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1.
Am J Perinatol ; 2022 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-36580978

RESUMO

OBJECTIVE: Our objective was to gauge adherence to nationally endorsed protocols in implementation of pulse oximetry (POx) screening for critical congenital heart disease (CCHD) in infants after mandate by all states and to assess associated characteristics. STUDY DESIGN: Between March and October 2019, an online questionnaire was administered to nurse supervisors who oversee personnel conducting POx screening. The questionnaire used eight questions regarding performance and interpretation of screening protocols to measure policy consistency, which is adherence to nationally endorsed protocols for POx screening developed by professional medical societies. Multilevel linear regression models evaluated associations between policy consistency and characteristics of hospitals and individuals, state of hospital location, early versus late mandate adopters, and state reporting requirements. RESULTS: Responses from 189 nurse supervisors spanning 38 states were analyzed. Only 17% received maximum points indicating full policy consistency, and 24% selected all four options for potential hypoxia that require a repeat screen. Notably, 33% did not recognize ≤90% SpO2 as an immediate failed screen and 31% responded that an infant with SpO2 of 89% in one extremity will be rescreened by nurses in an hour rather than receiving an immediate physician referral. Lower policy consistency was associated with lack of state reporting mandates (beta = -1.23 p = 0.01) and early adoption by states (beta = -1.01, p < 0.01). CONCLUSION: When presented with SpO2 screening values on a questionnaire, a low percentage of nurse supervisors selected responses that demonstrated adherence to nationally endorsed protocols for CCHD screening. Most notably, almost one-third of respondents did not recognize ≤90% SpO2 as a failed screen that requires immediate physician follow-up. In addition, states without reporting mandates and early adopter states were associated with low policy consistency. Implementing state reporting requirements might increase policy consistency, but some inconsistency may be the result of unique protocols in early adopter states that differ from nationally endorsed protocols. KEY POINTS: · Low adherence to nationally endorsed protocols.. · Inconsistent physician follow-up to hypoxia.. · Reporting improved consistency with national policy..

2.
Pediatr Cardiol ; 43(7): 1409-1428, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35460366

RESUMO

Ebstein's anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve abnormalities. Ebstein's anomaly affects the development of the tricuspid valve with widely varying morphology and, therefore, clinical presentation. Associated congenital cardiac lesions tend to be found more often in younger patients and may even be the reason for presentation. Presentation can vary from the most extreme form in fetal life, to asymptomatic diagnosis late in adult life. The most symptomatic patients need intensive care support in the neonatal period. This article summarizes and analyzes the literature on Ebstein's anomaly and provides a framework for the investigation, management, and follow-up of these patients, whether they present via fetal detection or late in adult life. For each age group, the clinical presentation, required diagnostic investigations, natural history, and management are described. The surgical options available for patients with Ebstein's anomaly are detailed and analyzed, starting from the initial mono-leaflet repairs to the most recent cone repair and its modifications. The review also assesses the effects of pregnancy on the Ebstein's circulation, and vice versa, the effects of Ebstein's on pregnancy outcomes. Finally, two attached appendices are provided for a structured echocardiogram protocol and key information useful for comprehensive Multi-Disciplinary Team discussion.


Assuntos
Anomalia de Ebstein , Cardiopatias Congênitas , Adulto , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/cirurgia , Ecocardiografia , Feto , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/terapia , Humanos , Recém-Nascido , Assistência ao Paciente , Valva Tricúspide/diagnóstico por imagem
3.
AJP Rep ; 10(4): e390-e394, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33214934

RESUMO

We present a case of a neonate born with kaposiform hemangioendothelioma (KHE), complicated by Kasabach-Merritt phenomenon (KMP) and other serious conditions, who was successfully treated with sirolimus. In addition to complications from thrombocytopenia and fluid overload, during the course of therapy, our patient experienced supratherapeutic drug levels at the commonly accepted starting dose of sirolimus. Patients with KHE and KMP should be closely monitored for potential complications of both the initial disease and unexpected side effects of treatments.

