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1.
Pediatr Cardiol ; 45(1): 100-106, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37750969

RESUMO

Prior authorization is a process that health insurance companies use to determine if a patient's health insurance will cover certain medical treatments, procedures, or medications. Prior authorization requests are common in adult congenital and pediatric cardiology (ACPC) due to need for advanced diagnostics, complex procedures, disease-specific medications, and the heterogeneity of the ACPC population. Prior authorizations in ACPC are rarely denied, but nonetheless, they are often accompanied by significant administrative burden on clinical care teams and delays in patient care. Prior authorizations have been implicated in worsening care inequities. The prior authorization process is insurer specific with differences between commercial and public insurers. Prior authorization rejections were previously found to be more common for women, racial minorities, those with low education, and in low-income groups. Prior authorization unduly burdens routine diagnostics, routine interventional and surgical procedures, and routine cardiac specific medication use in the ACPC population. This manuscript highlights the burdens of prior authorization and advocates for the elimination of prior authorization for ACPC patients.


Assuntos
Cardiologia , Autorização Prévia , Adulto , Criança , Humanos , Feminino
2.
Pediatr Cardiol ; 2024 May 16.
Artigo em Inglês | MEDLINE | ID: mdl-38753034

RESUMO

Mental health conditions are a common comorbidity among children living with heart disease. Children with congenital heart disease are more likely to have a mental health condition than their unaffected peers or peers with other chronic illnesses, and mental health risk persists across their lifetime. While poorer mental health in adults with congenital heart disease is associated with worse overall health outcomes, the association between mental health and cardiac outcomes for children with heart disease remains unknown. Despite this, it is suspected that mental health conditions go undiagnosed in children with heart disease and that many affected children and adolescents do not receive optimal mental health care. In this article, we review mental health in congenital heart disease across the lifespan, across domains of care, and across diagnoses. Further directions to support mental health care for children and adolescents with heart disease include practical screening and access to timely referral and mental health resources.

3.
Am J Perinatol ; 40(4): 363-374, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-33940650

RESUMO

OBJECTIVES: The objective of this review was to assess the impact of maternal preeclampsia or hyperglycemia on the body composition and cardiovascular health in the offspring. STUDY DESIGN: We conducted a systematic review utilizing PubMed, EBSCO, CINAHLPlus, Cochrane Library, and Web of Science to include all studies assessing the impact of preeclampsia/eclampsia and/or gestational/pregestational diabetes mellitus on the health of the offspring (children <10 years of age). The health measures included anthropometry, cardiac dimensions and function, and vascular function. We performed a meta-analysis using Review Manager software and computed net risk ratio (RR) with 95% confidence interval (CI) for dichotomous data and mean difference (MD) with 95% CI for continuous data. RESULTS: There were 6,376 studies in total, of which 45 were included in the review and 40 in the meta-analysis. The results demonstrated higher birth weight (MD: 0.12 kg; 95% CI: 0.06-0.18) and systolic and diastolic blood pressure (BP; MD: 5.98 mm Hg; 95% CI: 5.64-6.32 and MD: 3.27 mm Hg; 95% CI: 0.65-5.89, respectively) in the offspring of mothers with gestational diabetes compared to controls. In contrast, the offspring of mothers with preeclampsia had lower birth weight (MD: -0.41 kg; 95% CI: -0.7 to -0.11); however, they had increased systolic (MD: 2.2 mm Hg; 95% CI: 1.28-3.12) and diastolic BP (MD: 1.41 mm Hg; 95% CI: 0.3-2.52) compared to controls. There is lack of data to conduct a meta-analysis of cardiac morphology, functional, and vascular imaging parameters. CONCLUSION: These findings suggest that the in-utero milieu can have a permanent impact on the body composition and vascular health of the offspring. Future work warrants multicenter prospective studies to understand the mechanism and the actual effect of exposure to maternal hyperglycemia and high BP on the cardiovascular health of the offspring and long-term outcomes. KEY POINTS: · Adverse in-utero exposures may have an impact on cardiovascular risk in children.. · Maternal hyperglycemia/preeclampsia lead to changes in birthweight and BP.. · Limited echocardiographic and vascular imaging data in these cohorts necessitates future work..


