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2.
World J Pediatr ; 20(2): 143-152, 2024 02.
Artigo em Inglês | MEDLINE | ID: mdl-38133726

RESUMO

BACKGROUND: Recent infection with SARS­CoV­2 in children has been associated with multisystem inflammatory syndrome in children (MIS-C). SARS­CoV­2 has undergone different mutations. Few publications exist about specific variants and their correlation with the severity of MIS-C. METHODS: This was a single-center, retrospective study including all patients admitted with MIS-C at Rady Children's Hospital-San Diego between May 2020 and March 2022. Local epidemiologic data, including viral genomic information, were obtained from public records. Demographics, clinical presentation, laboratory values, and outcomes were obtained from electronic medical records. RESULTS: The analysis included 104 pediatric patients. Four MIS-C waves were identified. Circulating variants in San Diego during the first wave included clades 20A to C. During the second wave, there were variants from clades 20A to C, 20G, 21C (Epsilon), 20I (Alpha), and 20J (Gamma). The third wave had Delta strains (clades 21A, 21I, and 21J), and the fourth had Omicron variants (clades 21K, 21L, and 22C). MIS-C presented with similar symptoms and laboratory findings across all waves. More patients were admitted to the pediatric intensive care unit (PICU) (74%) and required inotropic support (63%) during the second wave. None of the patients required mechanical circulatory support, and only two required invasive ventilatory support. There was no mortality. CONCLUSIONS: The various strains of SARS-CoV-2 triggered MIS-C with differing severities, with the second wave having a more severe clinical course. Whether the differences in disease severity across variants were due to changes in the virus or other factors remains unknown.


Assuntos
COVID-19/complicações , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica , Humanos , Criança , SARS-CoV-2/genética , Estudos Retrospectivos , Progressão da Doença
3.
JACC Case Rep ; 26: 102077, 2023 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-38094174

RESUMO

The hypercoagulable state in Kawasaki disease (KD) may lead to complex cardiovascular sequelae. We present the case of a 2-month-old infant with complete KD complicated by giant coronary artery aneurysms, coronary sinus thrombosis, and post-myocardial infarction syndrome (Dressler syndrome), resulting in 2 distinct episodes of pericardial effusion. (Level of Difficulty: Intermediate.).

6.
Lancet Child Adolesc Health ; 7(10): 697-707, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37598693

RESUMO

BACKGROUND: Although Kawasaki disease is commonly regarded as a single disease entity, variability in clinical manifestations and disease outcome has been recognised. We aimed to use a data-driven approach to identify clinical subgroups. METHODS: We analysed clinical data from patients with Kawasaki disease diagnosed at Rady Children's Hospital (San Diego, CA, USA) between Jan 1, 2002, and June 30, 2022. Patients were grouped by hierarchical clustering on principal components with k-means parcellation based on 14 variables, including age at onset, ten laboratory test results, day of illness at the first intravenous immunoglobulin infusion, and normalised echocardiographic measures of coronary artery diameters at diagnosis. We also analysed the seasonality and Kawasaki disease incidence from 2002 to 2019 by subgroup. To explore the biological underpinnings of identified subgroups, we did differential abundance analysis on proteomic data of 6481 proteins from 32 patients with Kawasaki disease and 24 healthy children, using linear regression models that controlled for age and sex. FINDINGS: Among 1016 patients with complete data in the final analysis, four subgroups were identified with distinct clinical features: (1) hepatobiliary involvement with elevated alanine transaminase, gamma-glutamyl transferase, and total bilirubin levels, lowest coronary artery aneurysm but highest intravenous immunoglobulin resistance rates (n=157); (2) highest band neutrophil count and Kawasaki disease shock rate (n=231); (3) cervical lymphadenopathy with high markers of inflammation (erythrocyte sedimentation rate, C-reactive protein, white blood cell, and platelet counts) and lowest age-adjusted haemoglobin Z scores (n=315); and (4) young age at onset with highest coronary artery aneurysm but lowest intravenous immunoglobulin resistance rates (n=313). The subgroups had distinct seasonal and incidence trajectories. In addition, the subgroups shared 211 differential abundance proteins while many proteins were unique to a subgroup. INTERPRETATION: Our data-driven analysis provides insight into the heterogeneity of Kawasaki disease, and supports the existence of distinct subgroups with important implications for clinical management and research design and interpretation. FUNDING: US National Institutes of Health and the Irving and Francine Suknow Foundation.