4.
J Pediatr Gastroenterol Nutr ; 68(6): e94-e98, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31124990

RESUMO

OBJECTIVES: The aim of this study was to document the process of achieving full enteral feeding in infants with omphalocele and to identify factors that affect feeding success. METHODS: After institutional review board approval (study no. 5100169), 123 infants with omphalocele, born between 1993 and 2011 were reviewed. Mortalities were excluded. All survivors had complete follow-up. Variables suspected to impact enteral feeding in infants with non-giant versus giant omphalocele were compared. Independent t test, Mann-Whitney, and χ test were used. Regression evaluated for variable independence. RESULTS: Of 123 infants with omphalocele, 97 (79%) survived, 62/97 (64%) had non-giant, and 35/97 (36%) giant omphalocele. For survivors, the mean gestational age was 37 ±â€Š4 weeks with median follow-up of 4.4 years (range: 1.4-7.4 years). The median time to full feeds was 4 days (range: 0-85 days) for non-giant versus 8 days (range: 1-96 days) for giant, a significant difference (P < 0.01). Breast milk significantly decreased time to full feeds independent of omphalocele size. Giant omphalocele infants had a significantly higher incidence of respiratory insufficiency at birth (P < 0.01) and sac rupture (P = 0.02), but fewer chromosomal anomalies (P = 0.04). Respiratory insufficiency at birth (P < 0.01) and gastroesophageal reflux disease (P < 0.01) independently delayed feeding in omphalocele infants. CONCLUSIONS: Infants with non-giant omphalocele can achieve full enteral feeds within the first week of life, but giant omphalocele infants require significantly more time. Breast milk independently promotes feeding success whereas gastroesophageal reflux disease and respiratory insufficiency at birth independently delay feeding in infants with omphalocele.


Assuntos
Nutrição Enteral/métodos , Refluxo Gastroesofágico/terapia , Hérnia Umbilical/terapia , Leite Humano , Insuficiência Respiratória/terapia , Criança , Pré-Escolar , Feminino , Seguimentos , Refluxo Gastroesofágico/etiologia , Idade Gestacional , Hérnia Umbilical/complicações , Humanos , Lactente , Masculino , Análise de Regressão , Insuficiência Respiratória/etiologia , Fatores de Tempo , Resultado do Tratamento
5.
Pediatr Cardiol ; 39(6): 1165-1171, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29728721

RESUMO

The placenta is a complex organ that influences prenatal growth and development, and through fetal programming impacts postnatal health and well-being lifelong. Little information exists on placental pathology in the presence of congenital heart disease (CHD). Our objective is to characterize the placenta in CHD and investigate for distinctions based on type of malformation present. Placental pathology from singleton neonates prenatally diagnosed and delivered at > 37 weeks gestation was analyzed. Placental findings of absolute weight, placental weight-to-newborn birth weight ratio, chorangiosis, villus maturity, thrombosis, and infarction were recorded and analyzed based on four physiological categories of CHD: (1) single ventricle-aortic obstruction, (2) single ventricle-pulmonic obstruction, (3) two-ventricle anomalies, and (4) transposition of the great arteries (TGA). Associations between fetal Doppler assessments of middle cerebral/umbilical arterial flow and placental findings were investigated. A total of 120 cases of complex CHD were analyzed. Overall placental-to-birth weight ratios were < 10th percentile for 77% and < 3rd percentile for 49% with abnormalities of chorangiosis (18%), hypomature villi (15%), thrombosis (41%), and infarction (17%) common. There was no association between fetal Doppler flow measures and placental abnormalities. Newborns with TGA had the greatest degree of placental abnormality. Placentas of newborns with CHD are smaller than expected and manifest a number of vascular abnormalities, with TGA most prominent. Fetal Doppler does not correlate with these abnormalities. Studies investigating the relationship between placental abnormalities and postnatal outcomes may offer insight into the fetal origins of outcome variability in CHD.