Assuntos
Diabetes Gestacional , Hiperglicemia , Pré-Eclâmpsia , Gravidez , Criança , Feminino , Humanos , Pré-Eclâmpsia/epidemiologia , Peso ao Nascer , Estudos Prospectivos , Pressão Sanguínea/fisiologia , Diabetes Gestacional/epidemiologia , Hiperglicemia/complicações , Estudos Multicêntricos como Assunto
4.
Cardiol Young ; 33(8): 1277-1287, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37615116

RESUMO

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiologia , Cardiopatias , Adulto , Criança , Humanos
5.
J Pediatr ; 243: 208-213.e3, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-34952008

RESUMO

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.


Assuntos
COVID-19 , Miocardite , Adolescente , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Humanos , Miocardite/epidemiologia , Miocardite/etiologia , RNA Mensageiro
6.
BMC Cardiovasc Disord ; 22(1): 109, 2022 03 17.
Artigo em Inglês | MEDLINE | ID: mdl-35300601

RESUMO

BACKGROUND: Familial hypercholesterolemia (FH) due to a founder variant in Apolipoprotein B (ApoBR3500Q) is reported in 12% of the Pennsylvania Amish community. By studying a cohort of ApoBR3500Q heterozygotes and homozygotes, we aimed to characterize the biochemical and cardiac imaging features in children and young adults with a common genetic background and similar lifestyle. METHODS: We employed advanced lipid profile testing, carotid intima media thickness (CIMT), pulse wave velocity (PWV), and peripheral artery tonometry (PAT) to assess atherosclerosis in a cohort of Amish ApoBR3500Q heterozygotes (n = 13), homozygotes (n = 3), and their unaffected, age-matched siblings (n = 9). ApoBR3500Q homozygotes were not included in statistical comparisons. RESULTS: LDL cholesterol (LDL-C) was significantly elevated among ApoBR3500Q heterozygotes compared to sibling controls, though several ApoBR3500Q heterozygotes had LDL-C levels in the normal range. LDL particles (LDL-P), small, dense LDL particles, and ApoB were also significantly elevated among subjects with ApoBR3500Q. Despite these differences in serum lipids and particles, CIMT and PWV were not significantly different between ApoBR3500Q heterozygotes and controls in age-adjusted analysis. CONCLUSIONS: We provide a detailed description of the serum lipids, atherosclerotic plaque burden, vascular stiffness, and endothelial function among children and young adults with FH due to heterozygous ApoBR3500Q. Fasting LDL-C was lower than what is seen with other forms of FH, and even normal in several ApoBR3500Q heterozygotes, emphasizing the importance of cascade genetic testing among related individuals for diagnosis. We found increased number of LDL particles among ApoBR3500Q heterozygotes but an absence of detectable atherosclerosis.


Assuntos
Aterosclerose , Hiperlipoproteinemia Tipo II , Amish/genética , Apolipoproteínas B/genética , Espessura Intima-Media Carotídea , Criança , LDL-Colesterol , Humanos , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/genética , Mutação , Análise de Onda de Pulso , Receptores de LDL/genética , Adulto Jovem
7.
J Pediatr ; 238: 26-32.e1, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34339728