Assuntos
Aneurisma , Síndrome de Linfonodos Mucocutâneos , Estados Unidos , Humanos , Criança , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Imunoglobulinas Intravenosas/uso terapêutico , Proteômica , Análise por Conglomerados , Aneurisma/tratamento farmacológico
7.
JAMA Netw Open ; 6(5): e2314291, 2023 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-37200028

RESUMO

Importance: Cardiac dysfunction and myocarditis have emerged as serious complications of multisystem inflammatory syndrome in children (MIS-C) and vaccines against SARS-CoV-2. Understanding the role of autoantibodies in these conditions is essential for guiding MIS-C management and vaccination strategies in children. Objective: To investigate the presence of anticardiac autoantibodies in MIS-C or COVID-19 vaccine-induced myocarditis. Design, Setting, and Participants: This diagnostic study included children with acute MIS-C or acute vaccine myocarditis, adults with myocarditis or inflammatory cardiomyopathy, healthy children prior to the COVID-19 pandemic, and healthy COVID-19 vaccinated adults. Participants were recruited into research studies in the US, United Kingdom, and Austria starting January 2021. Immunoglobulin G (IgG), IgM, and IgA anticardiac autoantibodies were identified with immunofluorescence staining of left ventricular myocardial tissue from 2 human donors treated with sera from patients and controls. Secondary antibodies were fluorescein isothiocyanate-conjugated antihuman IgG, IgM, and IgA. Images were taken for detection of specific IgG, IgM, and IgA deposits and measurement of fluorescein isothiocyanate fluorescence intensity. Data were analyzed through March 10, 2023. Main Outcomes and Measures: IgG, IgM and IgA antibody binding to cardiac tissue. Results: By cohort, there were a total of 10 children with MIS-C (median [IQR] age, 10 [13-14] years; 6 male), 10 with vaccine myocarditis (median age, 15 [14-16] years; 10 male), 8 adults with myocarditis or inflammatory cardiomyopathy (median age, 55 [46-63] years; 6 male), 10 healthy pediatric controls (median age, 8 [13-14] years; 5 male), and 10 healthy vaccinated adults (all older than 21 years, 5 male). No antibody binding above background was observed in human cardiac tissue treated with sera from pediatric patients with MIS-C or vaccine myocarditis. One of the 8 adult patients with myocarditis or cardiomyopathy had positive IgG staining with raised fluorescence intensity (median [IQR] intensity, 11 060 [10 223-11 858] AU). There were no significant differences in median fluorescence intensity in all other patient cohorts compared with controls for IgG (MIS-C, 6033 [5834-6756] AU; vaccine myocarditis, 6392 [5710-6836] AU; adult myocarditis or inflammatory cardiomyopathy, 5688 [5277-5990] AU; healthy pediatric controls, 6235 [5924-6708] AU; healthy vaccinated adults, 7000 [6423-7739] AU), IgM (MIS-C, 3354 [3110-4043] AU; vaccine myocarditis, 3843 [3288-4748] AU; healthy pediatric controls, 3436 [3313-4237] AU; healthy vaccinated adults, 3543 [2997-4607] AU) and IgA (MIS-C, 3559 [2788-4466] AU; vaccine myocarditis, 4389 [2393-4780] AU; healthy pediatric controls, 3436 [2425-4077] AU; healthy vaccinated adults, 4561 [3164-6309] AU). Conclusions and Relevance: This etiological diagnostic study found no evidence of antibodies from MIS-C and COVID-19 vaccine myocarditis serum binding cardiac tissue, suggesting that the cardiac pathology in both conditions is unlikely to be driven by direct anticardiac antibody-mediated mechanisms.


Assuntos
COVID-19 , Miocardite , Adulto , Humanos , Masculino , Criança , Adolescente , Pessoa de Meia-Idade , Miocardite/etiologia , Vacinas contra COVID-19/efeitos adversos , Autoanticorpos , COVID-19/prevenção & controle , Pandemias , SARS-CoV-2 , Vacinação , Imunoglobulina G , Imunoglobulina A , Fluoresceínas , Imunoglobulina M
8.
Arch Dis Child ; 108(10): 833-838, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37258054

RESUMO

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.