Assuntos
Cardiopatias Congênitas/complicações , Doenças Placentárias/patologia , Placenta/patologia , Estudos de Coortes , Feminino , Desenvolvimento Fetal , Feto , Idade Gestacional , Cardiopatias Congênitas/patologia , Humanos , Recém-Nascido , Doenças Placentárias/etiologia , Gravidez , Ultrassonografia Pré-Natal/métodos
7.
Neonatology ; 112(3): 281-286, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28704835

RESUMO

BACKGROUND: Omphalocele is one of the most common abdominal wall defects. Many newborn infants born with omphalocele present with significant respiratory distress at birth, requiring mechanical ventilatory support, and have clinical evidence of pulmonary hypertension. Little information exists on the prevalence of and risk factors associated with pulmonary hypertension in this cohort of infants. OBJECTIVES: To describe the prevalence of and risk factors associated with pulmonary hypertension among infants with omphalocele. METHODS: This is a multicenter retrospective chart review of demographic data and clinical characteristics of infants with omphalocele admitted to the neonatal intensive care units of Loma Linda University Children's Hospital and Children's Mercy Hospital between 1994 and 2011. Echocardiogram images were reviewed for pulmonary hypertension, and statistical analyses were performed to identify risk factors associated with the presence of pulmonary hypertension. RESULTS: Pulmonary hypertension was diagnosed in 32/56 (57%) infants with omphalocele. Compared to infants without pulmonary hypertension, infants with pulmonary hypertension were more likely to have a liver-containing defect (16/32 [50%] vs. 5/24 [21%], p = 0.03), require intubation at birth (18/32 [56%] vs. 6/24 [17%], p = 0.03), and die during initial hospitalization (12/32 [38%] vs. 2/24 [8%], p = 0.01). CONCLUSION: The majority of infants with omphalocele have evidence of pulmonary hypertension which is associated with increased mortality. Echocardiograms to screen for pulmonary hypertension should be obtained at ≥2 days of life in infants with omphalocele, especially in those with liver within the omphalocele sac and/or in those infants who require intubation at birth to screen for pulmonary hypertension.


Assuntos
Hérnia Umbilical/epidemiologia , Hérnia Umbilical/terapia , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/terapia , Comorbidade , Feminino , Hérnia Umbilical/complicações , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/congênito , Lactente , Cuidado do Lactente/métodos , Cuidado do Lactente/normas , Recém-Nascido , Doenças do Recém-Nascido/epidemiologia , Doenças do Recém-Nascido/terapia , Unidades de Terapia Intensiva Neonatal , Masculino , Prevalência , Síndrome do Desconforto Respiratório do Recém-Nascido/epidemiologia , Síndrome do Desconforto Respiratório do Recém-Nascido/etiologia , Estudos Retrospectivos , Fatores de Risco
8.
J Thorac Cardiovasc Surg ; 148(5): 2181-8, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25109755

RESUMO

OBJECTIVE: Hypoxic-ischemic white mater brain injury commonly occurs in neonates with hypoplastic left heart syndrome (HLHS). Approximately one half of HLHS survivors will exhibit neurobehavioral symptoms believed to be associated with this injury, although the exact timing of the injury is unknown. METHODS: Neonates with HLHS were recruited for pre- and postoperative monitoring of cerebral oxygen saturation, cerebral oxygen extraction fraction, and cerebral blood flow using 2 noninvasive optical-based techniques: diffuse optical spectroscopy and diffuse correlation spectroscopy. Anatomic magnetic resonance imaging was performed before and approximately 1 week after surgery to quantify the extent and timing of the acquired white matter injury. The risk factors for developing new or worsened white matter injury were assessed using uni- and multivariate logistic regression. RESULTS: A total of 37 neonates with HLHS were studied. On univariate analysis, neonates who developed a large volume of new, or worsened, postoperative white matter injury had a significantly longer time to surgery (P=.0003). In a multivariate model, a longer time between birth and surgery, delayed sternal closure, and greater preoperative cerebral blood flow were predictors of postoperative white matter injury. Additionally, a longer time to surgery and greater preoperative cerebral blood flow on the morning of surgery correlated with lower cerebral oxygen saturation (P=.03 and P=.05, respectively) and greater oxygen extraction fraction (P=.05 for both). CONCLUSIONS: A longer time to surgery was associated with new postoperative white matter injury in otherwise healthy neonates with HLHS. The results suggest that earlier Norwood palliation might decrease the likelihood of acquiring postoperative white matter injury.