RESUMO

OBJECTIVES: To characterize the clinical course and outcomes of children 12-18 years of age who developed probable myopericarditis after vaccination with the Pfizer-BioNTech (BNT162b2) coronavirus disease 2019 (COVID-19) messenger RNA (mRNA) vaccine. STUDY DESIGN: A cross-sectional study of 25 children, aged 12-18 years, diagnosed with probable myopericarditis after COVID-19 mRNA vaccination as per the Centers for Disease Control and Prevention criteria for myopericarditis at 8 US centers between May 10, 2021, and June 20, 2021. We retrospectively collected the following data: demographics, severe acute respiratory syndrome coronavirus 2 virus detection or serologic testing, clinical manifestations, laboratory test results, imaging study results, treatment, and time to resolutions of symptoms. RESULTS: Most (88%) cases followed the second dose of vaccine, and chest pain (100%) was the most common presenting symptom. Patients came to medical attention a median of 2 days (range, <1-20 days) after receipt of Pfizer mRNA COVID-19 vaccination. All adolescents had an elevated plasma troponin concentration. Echocardiographic abnormalities were infrequent, and 92% showed normal cardiac function at presentation. However, cardiac magnetic resonance imaging, obtained in 16 patients (64%), revealed that 15 (94%) had late gadolinium enhancement consistent with myopericarditis. Most were treated with ibuprofen or an equivalent nonsteroidal anti-inflammatory drug for symptomatic relief. One patient was given a corticosteroid orally after the initial administration of ibuprofen or an nonsteroidal anti-inflammatory drug; 2 patients also received intravenous immune globulin. Symptom resolution was observed within 7 days in all patients. CONCLUSIONS: Our data suggest that symptoms owing to myopericarditis after the mRNA COVID-19 vaccination tend to be mild and transient. Approximately two-thirds of patients underwent cardiac magnetic resonance imaging, which revealed evidence of myocardial inflammation despite a lack of echocardiographic abnormalities.


Assuntos
Vacinas contra COVID-19/genética , COVID-19/prevenção & controle , Imagem Cinética por Ressonância Magnética/métodos , Miocardite/etiologia , SARS-CoV-2/imunologia , Vacinação/efeitos adversos , Vacinas Sintéticas/efeitos adversos , Adolescente , COVID-19/epidemiologia , COVID-19/genética , Vacinas contra COVID-19/efeitos adversos , Criança , Estudos Transversais , Feminino , Humanos , Incidência , Masculino , Miocardite/diagnóstico , Miocardite/epidemiologia , Pandemias , Estudos Retrospectivos , Estados Unidos/epidemiologia , Vacinas de mRNA
8.
Pediatr Cardiol ; 42(5): 1190-1199, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33856499

RESUMO

Overweight/obesity, prevalent cardiovascular risk factors in children, can be associated with increased risk of adverse outcomes in children with heart disease. The American College of Cardiology (ACC) developed quality metrics including a BMI metric related to identifying and counseling overweight and obese children presenting to cardiology clinics. This metric was used for a multicenter collaborative learning Quality improvement (QI) Project through the ACC Quality Network (QNet). Our aim was to increase the percentage of children between ages 3 and 18 years presenting to cardiology clinics at participating centers with BMI > 85th percentile who received appropriate counseling. Participating centers submitted data quarterly to QNet for a sample of patients who received counseling. A Key Driver Diagram was created to help teams drive improvement. Individual centers customized interventions and participated in network-wide educational learning sessions about QI and shared experience. Statistical process control charts were used. From 04/01/2017 to 09/30/2019, 27,511 patient visits were included. Among 32 participating centers, overall counseling rate was 54%. The BMI counseling rate increased from 25% in 2017Q2 to 54% in 2019Q3. There was a wide variation from 10 to 100% in the performance of individual centers. The overall rate of identification and counseling of overweight and obese children presenting to ambulatory cardiology clinics in participating centers is low. There is wide variation in the performance of centers, providing an opportunity for improvement. Using this multicenter learning approach, individual centers have demonstrated improvement. This demonstrates that collaborative learning approaches in QI can increase implementation of the metric.