Assuntos
Aneurisma Coronário , Doença da Artéria Coronariana , Síndrome de Linfonodos Mucocutâneos , Criança , Humanos , Lactente , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Infliximab/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Vasos Coronários , Aneurisma Coronário/tratamento farmacológico , Aneurisma Coronário/etiologia , Aneurisma Coronário/diagnóstico , Estudos Retrospectivos , Doença da Artéria Coronariana/tratamento farmacológico , Doença da Artéria Coronariana/etiologia
9.
JACC Case Rep ; 11: 101790, 2023 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-37077433

RESUMO

We present a 22-week fetus with isolated absent aortic valve and inverse circular shunt. The pregnancy was interrupted. Here, echocardiography and pathology images demonstrate this rare entity. Whole genome sequencing revealed a potentially disease-causing variant in the APC gene. Whole genome sequencing should be considered in severe and rare fetal diseases. (Level of Difficulty: Advanced.).

10.
JAMA Netw Open ; 6(1): e2248987, 2023 01 03.
Artigo em Inglês | MEDLINE | ID: mdl-36595296

RESUMO

Importance: Data are limited regarding adverse reactions after COVID-19 vaccination in patients with a history of multisystem inflammatory syndrome in children (MIS-C). The lack of vaccine safety data in this unique population may cause hesitancy and concern for many families and health care professionals. Objective: To describe adverse reactions following COVID-19 vaccination in patients with a history of MIS-C. Design, Setting, and Participants: In this multicenter cross-sectional study including 22 North American centers participating in a National Heart, Lung, and Blood Institute, National Institutes of Health-sponsored study, Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children (MUSIC), patients with a prior diagnosis of MIS-C who were eligible for COVID-19 vaccination (age ≥5 years; ≥90 days after MIS-C diagnosis) were surveyed between December 13, 2021, and February 18, 2022, regarding COVID-19 vaccination status and adverse reactions. Exposures: COVID-19 vaccination after MIS-C diagnosis. Main Outcomes and Measures: The main outcome was adverse reactions following COVID-19 vaccination. Comparisons were made using the Wilcoxon rank sum test for continuous variables and the χ2 or Fisher exact test for categorical variables. Results: Of 385 vaccine-eligible patients who were surveyed, 185 (48.1%) received at least 1 vaccine dose; 136 of the vaccinated patients (73.5%) were male, and the median age was 12.2 years (IQR, 9.5-14.7 years). Among vaccinated patients, 1 (0.5%) identified as American Indian/Alaska Native, non-Hispanic; 9 (4.9%) as Asian, non-Hispanic; 45 (24.3%) as Black, non-Hispanic; 59 (31.9%) as Hispanic or Latino; 53 (28.6%) as White, non-Hispanic; 2 (1.1%) as multiracial, non-Hispanic; and 2 (1.1%) as other, non-Hispanic; 14 (7.6%) had unknown or undeclared race and ethnicity. The median time from MIS-C diagnosis to first vaccine dose was 9.0 months (IQR, 5.1-11.9 months); 31 patients (16.8%) received 1 dose, 142 (76.8%) received 2 doses, and 12 (6.5%) received 3 doses. Almost all patients received the BNT162b2 vaccine (347 of 351 vaccine doses [98.9%]). Minor adverse reactions were observed in 90 patients (48.6%) and were most often arm soreness (62 patients [33.5%]) and/or fatigue (32 [17.3%]). In 32 patients (17.3%), adverse reactions were treated with medications, most commonly acetaminophen (21 patients [11.4%]) or ibuprofen (11 [5.9%]). Four patients (2.2%) sought medical evaluation, but none required testing or hospitalization. There were no patients with any serious adverse events, including myocarditis or recurrence of MIS-C. Conclusions and Relevance: In this cross-sectional study of patients with a history of MIS-C, no serious adverse events were reported after COVID-19 vaccination. These findings suggest that the safety profile of COVID-19 vaccination administered at least 90 days following MIS-C diagnosis appears to be similar to that in the general population.


Assuntos
COVID-19 , Doenças do Tecido Conjuntivo , Estados Unidos/epidemiologia , Criança , Humanos , Masculino , Pré-Escolar , Feminino , Vacinas contra COVID-19/efeitos adversos , Vacina BNT162 , COVID-19/epidemiologia , COVID-19/prevenção & controle , Estudos Transversais , Vacinação/efeitos adversos
11.
Pediatr Cardiol ; 44(2): 381-387, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36383234