Assuntos
Circulação Cerebrovascular , Hemodinâmica , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Hipóxia-Isquemia Encefálica/etiologia , Leucoencefalopatias/etiologia , Procedimentos de Norwood/efeitos adversos , Tempo para o Tratamento , Biomarcadores/sangue , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Hipóxia-Isquemia Encefálica/diagnóstico , Hipóxia-Isquemia Encefálica/fisiopatologia , Recém-Nascido , Leucoencefalopatias/diagnóstico , Leucoencefalopatias/fisiopatologia , Modelos Logísticos , Imageamento por Ressonância Magnética , Masculino , Análise Multivariada , Oxigênio/sangue , Cuidados Paliativos , Fatores de Proteção , Fatores de Risco , Espectroscopia de Luz Próxima ao Infravermelho , Fatores de Tempo , Resultado do Tratamento
9.
J Thorac Cardiovasc Surg ; 147(4): 1312-8, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23879933

RESUMO

BACKGROUND: Preoperative brain injury is common in neonates with complex congenital heart disease. Increasing evidence suggests a complex interaction of prenatal and postnatal risk factors for development of brain white matter injury, called periventricular leukomalacia (PVL), in neonates with complex congenital heart disease. To date, there remains a limited understanding of the risk factors contributing to preoperative PVL in hypoplastic left heart syndrome (HLHS). METHODS: Neonates with HLHS or HLHS variants from 3 prospective magnetic resonance imaging studies (2003-2010) were selected for this cohort. Preoperative brain magnetic resonance imaging was performed the morning of the surgery. Stepwise multilogistic regression of patient characteristics, mode of delivery (cesarean section vs vaginal), time of diagnosis (prenatal vs postnatal), HLHS subtypes, brain total maturation score, time to surgery, individual averaged daily preoperative blood gases, and complete blood cell count values was used to determine significant associations. RESULTS: A total of 57 neonates with HLHS were born at 38.7 ± 2.3 weeks; 86% (49/57) had a prenatal diagnosis, with 31% (18/57) delivered by cesarean section. HLHS with aortic atresia (AA) was common in this cohort, 71% (41/57). Preoperative PVL was identified in 19% (11/57). Male patients with AA (P = .004) were at higher risk for PVL. Lower total brain maturation score was also identified as a strong predictor for preoperative PVL (P = .005). CONCLUSIONS: In neonates with HLHS, nonmodifiable patient-related factors, including male sex with AA (lack of antegrade blood flow) and lower total brain maturation score, placed neonates at the greatest risk for preoperative white matter injury.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Leucomalácia Periventricular/complicações , Feminino , Humanos , Recém-Nascido , Leucomalácia Periventricular/epidemiologia , Masculino , Período Pré-Operatório , Estudos Prospectivos , Fatores de Risco
10.
J Ultrasound Med ; 32(12): 2115-23, 2013 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24277893

RESUMO

OBJECTIVES: The purpose of this study was to test the hypothesis that using the formula of a prolate ellipsoid to estimate parabiotic twin mass correlates better with findings of pump twin compromise than using the sonographic method of Moore et al (Am J Obstet Gynecol 1990; 163:907-912). METHODS: A 10-year retrospective review was performed to identify all cases of the suspected twin reversed arterial perfusion (TRAP) sequence. Parabiotic twin mass was estimated by summing body and extremity volumes calculated using the prolate ellipsoid formula (width × height × length × 0.523). Parabiotic twin mass was also estimated using the sonographic Moore method [1.21 × length(2) - (1.66 × length)]. Parabiotic twin mass estimated by both methods was correlated with sonographic findings associated with increased risk of pump twin compromise. RESULTS: Fifty-nine pregnancies complicated by TRAP were identified. Using the prolate ellipsoid formula, the parabiotic twin mean sizes ± SD (as a percentage of pump twin weight) were 103.0% ± 52.0% and 56.9% ± 44.3% in cases with and without pump twin compromise (P = .0005), respectively. Using the sonographic Moore method, the mean parabiotic twin sizes were 122.9% ± 54.3% and 99.6% ± 62.8% in cases with and without pump twin compromise (P = .14). The median estimated masses of the parabiotic twin were 197 ± 219 g using the prolate ellipsoid formula and 310 ± 212 g using the sonographic Moore method (P = .0001). A parabiotic twin size greater than 70% of the pump twin correlated with findings associated with increased risk of pump twin compromise when using the prolate ellipsoid formula (P = .002) but not the sonographic Moore method (P = .09). CONCLUSIONS: Sonographic findings associated with increased risk of pump twin compromise correlate better with prolate ellipsoid estimates of parabiotic twin mass. The median estimated mass of the parabiotic twin was statistically larger when calculated by the sonographic Moore method than by the prolate ellipsoid formula.