Assuntos
Índice de Massa Corporal , Aconselhamento/métodos , Obesidade/terapia , Adolescente , Cardiologia/normas , Criança , Pré-Escolar , Aconselhamento/estatística & dados numéricos , Humanos , Melhoria de Qualidade , Estados Unidos
9.
Pediatr Cardiol ; 42(2): 234-254, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33388850

RESUMO

In the very young child (less than eight years of age), transient loss of consciousness represents a diagnostic and management dilemma for clinicians. While most commonly benign, syncope may be due to cardiac dysfunction which can be life-threatening. It can be secondary to an underlying ion channelopathy, cardiac inflammation, cardiac ischemia, congenital heart disease, cardiomyopathy, or pulmonary hypertension. Patients with genetic disorders require careful evaluation for a cardiac cause of syncope. Among the noncardiac causes, vasovagal syncope is the most common etiology. Breath-holding spells are commonly seen in this age group. Other causes of transient loss of consciousness include seizures, neurovascular pathology, head trauma, psychogenic pseudosyncope, and factitious disorder imposed on another and other forms of child abuse. A detailed social, present, past medical, and family medical history is important when evaluating loss of consciousness in the very young. Concerning characteristics of syncope include lack of prodromal symptoms, no preceding postural changes or occurring in a supine position, after exertion or a loud noise. A family history of sudden unexplained death, ion channelopathy, cardiomyopathy, or congenital deafness merits further evaluation. Due to inherent challenges in diagnosis at this age, often there is a lower threshold for referral to a specialist.


Assuntos
Síncope/diagnóstico , Síncope/etiologia , Arritmias Cardíacas/complicações , Cardiomiopatias/complicações , Criança , Pré-Escolar , Diagnóstico Diferencial , Cardiopatias Congênitas/complicações , Humanos , Hipertensão Pulmonar/complicações , Masculino , Convulsões/complicações , Síncope Vasovagal/complicações , Inconsciência/diagnóstico , Inconsciência/etiologia
10.
Pediatr Cardiol ; 42(1): 19-35, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33015722

RESUMO

Coronavirus disease 2019 (COVID-19) has affected patients across all age groups, with a wide range of illness severity from asymptomatic carriers to severe multi-organ dysfunction and death. Although early reports have shown that younger age groups experience less severe disease than older adults, our understanding of this phenomenon is in continuous evolution. Recently, a severe multisystem inflammatory syndrome in children (MIS-C), with active or recent COVID-19 infection, has been increasingly reported. Children with MIS-C may demonstrate signs and symptoms of Kawasaki disease, but also have some distinct differences. These children have more frequent and severe gastrointestinal symptoms and are more likely to present with a shock-like presentation. Moreover, they often present with cardiovascular involvement including myocardial dysfunction, valvulitis, and coronary artery dilation or aneurysms. Here, we present a review of the literature and summary of our current understanding of cardiovascular involvement in children with COVID-19 or MIS-C and identifying the role of a pediatric cardiologist in caring for these patients.


Assuntos
COVID-19/terapia , Cardiologistas , Pandemias , Pediatria , Papel do Médico , Síndrome de Resposta Inflamatória Sistêmica/terapia , Idoso , COVID-19/diagnóstico , Criança , Feminino , Humanos , Masculino , Medicina , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/fisiopatologia , SARS-CoV-2/patogenicidade , Índice de Gravidade de Doença , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico
11.
Mol Genet Metab ; 131(3): 316-324, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-33127324

RESUMO

Propionic acidemia (PA) is caused by inherited deficiency of mitochondrial propionyl-CoA carboxylase (PCC) and results in significant neurodevelopmental and cardiac morbidity. However, relationships among therapeutic intervention, biochemical markers, and disease progression are poorly understood. Sixteen individuals homozygous for PCCB c.1606A > G (p.Asn536Asp) variant PA participated in a two-week suspension of therapy. Standard metabolic markers (plasma amino acids, blood spot methylcitrate, plasma/urine acylcarnitines, urine organic acids) were obtained before and after stopping treatment. These same markers were obtained in sixteen unaffected siblings. Echocardiography and electrocardiography were obtained from all subjects. We characterized the baseline biochemical phenotype of untreated PCCB c.1606A > G homozygotes and impact of treatment on PCC deficiency biomarkers. Therapeutic regimens varied widely. Suspension of therapy did not significantly alter branched chain amino acid levels, their alpha-ketoacid derivatives, or urine ketones. Carnitine supplementation significantly increased urine propionylcarnitine and its ratio to total carnitine. Methylcitrate blood spot and urine levels did not correlate with other biochemical measures or cardiac outcomes. Treatment of PCCB c.1606A > G homozygotes with protein restriction, prescription formula, and/or various dietary supplements has a limited effect on core biomarkers of PCC deficiency. These patients require further longitudinal study with standardized approaches to better understand the relationship between biomarkers and disease burden.