RESUMO

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


Assuntos
Aneurisma Coronário , Doença da Artéria Coronariana , Síndrome de Linfonodos Mucocutâneos , Isquemia Miocárdica , Humanos , Aneurisma Coronário/diagnóstico por imagem , Aneurisma Coronário/etiologia , Ecocardiografia sob Estresse , Síndrome de Linfonodos Mucocutâneos/complicações , Doença da Artéria Coronariana/complicações , Isquemia Miocárdica/complicações , Teste de Esforço , Angiografia Coronária
12.
Int J Mol Sci ; 23(13)2022 Jun 29.
Artigo em Inglês | MEDLINE | ID: mdl-35806225

RESUMO

We studied SARS-CoV-2-specific T cell responses in 22 subacute MIS-C children enrolled in 2021 and 2022 using peptide pools derived from SARS-CoV-2 spike or nonspike proteins. CD4+ and CD8+ SARS-CoV-2-specific T cells were detected in 5 subjects, CD4+ T helper (Th) responses alone were detected in 12 subjects, and CD8+ cytotoxic T cell (CTL) responses alone were documented in 1 subject. Notably, a sizeable subpopulation of CD4- CD8- double-negative (DN) T cells out of total CD3+ T cells was observed in MIS-C (median: 14.5%; IQR 8.65-25.3) and recognized SARS-CoV-2 peptides. T cells bearing the Vß21.3 T cell receptor (TcRs), previously reported as pathogenic in the context of MIS-C, were detected in high frequencies, namely, in 2.8% and 3.9% of the CD4+ and CD8+ T cells, respectively. However, Vß21.3 CD8+ T cells that responded to SARS-CoV-2 peptides were detected in only a single subject, suggesting recognition of nonviral antigens in the majority of subjects. Subjects studied 6-14 months after MIS-C showed T cell epitope spreading, meaning the activation of T cells that recognize more SARS-CoV-2 peptides following the initial expansion of T cells that see immunodominant epitopes. For example, subjects that did not recognize nonspike proteins in the subacute phase of MIS-C showed good Th response to nonspike peptides, and/or CD8+ T cell responses not appreciable before arose over time and could be detected in the 6-14 months' follow-up. The magnitude of the Th and CTL responses also increased over time. In summary, patients with MIS-C associated with acute lymphopenia, a classical feature of MIS-C, showed a physiological response to the virus with a prominent role for virus-specific DN T cells.


Assuntos
COVID-19 , SARS-CoV-2 , Linfócitos T CD4-Positivos , Linfócitos T CD8-Positivos , COVID-19/complicações , Criança , Humanos , Peptídeos/metabolismo , Síndrome de Resposta Inflamatória Sistêmica
13.
Clin Infect Dis ; 75(9): 1645-1648, 2022 10 29.
Artigo em Inglês | MEDLINE | ID: mdl-35445250

RESUMO

Our study demonstrates that children who developed severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination-induced myocarditis and may not receive another vaccination, could be susceptible to infection with Omicron and emerging variants. We observed higher neutralizing antibody titers in myocarditis patients vs. healthy vaccinated children, but significantly lower neutralization titers against Omicron in both groups.


Assuntos
COVID-19 , Miocardite , Criança , Humanos , SARS-CoV-2 , Testes de Neutralização , Anticorpos Antivirais , Miocardite/etiologia , COVID-19/prevenção & controle , Vacinação/efeitos adversos , Anticorpos Neutralizantes
14.
Circulation ; 145(5): 345-356, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34865500