Assuntos
Algoritmos , Transfusão Feto-Fetal/diagnóstico por imagem , Transfusão Feto-Fetal/embriologia , Aumento da Imagem/métodos , Interpretação de Imagem Assistida por Computador/métodos , Ultrassonografia Pré-Natal/métodos , Feminino , Humanos , Masculino , Gravidez , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
11.
Pediatr Res ; 73(5): 668-73, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-23403802

RESUMO

BACKGROUND: Sodium bicarbonate (NaHCO3) is a common treatment for metabolic acidemia; however, little definitive information exists regarding its treatment efficacy and cerebral hemodynamic effects. This pilot observational study quantifies relative changes in cerebral blood flow (ΔrCBF) and oxy- and deoxyhemoglobin concentrations (ΔHbO2 and ΔHb) due to bolus administration of NaHCO3 in patients with mild base deficits. METHODS: Infants and children with hypoplastic left heart syndrome (HLHS) were enrolled before cardiac surgery. NaHCO3 was given as needed for treatment of base deficit. Diffuse optical spectroscopies were used for 15 min postinjection to noninvasively monitor ΔHb, ΔHbO2, and ΔrCBF relative to baseline before NaHCO3 administration. RESULTS: Twenty-two anesthetized and mechanically ventilated patients with HLHS (aged 1 d to 4 y) received a median (interquartile range) dose of 1.1 (0.8, 1.8) mEq/kg NaHCO3 administered intravenously over 10-20 s to treat a median (interquartile range) base deficit of -4 (-6, -3) mEq/l. NaHCO3 caused significant dose-dependent increases in ΔrCBF; however, population-averaged ΔHb and ΔHbO2 as compared with those of controls were not significant. CONCLUSIONS: Dose-dependent increases in cerebral blood flow (CBF) caused by bolus administration of NaHCO3 are an important consideration in vulnerable populations wherein risk of rapid CBF fluctuations does not outweigh the benefit of treating a base deficit.


Assuntos
Circulação Cerebrovascular/efeitos dos fármacos , Bicarbonato de Sódio/farmacologia , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino
12.
J Thorac Cardiovasc Surg ; 145(1): 196-203, 205.e1; discussion 203-5, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23111021

RESUMO

OBJECTIVE: The early postoperative period following neonatal cardiac surgery is a time of increased risk for brain injury, yet the mechanisms underlying this risk are unknown. To understand these risks more completely, we quantified changes in postoperative cerebral metabolic rate of oxygen (CMRO(2)), oxygen extraction fraction (OEF), and cerebral blood flow (CBF) compared with preoperative levels by using noninvasive optical modalities. METHODS: Diffuse optical spectroscopy and diffuse correlation spectroscopy were used concurrently to derive cerebral blood flow and oxygen utilization postoperatively for 12 hours. Relative changes in CMRO(2), OEF, and CBF were quantified with reference to preoperative data. A mixed-effect model was used to investigate the influence of total support time and deep hypothermic circulatory arrest duration on relative changes in CMRO(2), OEF, and CBF. RESULTS: Relative changes in CMRO(2), OEF, and CBF were assessed in 36 patients, 21 with single-ventricle defects and 15 with 2-ventricle defects. Among patients with single-ventricle lesions, deep hypothermic circulatory arrest duration did not affect relative changes in CMRO(2), CBF, or OEF (P > .05). Among 2-ventricle patients, total support time was not a significant predictor of relative changes in CMRO(2) or CBF (P > .05), although longer total support time was associated significantly with greater increases in relative change of postoperative OEF (P = .008). CONCLUSIONS: Noninvasive diffuse optical techniques were used to quantify postoperative relative changes in CMRO(2), CBF, and OEF for the first time in this observational pilot study. Pilot data suggest that surgical duration does not account for observed variability in the relative change in CMRO(2), and that more comprehensive clinical studies using the new technology are feasible and warranted to elucidate these issues further.