Assuntos
Carbono-Carbono Ligases/genética , Coração/fisiopatologia , Transtornos do Neurodesenvolvimento/genética , Acidemia Propiônica/genética , Ácidos/sangue , Ácidos/urina , Adolescente , Adulto , Aminoácidos/sangue , Aminoácidos/urina , Biomarcadores/sangue , Biomarcadores/urina , Carbono-Carbono Ligases/sangue , Carbono-Carbono Ligases/urina , Carnitina/sangue , Carnitina/urina , Criança , Pré-Escolar , Ecocardiografia , Feminino , Coração/diagnóstico por imagem , Humanos , Masculino , Mitocôndrias/genética , Mitocôndrias/metabolismo , Mutação/genética , Transtornos do Neurodesenvolvimento/sangue , Transtornos do Neurodesenvolvimento/diagnóstico por imagem , Transtornos do Neurodesenvolvimento/urina , Compostos Orgânicos/sangue , Compostos Orgânicos/urina , Fenótipo , Acidemia Propiônica/sangue , Acidemia Propiônica/diagnóstico por imagem , Acidemia Propiônica/urina , Adulto Jovem
12.
Pediatr Cardiol ; 41(6): 1081-1091, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32656626

RESUMO

Due to the COVID-19 pandemic, there has been an increased interest in telehealth as a means of providing care for children by a pediatric cardiologist. In this article, we provide an overview of telehealth utilization as an extension of current pediatric cardiology practices and provide some insight into the rapid shift made to quickly implement these telehealth services into our everyday practices due to COVID-19 personal distancing requirements. Our panel will review helpful tips into the selection of appropriate patient populations and specific cardiac diagnoses for telehealth that put patient and family safety concerns first. Numerous practical considerations in conducting a telehealth visit must be taken into account to ensure optimal use of this technology. The use of adapted staffing and billing models and expanded means of remote monitoring will aid in the incorporation of telehealth into more widespread pediatric cardiology practice. Future directions to sustain this platform include the refinement of telehealth care strategies, defining best practices, including telehealth in the fellowship curriculum and continuing advocacy for technology.


Assuntos
Cardiologia , Infecções por Coronavirus , Cardiopatias/terapia , Monitorização Fisiológica , Pandemias , Pediatria , Pneumonia Viral , Consulta Remota , Telemedicina , Betacoronavirus , COVID-19 , Cardiologia/educação , Cardiologia/tendências , Criança , Currículo , Previsões , Cardiopatias/diagnóstico , Humanos , Monitorização Fisiológica/métodos , Monitorização Fisiológica/tendências , Pediatria/educação , Pediatria/tendências , Consulta Remota/métodos , Consulta Remota/tendências , SARS-CoV-2 , Telemedicina/métodos , Telemedicina/tendências
13.
Cardiol Young ; 29(4): 467-474, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30940265

RESUMO

OBJECTIVE: The objective of this study was to assess differences in myocardial systolic and diastolic function and vascular function in children 2-5 years of age born to diabetic as compared to non-diabetic mothers. METHODS: This study was a retrospective cohort conducted in 2016 at The Aga Khan University Hospital, Karachi, Pakistan. It included children between 2 and 5 years of age born to mothers with and without exposure to diabetes in utero (n = 68 in each group) and who were appropriate for gestational age. Myocardial morphology and function using echocardiogram and carotid intima media thickness (cIMT) and pulse wave velocity was performed to evaluate cardiac function as well as macrovascular remodelling in these children. Multiple linear regression was used to compare the groups. RESULTS: There was no significant difference in cardiac morphology, myocardial systolic and diastolic function, and macrovascular assessment between the exposed and unexposed groups of AGA children. Subgroup analysis demonstrated a significantly decreased mitral E/A ratio in children whose mothers were on medications as compared to those on dietary control (median [IQR] = 1.7 [1.6-1.9] and 1.56 [1.4-1.7], respectively, p = 0.02), and a higher cIMT in children whose mothers were on medication as compared to controls (0.48 [0.44-0.52] and 0.46 [0.44-0.50], respectively, p = 0.03). CONCLUSION: In utero exposure to uncontrolled maternal diabetes has an effect on the cardiovascular structure and function in children aged 2-5 years. However, future work requires long-term follow-up from fetal to adult life to assess these changes over the life course.