RESUMO

BACKGROUND: Understanding the clinical course and short-term outcomes of suspected myocarditis after the coronavirus disease 2019 (COVID-19) vaccination has important public health implications in the decision to vaccinate youth. METHODS: We retrospectively collected data on patients <21 years old presenting before July 4, 2021, with suspected myocarditis within 30 days of COVID-19 vaccination. Lake Louise criteria were used for cardiac MRI findings. Myocarditis cases were classified as confirmed or probable on the basis of the Centers for Disease Control and Prevention definitions. RESULTS: We report on 139 adolescents and young adults with 140 episodes of suspected myocarditis (49 confirmed, 91 probable) at 26 centers. Most patients were male (n=126, 90.6%) and White (n=92, 66.2%); 29 (20.9%) were Hispanic; and the median age was 15.8 years (range, 12.1-20.3; interquartile range [IQR], 14.5-17.0). Suspected myocarditis occurred in 136 patients (97.8%) after the mRNA vaccine, with 131 (94.2%) after the Pfizer-BioNTech vaccine; 128 (91.4%) occurred after the second dose. Symptoms started at a median of 2 days (range, 0-22; IQR, 1-3) after vaccination. The most common symptom was chest pain (99.3%). Patients were treated with nonsteroidal anti-inflammatory drugs (81.3%), intravenous immunoglobulin (21.6%), glucocorticoids (21.6%), colchicine (7.9%), or no anti-inflammatory therapies (8.6%). Twenty-six patients (18.7%) were in the intensive care unit, 2 were treated with inotropic/vasoactive support, and none required extracorporeal membrane oxygenation or died. Median hospital stay was 2 days (range, 0-10; IQR, 2-3). All patients had elevated troponin I (n=111, 8.12 ng/mL; IQR, 3.50-15.90) or T (n=28, 0.61 ng/mL; IQR, 0.25-1.30); 69.8% had abnormal ECGs and arrhythmias (7 with nonsustained ventricular tachycardia); and 18.7% had left ventricular ejection fraction <55% on echocardiogram. Of 97 patients who underwent cardiac MRI at a median 5 days (range, 0-88; IQR, 3-17) from symptom onset, 75 (77.3%) had abnormal findings: 74 (76.3%) had late gadolinium enhancement, 54 (55.7%) had myocardial edema, and 49 (50.5%) met Lake Louise criteria. Among 26 patients with left ventricular ejection fraction <55% on echocardiogram, all with follow-up had normalized function (n=25). CONCLUSIONS: Most cases of suspected COVID-19 vaccine myocarditis occurring in persons <21 years have a mild clinical course with rapid resolution of symptoms. Abnormal findings on cardiac MRI were frequent. Future studies should evaluate risk factors, mechanisms, and long-term outcomes.


Assuntos
Vacinas contra COVID-19/efeitos adversos , COVID-19/prevenção & controle , Miocardite/diagnóstico por imagem , Miocardite/fisiopatologia , Adolescente , Criança , Eletrocardiografia/métodos , Feminino , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Miocardite/sangue , Miocardite/etiologia , Estudos Retrospectivos , Fatores de Tempo , Adulto Jovem
15.
World J Pediatr Congenit Heart Surg ; 12(2): 286-290, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33684014

RESUMO

Van Praagh (VP) A3 variant of truncus arteriosus (or common arterial trunk) is defined by only one pulmonary artery (usually the right) originating from the common trunk, while the other lung is supplied either by collaterals or a pulmonary artery arising from the aortic arch. This report describes a staged approach to manage a VP-A3 variant truncus arteriosus with ductal origin of the left pulmonary artery (LPA), a hypoplastic right pulmonary artery, and cyanosis. Initially, the ductal portion of the proximal LPA was stented with a Resolute Onyx drug-eluting stent. The pulmonary arteries grew and at four months of age had an acceptable McGoon ratio and Nakata index. The patient then underwent repair which included unifocalization of the branch pulmonary arteries, closure of the ventricular septal defect, and placement of a right ventricle-to-pulmonary artery homograft conduit.


Assuntos
Aorta Torácica/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Stents Farmacológicos , Artéria Pulmonar/cirurgia , Persistência do Tronco Arterial/cirurgia , Adulto , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Tomografia Computadorizada por Raios X , Persistência do Tronco Arterial/diagnóstico
17.
Pediatr Cardiol ; 40(7): 1439-1444, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31367952

RESUMO

National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13-20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.


Assuntos
Cardiologia/métodos , Conhecimentos, Atitudes e Prática em Saúde , Cardiopatias Congênitas/psicologia , Pediatria/métodos , Transição para Assistência do Adulto/normas , Adolescente , Estudos Transversais , Feminino , Cardiopatias Congênitas/terapia , Humanos , Masculino , Educação de Pacientes como Assunto , Inquéritos e Questionários
18.
J Matern Fetal Neonatal Med ; 31(7): 940-945, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28277918

RESUMO

Objective: We sought to examine the concordance of prenatally diagnosed congenital cardiac disease with postnatal echocardiography.Study design: Patients who underwent fetal echocardiograms performed by pediatric cardiologists at a single referral center from January to December 2014 were reviewed. Fetal echocardiography exams were performed in accordance with guidelines by the American Institute of Ultrasound in Medicine (AIUM) and the American Heart Association (AHA) guidelines (2013 and 2014, respectively). The concordance of prenatal diagnosis was compared to postnatal echocardiograms.Result: One hundred and six patients were included. Overall, the prenatal diagnosis precisely matched the postnatal diagnosis in 69.8%, minor discrepancies were seen in 14.2% and major differences were seen in 16% of cases. Three cases with major differences resulted in a more guarded postnatal prognosis; all three were fetuses with complex disease where the additional finding of total anomalous pulmonary venous return (TAPVR) was missed.Conclusion: Fetal echocardiograms performed in our specialized fetal cardiology program are in high agreement with postnatal diagnosis of congenital cardiac disease. A worse postnatal prognosis is uncommon. Emphasis on pulmonary venous imaging in complex congenital heart disease is vital.