Assuntos
Encéfalo/irrigação sanguínea , Encéfalo/metabolismo , Procedimentos Cirúrgicos Cardíacos , Circulação Cerebrovascular , Cardiopatias Congênitas/cirurgia , Consumo de Oxigênio , Oxigênio/sangue , Velocidade do Fluxo Sanguíneo , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Parada Circulatória Induzida por Hipotermia Profunda , Feminino , Cardiopatias Congênitas/sangue , Humanos , Lactente , Masculino , Projetos Piloto , Estudos Prospectivos , Fluxo Sanguíneo Regional , Espectroscopia de Luz Próxima ao Infravermelho , Fatores de Tempo , Resultado do Tratamento
13.
J Thorac Cardiovasc Surg ; 143(3): 535-42, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22340027

RESUMO

OBJECTIVE: Evaluate the impact of near-term delivery on neurodevelopmental (ND) outcomes in children with congenital heart disease (CHD). METHODS: Secondary analysis of data from a study of genetic polymorphisms and ND outcomes after cardiac surgery in infants. The effect of gestational age (GA) as a continuous variable on ND outcomes was evaluated using general linear regression models. GA was also evaluated as a categorical variable to seek a threshold for better outcomes. ND domains tested at 4 years of age included cognition, language skills, attention, impulsivity, memory, executive function, social competence, visual-motor, and fine-motor skills. RESULTS: ND outcomes and GA were available for 378 infants. Median GA was 39 weeks (range, 28-42 weeks) with 351 born at 36 weeks or more (near-term/term). In univariate analysis of the near-term/term subgroup, older GA predicted better performance for cognition, visual-motor, and fine-motor skills. After covariate adjustment, older GA predicted better performance for fine-motor skills (P = .018). Performance for cognition, language, executive function, social skills, visual-motor, and fine-motor skills was better for those born at 39 to 40 weeks of GA or more versus those born at less than 39 weeks (all P < .05). CONCLUSIONS: These findings are consistent with the hypothesis that delivery before 39 to 40 weeks of GA is associated with worse outcomes in patients with CHD. Early delivery of a child with CHD is often indicated because of maternal or fetal health issues. In the absence of these concerns, these data suggest that elective (or spontaneous) delivery at 39 to 40 weeks of GA is associated with better ND outcomes.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Comportamento Infantil , Desenvolvimento Infantil , Deficiências do Desenvolvimento/etiologia , Cardiopatias Congênitas/cirurgia , Recém-Nascido Prematuro , Fatores Etários , Atenção , Linguagem Infantil , Pré-Escolar , Cognição , Deficiências do Desenvolvimento/diagnóstico , Deficiências do Desenvolvimento/psicologia , Função Executiva , Feminino , Idade Gestacional , Humanos , Comportamento Impulsivo , Lactente , Recém-Nascido , Modelos Lineares , Masculino , Memória , Destreza Motora , Testes Neuropsicológicos , Philadelphia , Valor Preditivo dos Testes , Desempenho Psicomotor , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Comportamento Social , Resultado do Tratamento
14.
Prog Pediatr Cardiol ; 29(2): 79-85, 2010 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-20802830

RESUMO

Brain and heart development occurs simultaneously in the fetus with congenital heart disease. Early morphogenetic programs in each organ share common genetic pathways. Brain development occurs across a more protracted time-course with striking brain growth and activity-dependent formation and refinement of connections in the third trimester. This development is associated with increased metabolic activity and the brain is dependent upon the heart for oxygen and nutrient delivery. Congenital heart disease leads to derangements of fetal blood flow that result in impaired brain growth and development that can be measured with advanced magnetic resonance imaging. Delayed development results in a unique vulnerability to cerebral white matter injury in newborns with congenital heart disease. Delayed brain development and acquired white matter injury may underlay mild but pervasive neurodevelopmental impairment commonly observed in children following neonatal congenital heart surgery.

