUMO
BACKGROUND: The American College of Cardiology Quality Network enables national benchmarking and collaborative quality improvement through vetted metrics. We describe here our initial experience with the Quality Network. METHODS: Quarterly data for metrics pertaining to chest pain, Kawasaki disease, tetralogy of Fallot, elevated body mass index, and others were shared with the collaboratives for benchmarking. National improvement efforts focussed on counselling for elevated body mass index and 22q11.2 testing in tetralogy of Fallot. Improvement strategies included developing multi-disciplinary workgroups, educational materials, and electronic health record advances. RESULTS: Chest pain metric performance was high compared with national means: obtaining family history (90-100% versus 51-77%), electrocardiogram (100% versus 89-99%), and echocardiogram for exertional complaints (95-100% versus 74-96%). Kawasaki metric performance was high, including obtaining coronary measurements (100% versus 85-97%), prescribing aspirin (100% versus 86-99%), follow-up with imaging (100% versus 85-98%), and documenting no activity restriction without coronary aneurysms (83-100% versus 64-93%). Counselling for elevated body mass index was variable (25-75% versus 31-50%) throughout quality improvement efforts. Testing for 22q11.2 deletion in tetralogy of Fallot patients was consistently above the national mean (60-85% versus 54-68%) with improved genetics data capture. CONCLUSION: The Quality Network promotes meaningful benchmarking and collaborative quality improvement. Our high performance for chest pain and Kawasaki metrics is likely related to previous improvement efforts in chest pain management and a dedicated Kawasaki team. Uptake of counselling for elevated body mass index is variable; stronger engagement among numerous providers is needed. Recommendations for 22q11.2 testing in tetralogy of Fallot were widely recognised and implemented.
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Assistência Ambulatorial/normas , Cardiologia/normas , Comportamento Cooperativo , Cardiopatias Congênitas/terapia , Melhoria de Qualidade/organização & administração , Adulto , Benchmarking , Criança , Humanos , Sociedades Médicas , Estados UnidosRESUMO
Importance: American Heart Association guidelines recommend echocardiography in Kawasaki disease at baseline, 1 to 2 weeks, and 4 to 6 weeks after treatment to detect coronary artery abnormalities. However, these examinations are expensive and may require sedation in young children, which is burdensome and carries some risk. Objective: To assess the benefit of additional echocardiographic imaging at 6 weeks in patients with uncomplicated Kawasaki disease who had previously normal coronary arteries. Design, Setting, and Participants: This is a retrospective review of patients with Kawasaki disease who were cared for between 1995 and 2014 in 2 academic pediatric referral practices Eligibility criteria included receiving intravenous immunoglobulin treatment for acute Kawasaki disease at a center; the absence of significant congenital heart disease; available echocardiographic measurements of both the right and left anterior descending coronary arteries at 10 days or less after diagnosis (baseline), 2 (±1) weeks, and 6 (±3) weeks of illness; and normal coronary arteries at baseline and 2 weeks, defined as maximum coronary artery z scores less than 2.0 and no distal aneurysms. Data analysis was completed from March 2015 to November 2015. Main Outcomes and Measures: The number of patients with right coronary artery or left anterior descending coronary artery z scores of 2.0 or more at 6 weeks. Results: The median age of the 464 included patients was 3.3 years (interquartile range, 1.8-5.4 years); 264 (56.9%) were male, 351 of 414 for whom data were available (84.8%) had complete Kawasaki disease, and 66 (14.2%) received additional intravenous immunoglobulin treatment. At 6 weeks of illness, 456 patients (98.3%) who had had normal coronary artery z scores at baseline and 2 weeks continued to have normal z scores. Of the remaining 8 patients (1.7%), the maximum z score within 6 weeks was 2.0 to 2.4 in 5 patients (1.2%), 2.5 to 2.9 in 1 patient (0.2%), and 3.0 or more in 2 patients (0.4% [95% CI, 0.1%-1.5%]). Coronary artery dimensions ultimately normalized in all but 1 patient, who had minimal dilation at 6 weeks (right coronary artery z score, 2.1). Sensitivity analyses using less restrictive cut points (eg, a maximum z score <2.5) or less restrictive timing windows (eg, considering patients with incomplete echocardiographic data within 21 days) gave similar results; in these analyses, 454 to 463 of 464 patients (98% to 99.7%) had coronary artery z scores of less than 2.5 at 6 weeks. Conclusions and Relevance: New abnormalities in coronary arteries are rarely detected at 6 weeks in patients with Kawasaki disease who have normal measurements at baseline and 2 weeks of illness, suggesting that the 6-week echocardiographic imaging may be unnecessary in patients with uncomplicated Kawasaki disease and z scores less than 2.0 in the first 2 weeks of illness.