Assuntos
Doenças Cardiovasculares/diagnóstico por imagem , Doenças Cardiovasculares/etiologia , Hiperglicemia/complicações , Gravidez em Diabéticas , Efeitos Tardios da Exposição Pré-Natal , Remodelação Vascular , Adulto , Sistema Cardiovascular/patologia , Espessura Intima-Media Carotídea , Pré-Escolar , Diástole , Ecocardiografia , Feminino , Humanos , Modelos Lineares , Masculino , Mães , Paquistão , Gravidez , Análise de Onda de Pulso , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Sístole , Adulto Jovem
14.
J Pediatr ; 191: 145-151, 2017 12.
Artigo em Inglês | MEDLINE | ID: mdl-29173298

RESUMO

OBJECTIVE: To evaluate clinical outcome of patients with Ellis-van Creveld syndrome (EVC) in whom congenital heart disease (CHD) repair was delayed intentionally to reduce the risk of postoperative respiratory morbidity and mortality. STUDY DESIGN: This retrospective review of 51 EVC c.1886+5G>T homozygotes born between 2005 and 2014 focused on 18 subjects who underwent surgery for CHD, subdivided into early (mean, 1.3 months) vs delayed (mean, 50.1 months) repair. RESULTS: Growth trajectories differed between control subjects and patients with EVC, and CHD was associated with slower weight gain. Relative to controls, infants with EVC had a 40%-75% higher respiratory rates (independent of CHD) accompanied by signs of compensated respiratory acidosis. Blood gases and respiratory rates approached normal values by age 4 years. Hemodynamically significant CHD was present in 23 children, 18 (78%) of whom underwent surgical repair. Surgery was performed at 1.3 ± 1.3 months for children born between 2005 and 2009 (n = 9) and 50.1 ± 40.2 months (P = .009) for children born between 2010 and 2014 (n = 9). The latter had shorter postoperative mechanical ventilation (1.1 ± 2.4 days vs 49.6 ± 57.1 days; P = .075), shorter intensive care duration of stay (16 ± 24 days vs 48.6 ± 44.2 days; P = .155), and no postoperative tracheostomies (vs 60%; P = .028) or deaths (vs 44%; P = .082). CONCLUSION: Among children with EVC and possibly other short-rib thoracic dysplasias, delayed surgical repair of CHD reduces postoperative morbidity and improves survival. Respiratory rate serves as a simple indicator for optimal timing of surgical repair.


Assuntos
Síndrome de Ellis-Van Creveld , Cardiopatias Congênitas/cirurgia , Pré-Escolar , Síndrome de Ellis-Van Creveld/mortalidade , Síndrome de Ellis-Van Creveld/fisiopatologia , Feminino , Seguimentos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/prevenção & controle , Taxa Respiratória , Estudos Retrospectivos , Toracotomia , Fatores de Tempo , Resultado do Tratamento , Aumento de Peso
15.
Cardiol Young ; 27(10): 2003-2005, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29286264

RESUMO

The Seventh World Congress of Pediatric Cardiology was held in Barcelona in July, 2017. The central philosophy of the congress was "bridging together" all major specialties in the field. This article summarises the highlights of the meeting as it relates to ambulatory paediatric cardiology. There is a now a more unified approach to children with CHD, including assessment of neuro-developmental outcomes. The new World Heart Foundation criteria for the diagnosis of rheumatic fever remain controversial.