15.
Semin Perinatol ; 34(1): 46-56, 2010 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-20109972

RESUMO

Advances in medical and surgical care of the high-risk neonate have led to increased survival. A significant number of these neonates suffer from neurodevelopmental delays and failure in school. The focus of clinical research has shifted to understanding events contributing to neurological morbidity in these patients. Assessing changes in cerebral oxygenation and regulation of cerebral blood flow (CBF) is important in evaluating the status of the central nervous system. Traditional CBF imaging methods fail for both ethical and logistical reasons. Optical near infrared spectroscopy (NIRS) is increasingly being used for bedside monitoring of cerebral oxygenation and blood volume in both very low birth weight infants and neonates with congenital heart disease. Although trends in CBF may be inferred from changes in cerebral oxygenation and/or blood volume, NIRS does not allow a direct measure of CBF in these populations. Two relatively new modalities, arterial spin-labeled perfusion magnetic resonance imaging and optical diffuse correlation spectroscopy, provide direct, noninvasive measures of cerebral perfusion suitable for the high-risk neonates. Herein we discuss the instrumentation, applications, and limitations of these noninvasive imaging techniques for measuring and/or monitoring CBF.


Assuntos
Circulação Cerebrovascular/fisiologia , Imageamento por Ressonância Magnética/métodos , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Eritrócitos/fisiologia , Humanos , Recém-Nascido , Imageamento por Ressonância Magnética/normas , Espectroscopia de Luz Próxima ao Infravermelho/normas , Marcadores de Spin
16.
Am J Cardiol ; 101(11): 1652-6, 2008 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-18489946

RESUMO

Double-inlet left ventricle (DILV) includes a diverse range of anatomic variables that affect the surgical strategy. The aim of this study was to determine the impact of anatomic subtype, associated anomalies, and fetal diagnosis on the management and outcomes of fetuses and infants with DILV. The outcomes of fetuses and infants with DILV diagnosed from 1990 to 2004 at 3 major referral centers were reviewed. Sixty-five cases of DILV were detected prenatally. Twenty-one of these pregnancies were terminated, including 17 of 37 (46%) in which the diagnoses were made at < or =24 weeks of gestation. An additional 106 patients were diagnosed with DILV within the first 3 months of life. The percentage of patients diagnosed prenatally increased significantly over the study period. Transplantation-free survival was 88%, 82%, 79%, and 76% at 1 month, 1 year, 5 years, and 10 years, respectively. Factors associated with improved survival in univariate analysis included year of birth after 1994, no neonatal Norwood or Damus procedure, and no neonatal surgery at all. In multivariate analysis, any neonatal surgery was the only factor associated with worse survival. Associated anomalies and prenatal diagnosis were not associated with postnatal outcome. In conclusion, although the frequency of prenatal diagnosis of DILV has increased significantly over the past 15 years, prenatal diagnosis is not associated with better postnatal survival. During this same period, postnatal survival has improved substantially. Neonatal surgery of any type was the only independent risk factor for worse survival.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Ecocardiografia/métodos , Coração Fetal/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Ultrassonografia Pré-Natal/métodos , Diagnóstico Diferencial , Feminino , Seguimentos , Idade Gestacional , Cardiopatias Congênitas/embriologia , Cardiopatias Congênitas/cirurgia , Humanos , Recém-Nascido , Ontário/epidemiologia , Gravidez , Resultado da Gravidez , Estudos Retrospectivos , Índice de Gravidade de Doença , Taxa de Sobrevida/tendências , Fatores de Tempo , Resultado do Tratamento , Estados Unidos/epidemiologia
17.
Am Heart J ; 153(5): 772-8, 2007 May.
Artigo em Inglês | MEDLINE | ID: mdl-17452152