Assuntos
Vasos Coronários/diagnóstico por imagem , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Pré-Escolar , Doença da Artéria Coronariana/diagnóstico por imagem , Doença da Artéria Coronariana/patologia , Vasos Coronários/patologia , Ecocardiografia , Feminino , Seguimentos , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: Accurate risk prediction of coronary artery aneurysms (CAAs) in North American children with Kawasaki disease remains a clinical challenge. We sought to determine the predictive utility of baseline coronary dimensions adjusted for body surface area (z scores) for future CAAs in Kawasaki disease and explored the extent to which addition of established Japanese risk scores to baseline coronary artery z scores improved discrimination for CAA development. METHODS AND RESULTS: We explored the relationships of CAA with baseline z scores; with Kobayashi, Sano, Egami, and Harada risk scores; and with the combination of baseline z scores and risk scores. We defined CAA as a maximum z score (zMax) ≥2.5 of the left anterior descending or right coronary artery at 4 to 8 weeks of illness. Of 261 patients, 77 patients (29%) had a baseline zMax ≥2.0. CAAs occurred in 15 patients (6%). CAAs were strongly associated with baseline zMax ≥2.0 versus <2.0 (12 [16%] versus 3 [2%], respectively, P<0.001). Baseline zMax ≥2.0 had a C statistic of 0.77, good sensitivity (80%), and excellent negative predictive value (98%). None of the risk scores alone had adequate discrimination. When high-risk status per the Japanese risk scores was added to models containing baseline zMax ≥2.0, none were significantly better than baseline zMax ≥2.0 alone. CONCLUSIONS: In a North American center, baseline zMax ≥2.0 in children with Kawasaki disease demonstrated high predictive utility for later development of CAA. Future studies should validate the utility of our findings.
Assuntos
Aneurisma Coronário/etiologia , Vasos Coronários/diagnóstico por imagem , Técnicas de Apoio para a Decisão , Ecocardiografia , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Fatores Etários , Pré-Escolar , Aneurisma Coronário/diagnóstico , Feminino , Humanos , Imunossupressores/uso terapêutico , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , América do Norte , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Medição de Risco , Fatores de RiscoRESUMO
OBJECTIVE: To describe the safety and efficacy of warfarin for patients with Kawasaki disease and giant coronary artery aneurysms (CAAs, ≥8 mm). Giant aneurysms are managed with combined anticoagulation and antiplatelet therapies, heightening risk of bleeding complications. STUDY DESIGN: We reviewed the time in therapeutic range; percentage of international normalization ratios (INRs) in range (%); bleeding events, clotting events; INRs ≥6; INRs ≥5 and <6; and INRs <1.5. RESULTS: In 9 patients (5 male), median age 14.4 years (range 7.1-22.8 years), INR testing was prescribed weekly to monthly and was done by home monitor (n = 5) or laboratory (n = 3) or combined (1). Median length of warfarin therapy was 7.2 years (2.3-13.3 years). Goal INR was 2.0-3.0 (n = 6) or 2.5-3.5 (n = 3), based on CAA size and history of CAA thrombosis. All patients were treated with aspirin; 1 was on dual antiplatelet therapy and warfarin. The median time in therapeutic range was 59% (37%-85%), and median percentage of INRs in range was 68% (52%-87%). INR >6 occurred in 3 patients (4 events); INRs ≥5 <6 in 7 patients (12 events); and INR <1.5 in 5 patients (28 events). The incidence of major bleeding events and clinically relevant nonmajor bleeding events were each 4.3 per 100 patient-years (95% CI 0.9-12.6). New asymptomatic coronary thrombosis was detected by imaging in 2 patients. CONCLUSIONS: Bleeding and clotting complications are common in patients with Kawasaki disease on warfarin and aspirin, with INRs in range only two-thirds of the time. Future studies should evaluate the use of direct oral anticoagulants in children as an alternative to warfarin.