Assuntos
Procedimentos Cirúrgicos Ambulatórios , Cardiopatias Congênitas/cirurgia , Pediatria , Febre Reumática/diagnóstico , Congressos como Assunto , Humanos , Sociedades Médicas
16.
J Am Heart Assoc ; 13(13): e032415, 2024 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-38934870

RESUMO

While significant progress has been made in reducing disparities within the US health care system, notable gaps remain. This article explores existing disparities within pediatric congenital heart disease care. Congenital heart disease, the most common birth defect and a leading cause of infant death, has garnered substantial attention, revealing certain disparities within the US health care system. Factors such as race, ethnicity, insurance coverage, socioeconomic status, and geographic location are all commonalities that significantly affect health disparities in pediatric congenital heart disease. This comprehensive review sheds light on disparities from diverse perspectives in pediatric care, demonstrates the inequities and inequalities leading to these disparities, presents effective solutions, and issues a call to action for providers, institutions, and the health care system. Recognizing and addressing these disparities is imperative for ensuring equitable care and enhancing the long-term well-being of children affected by congenital heart disease. Implementing robust, evidence-based frameworks that promote responsible and safe interventions is fundamental to enduring change.


Assuntos
Disparidades em Assistência à Saúde , Cardiopatias Congênitas , Humanos , Cardiopatias Congênitas/terapia , Cardiopatias Congênitas/epidemiologia , Disparidades em Assistência à Saúde/etnologia , Criança , Acessibilidade aos Serviços de Saúde/organização & administração , Estados Unidos/epidemiologia , Lactente , Recém-Nascido , Fatores Socioeconômicos , Pré-Escolar
17.
J Am Heart Assoc ; 13(9): e032837, 2024 05 07.
Artigo em Inglês | MEDLINE | ID: mdl-38639355

RESUMO

Evidence from medicine and other fields has shown that gender diversity results in better decision making and outcomes. The incoming workforce of congenital heart specialists (especially in pediatric cardiology) appears to be more gender balanced, but past studies have shown many inequities. Gender-associated differences in leadership positions, opportunities presented for academic advancement, and recognition for academic contributions to the field persist. In addition, compensation packages remain disparate if evaluated based on gender with equivalent experience and expertise. This review explores these inequities and has suggested individual and institutional changes that could be made to recruit and retain women, monitor the climate of the institution, and identify and eliminate bias in areas like salary and promotions.


Assuntos
Equidade de Gênero , Cardiopatias Congênitas , Médicas , Humanos , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/terapia , Feminino , Médicas/estatística & dados numéricos , Médicas/tendências , Masculino , Liderança , Cardiologia/tendências , Pediatria/tendências , Salários e Benefícios , Sexismo/tendências , Fatores Sexuais , Cardiologistas/tendências
18.
Pediatr Cardiol ; 34(5): 1237-43, 2013 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23377382

RESUMO

The development of echocardiographic ventricular wall motion abnormalities and ST segment changes with exercise may enhance the detection of myocardial ischemia in children with aortic valve stenosis (AS). This study aimed to assess the relationship between the exercise wall motion index (WMIe), ST segment depression (STd), and overall functionality in asymptomatic children with isolated AS. A prospective interpretation of collected stress echocardiographic images was performed. The 98 children who met the inclusion criteria had a mean age of 12.8 years and a male/female ratio of 4/1. Group 1 (mild AS) was composed of 70 children, and group 2 (moderate or severe AS) was composed of 28 children. Abnormal WMIe was seen in 8 patients (5 in group 1 and 3 in group 2), and significant STd was observed in 13 children (3 in group 1 and 10 in group 2). Four (50 %) of the eight patients with abnormal WMIe also had significant STd. Severity of stenosis was associated with STd (odds ratio [OR], 12.0; 95 % CI 3.0-49.0), logistic regression). A significant association also existed between abnormal WMIe and STd (OR, 9.0; 95 % CI 1.9-42.0, logistic regression). Exercise duration was significantly shorter in group 2 (12 ± 4.52 min) than in group 1 (13 ± 5.28 min) (p = 0.02, analysis of covariance). The appearance of wall motion abnormalities and STd during exercise may be helpful in detecting inducible, functionally important myocardial ischemia in asymptomatic children with AS. Stress echocardiography may be a useful adjunct to more traditional exercise testing in risk stratifying asymptomatic children with AS.