RESUMO

BACKGROUND: The outcome of prenatally diagnosed tricuspid atresia (TA) is undefined. We sought to characterize clinical and echocardiographic features of fetal TA and to determine factors associated with mortality. METHODS AND RESULTS: All fetuses with TA (n = 88) seen at 3 tertiary care institutions from 1990 to 2005 were reviewed. There were 58 liveborn infants (median gestational age 38 weeks, range 24-40 weeks), 4 in utero demises, 25 terminations of pregnancy, and 1 mother lost to follow-up. Obstruction was present at the pulmonary valve in 27 (45%), aortic valve in 6 (10%), and aortic arch in 15 (25%). Three neonates received compassionate care, 1 died with multiple extracardiac anomalies, 2 were lost to follow-up, and 52 liveborns were actively managed with Blalock-Taussig shunt (23), Norwood palliation (14), pulmonary artery band (10), bidirectional cavopulmonary connection (3), atrial septostomy (1), and right outflow stent (1). Of those actively managed, there were 7 (14%) of 52 who died. Kaplan-Meier estimates of survival were 91% at 1 month, 87% at 6 months, and 83% at 1 year with no subsequent deaths for 13 years. By multivariate analysis, 2 independent factors were associated with an increase in time-related mortality in the actively managed group: presence of chromosomal anomaly or syndrome (P = .005) and use of extracorporeal membrane oxygenation (P = .002). CONCLUSIONS: This is the largest study describing TA in fetus. Compared with published observations of TA diagnosed postnatally, antenatal diagnosis of TA appears to have similar short-term survival in pregnancies surviving to birth.


Assuntos
Doenças Fetais/diagnóstico , Doenças Fetais/epidemiologia , Diagnóstico Pré-Natal/estatística & dados numéricos , Atresia Tricúspide/diagnóstico , Atresia Tricúspide/epidemiologia , Anormalidades Múltiplas/epidemiologia , Aborto Induzido/estatística & dados numéricos , Boston/epidemiologia , Causas de Morte , Transtornos Cromossômicos/epidemiologia , Comorbidade , Ecocardiografia , Oxigenação por Membrana Extracorpórea/estatística & dados numéricos , Feminino , Doenças Fetais/genética , Doenças Fetais/terapia , Idade Gestacional , Humanos , Masculino , Ontário/epidemiologia , Avaliação de Processos e Resultados em Cuidados de Saúde , Gravidez , Resultado da Gravidez , Estudos Retrospectivos , São Francisco/epidemiologia , Análise de Sobrevida , Atresia Tricúspide/genética , Atresia Tricúspide/terapia
18.
Eur J Appl Physiol ; 92(3): 285-8, 2004 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-15083364

RESUMO

There is still considerable debate and some confusion as to the most appropriate method of scaling or normalizing maximum oxygen uptake (VO2max) for differences in body mass (m) in both adults and children. Previous studies on adult populations have demonstrated that although the traditional ratio standard VO2max (ml kg(-1) min(-1)) fails to render VO2max independent of body mass, the ratio standard is still the best predictor of running performance. However, no such evidence exists in children. Hence, the purpose of the present study was to investigate whether the ratio standard is still the most appropriate method of normalising VO2max to predict 1-mile run speed in a group of 12-year-old children (n=36). Using a power function model and log-linear regression, the best predictor of 1-mile run speed was given by: speed (m s(-1))=55.1 VO2max(0.986) m(-0.96). With both the VO2max and body mass exponents being close to unity but with opposite signs, the model suggest the best predictor of 1-mile run speed is almost exactly the traditional ratio standard recorded in the units (ml kg(-1) min(-1)). Clearly, reporting the traditional ratio standard VO2max, recorded in the units (ml kg(-1) min(-1)), still has an important place in publishing the results of studies investigating cardiovascular fitness of both children and adults.


Assuntos
Algoritmos , Antropometria/métodos , Índice de Massa Corporal , Consumo de Oxigênio/fisiologia , Exame Físico/métodos , Exame Físico/normas , Corrida/fisiologia , Criança , Humanos , Masculino , Modelos Biológicos , Modelos Estatísticos , Oxigênio/metabolismo , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Análise e Desempenho de Tarefas , Estados Unidos
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