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Anticoagulantes/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Varfarina/uso terapêutico , Adolescente , Anticoagulantes/efeitos adversos , Criança , Feminino , Hemorragia/induzido quimicamente , Hemorragia/epidemiologia , Humanos , Incidência , Coeficiente Internacional Normatizado , Masculino , Estudos Retrospectivos , Trombose/induzido quimicamente , Trombose/epidemiologia , Varfarina/efeitos adversos , Adulto JovemRESUMO
BACKGROUND: Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries. METHODS AND RESULTS: To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. CONCLUSIONS: These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances.
Assuntos
American Heart Association , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Síndrome de Linfonodos Mucocutâneos/terapia , Algoritmos , Tomada de Decisão Clínica , Consenso , Procedimentos Clínicos/normas , Técnicas de Apoio para a Decisão , Humanos , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Valor Preditivo dos Testes , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Estados UnidosAssuntos
Ecocardiografia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Adolescente , Adulto , Criança , Pré-Escolar , Comorbidade , Feminino , Humanos , Lactente , Masculino , Prevalência , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Coronary artery (CA) Z scores are commonly used for clinical decisions in Kawasaki disease, including treatment, anticoagulation, and duration and frequency of follow-up. The aim of this study was to evaluate CA measurement reproducibility, Z score calculation variability, and the impact of variability on management. METHODS: Twenty-one patients with Kawasaki disease with right CA (RCA) or left anterior descending CA (LAD) Z scores of 1.5 to 3 (group 1) were randomly selected, and all patients with Kawasaki disease with Z scores of 7 to 14 for either the RCA or LAD (n = 20; group 2) were included from March 2008 to May 2014. Two echocardiographers measured left main CA, LAD, and RCA dimensions. The inter- and intraobserver reliability of absolute measurements was calculated, and the CA Z scores derived from three commonly used formulas were compared. RESULTS: Median age at echocardiography was 1.2 years (range, 0.2-11.5 years), and 68% of subjects (n = 28) were male. Interobserver reliability was high for the LAD (intraclass correlation coefficient [ICC], 96.79%) and RCA (ICC, 93.31%) and lower for the left main CA (ICC, 73.54%). Intraobserver reliability was also high for the LAD and RCA (ICC, 99.08% and 97.74%) and lower for the left main CA (ICC, 80.88%). Calculated Z scores were similar among the three formulas for group 1 but varied markedly in group 2. Calculated Z scores using the same CA measurement in each of the three formulas resulted in different clinical management in up to seven of 21 group 1 patients (22%) and in up to 10 of 20 group 2 patients (50%). CONCLUSIONS: Although CA measurements have high inter- and intraobserver agreement, CA Z scores vary dramatically on the basis of the Z score formula at larger CA dimensions. Discrepancies in CA Z score calculators may affect clinical decision making.
Assuntos
Aneurisma Coronário/diagnóstico por imagem , Ecocardiografia/métodos , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Criança , Pré-Escolar , Aneurisma Coronário/fisiopatologia , Feminino , Humanos , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/fisiopatologia , Reprodutibilidade dos TestesRESUMO
OBJECTIVE: It has been claimed that the aneurysm rate for Kawasaki disease (KD) patients in Japan is lower than in the U.S. However it has been difficult to compare coronary artery (CA) outcomes between the two countries because of different definitions for CA abnormalities. Therefore, we compared CA internal diameters between Japanese and U.S. KD patients using standard definitions and methods. STUDY DESIGN: We retrospectively reviewed CA outcomes in 1082 KD patients from 2 centers in the U.S. and 3 centers in Japan and compared Z-max scores (maximum internal diameter for the left anterior descending or right coronary artery expressed as standard deviation units from the mean (Z-score) normalized for body surface area) obtained within 12 weeks after onset and calculated using two different regression equations from Canada (Dallaire) and Japan (Fuse). We defined a Z-max of < 2.5 as normal and a Z-max of ≥ 10 as giant aneurysm. RESULT: The median Z-max for the U.S. and Japanese subjects was 1.9 and 2.3 SD units, respectively (p < 0.001). There was no significant difference in rates of patients with Z-max ≥ 5.0 between the countries. In a multivariable model adjusting for age, sex, and treatment response, being Japanese was still associated with a higher Z-max score. CONCLUSION: Previously reported differences in aneurysm rates between Japan and the U.S. likely resulted from use of different definitions and nomenclature. Adoption of Z-scores as a standard for reporting CA internal diameters will allow meaningful comparisons among different countries and will facilitate international, collaborative clinical trials.