Assuntos
Estenose da Valva Aórtica/diagnóstico por imagem , Ecocardiografia sob Estresse , Isquemia Miocárdica/diagnóstico por imagem , Adolescente , Estenose da Valva Aórtica/fisiopatologia , Criança , Teste de Esforço , Feminino , Humanos , Masculino , Isquemia Miocárdica/fisiopatologia , Estudos Prospectivos , Índice de Gravidade de Doença
19.
Interv Cardiol (Lond) ; 15(Suppl 17): 433-437, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37593691

RESUMO

Familial Hypercholesterolemia (FH) is an autosomal dominant genetic disorder that causes increased low density lipoprotein cholesterol (LDL-C) levels and a higher risk of premature atherosclerosis and cardiovascular disease (CVD). Common causes of FH include inherited genetic mutations in the LDLR, APOB, and PCSK9 genes. LDLR, APOB, and PCSK9 mutations account for 79%, 5%, and <1% of cases of FH respectively. Apolipoprotein B (ApoB) is the necessary atherogenic lipoprotein which can serve as a determinant of cardiovascular disease including hypercholesterolemia. A founder variant in Apolipoprotein B (APOB p.R3527Q) causes FH and is found in 12% of the Pennsylvania Amish population. This article provides an overview of ApoB metabolism and clinical manifestations associated with APOB mutations. An understanding of the clinical manifestations caused by APOB p.R3527Q can be beneficial for the clinical diagnosis and treatment of FH in the Amish. Based on previous studies, changes in LDL cholesterol (LDL-C), LDL particles (LDL-P), small dense LDL particles, and ApoB levels can be seen among these patients putting them at an increased risk for atherosclerotic issues, vascular hardening, and changes in endothelial function, particularly among homozygous individuals.

20.
Arch Dis Child ; 108(4): 258-263, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36288921

RESUMO

BACKGROUND: Children born small for gestational age (SGA) may experience more long-term neurodevelopmental issues than those born appropriate for gestational age (AGA). This study aimed to assess differences in the neurodevelopment of children born SGA or AGA within a periurban community in Pakistan. METHODS: This was a prospective cohort study in which study participants were followed from the pilot Doppler cohort study conducted in 2018. This pilot study aimed to develop a pregnancy risk stratification model using machine learning on fetal Dopplers. This project identified 119 newborns who were born SGA (2.4±0.4 kg) based on International Fetal and Newborn Growth Consortium standards. We assessed 180 children (90 SGA and 90 AGA) between 2 and 4 years of age (76% of follow-up rate) using the Malawi Developmental Assessment Tool (MDAT). FINDINGS: Multivariable linear regression analysis comparing the absolute scores of MDAT showed significantly lower fine motor scores (ß: -0.98; 95% CI -1.90 to -0.06) among SGAs, whereas comparing the z-scores using multivariable logistic regression, SGA children had three times higher odds of overall z-scores ≤-2 (OR: 3.78; 95% CI 1.20 to 11.89) as compared with AGA children. INTERPRETATION: SGA exposure is associated with poor performance on overall MDAT, mainly due to changes in the fine motor domain in young children. The scores on the other domains (gross motor, language and social) were also lower among SGAs; however, none of these reached statistical significance. There is a need to design follow-up studies to assess the impact of SGA on child's neurodevelopmental trajectory and school performance.


Assuntos
Retardo do Crescimento Fetal , Recém-Nascido Pequeno para a Idade Gestacional , Gravidez , Feminino , Criança , Humanos , Recém-Nascido , Pré-Escolar , Idade Gestacional , Estudos de Coortes , Estudos Prospectivos , Paquistão/epidemiologia , Projetos Piloto
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