19.
Am J Cardiol ; 114(11): 1676-81, 2014 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-25307199

RESUMO

Kawasaki disease (KD) is the leading cause of acquired coronary disease in children and may lead to subsequent myocardial ischemia and infarction. Because coronary computed tomographic angiography (CTA) is the most sensitive noninvasive test in patients with atherosclerosis, the aim of this study was to retrospectively evaluate coronary CTA performed in patients with KD for aneurysm, stenosis, and calcified and noncalcified coronary artery disease (CAD). Clinical histories and prior stress and imaging test results were reviewed. Thirty-two patients underwent coronary CTA for KD, and 385 coronary segments were evaluated. Twenty-three of 32 patients had ≥1 diseased coronary segment. There were 20 aneurysms, 7 lesions, and 75 segments (20%) with nonobstructive CAD (16% noncalcified, 2% calcified, and 2% mixed). All nonobstructive and obstructive CAD was in patients with histories of acute-phase coronary artery dilatation or aneurysm (echocardiographic z score 4 to 44), and were almost always associated with normal stress imaging test results on follow-up. No lesion or CAD was found in coronary computed tomographic angiographic studies performed in a control group referred for other indications (n = 32, 422 segments evaluated). The median coronary computed tomographic angiographic dose-length product was 59 mGy cm (interquartile range 32 to 131), the median unadjusted radiation dose was 0.8 mSv (interquartile range 0.4 to 1.8), and the median age- and size-adjusted radiation dose was 1.3 mSv (interquartile range 0.7 to 2.3). In conclusion, high-risk patients with histories of KD had nonobstructive and obstructive CAD not visualized by other noninvasive imaging tests. In properly selected high-risk patients with KD, coronary CTA may identify a subset at increased risk for future coronary pathology who may benefit from medical therapy.


Assuntos
Aneurisma Coronário/diagnóstico por imagem , Doença da Artéria Coronariana/diagnóstico por imagem , Estenose Coronária/diagnóstico por imagem , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Calcificação Vascular/diagnóstico por imagem , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos de Coortes , Aneurisma Coronário/etiologia , Angiografia Coronária , Doença da Artéria Coronariana/etiologia , Estenose Coronária/etiologia , Feminino , Humanos , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Calcificação Vascular/etiologia
20.
Artigo em Inglês | MEDLINE | ID: mdl-23561815

RESUMO

Unbalanced atrioventricular septal defect (uAVSD) is a challenging lesion with suboptimal outcomes in the current era. Severe forms of uAVSD mandate univentricular repair with well-documented outcomes. Determining the feasibility of biventricular repair (BVR) in patients with moderate forms of uAVSD is difficult. Ventricular hypoplasia has traditionally formed the cornerstone of defining uAVSD. However, malalignment of the atrioventricular junction and related derangements of the anatomy and physiology of the atrioventricular inflow play a central role in establishing and sustaining a biventricular end state. Atrioventricular valve index, left ventricular inflow index, and right ventricle/left ventricle inflow angle are important recently described measures of inflow physiology. Additional patient anatomic and physiologic factors that impact BVR feasibility undoubtedly exist. A recently launched Congenital Heart Surgeons Society prospective inception cohort study will address these and other issues that impair our ability to predict BVR feasibility in uAVSD.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/cirurgia , Valva Mitral/cirurgia , Valva Tricúspide/cirurgia , Procedimentos Cirúrgicos Cardíacos/mortalidade , Estudos de Coortes , Ecocardiografia Doppler , Estudos de Viabilidade , Feminino , Defeitos dos Septos Cardíacos/mortalidade , Humanos , Recém-Nascido , Masculino , Valva Mitral/diagnóstico por imagem , Prognóstico , Medição de Risco , Índice de Gravidade de Doença , Taxa de Sobrevida , Resultado do Tratamento , Valva Tricúspide/diagnóstico por imagem , Ultrassonografia Doppler em Cores/métodos
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