Asunto(s)
Procedimientos Quirúrgicos Cardíacos/normas , Cardiología/normas , Recuperación Mejorada Después de la Cirugía/normas , Cardiopatías Congénitas/cirugía , Pediatría/normas , Factores de Edad , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Consenso , Técnica Delphi , Medicina Basada en la Evidencia/normas , Humanos , Recuperación de la Función , Factores de Tiempo , Resultado del TratamientoRESUMEN
OBJECTIVE: To describe the assessment of Fontan-associated liver disease and determine the clinical and imaging measures that may identify hepatic morbidity risk in isolated heart transplantation candidates and trend those measures post-isolated heart transplantation. STUDY DESIGN: Retrospective analysis of pre-isolated heart transplantation and post-isolated heart transplantation Fontan-associated liver disease (FALD) status using blood tests, magnetic resonance imaging (MRI), and liver biopsy analysis within 6 months before isolated heart transplantation and 12 months after isolated heart transplantation in 9 consecutive patients with Fontan. Pre- and post-isolated heart transplantation standard laboratory values; varices, ascites, splenomegaly, thrombocytopenia (VAST) score; Fontan liver MRI score; liver biopsy scores; Model for End-stage Liver Disease (MELD); MELD excluding the International Normalized Ratio (MELD-XI); AST to platelet ratio index, and cardiac catheterization data were compared. RESULTS: Pretransplantation maximum MELD and MELD-XI was 15 and 16, respectively. Central venous pressures and VAST scores decreased significantly post-transplantation. In 5 paired studies, Fontan liver MRI score maximum was 10 pretransplantation and decreased significantly post-transplantation. Arterially enhancing nodules on MRI persisted in 2 patients post-transplantation. Pretransplantation and post-transplantation liver biopsy scores did not differ in 4 paired biopsy specimens. CONCLUSIONS: Patients with FALD and MELD <15, MELD-XI <16, Fontan liver MRI score <10, and VAST score ≤2 can have successful short-term isolated heart transplantation outcomes. Liver MRI and VAST scores improved post-transplantation. Post-transplantation liver biopsy scores did not change significantly. Pretransplantation liver biopsy demonstrating fibrosis alone should not exclude consideration of isolated heart transplantation. The persistence of hepatic vascular remodeling and fibrosis post-isolated heart transplantation suggests that continued surveillance for hepatic complications post-transplantation for patients with Fontan is reasonable.
Asunto(s)
Procedimiento de Fontan/efectos adversos , Trasplante de Corazón , Hepatopatías/diagnóstico , Selección de Paciente , Adolescente , Ascitis/diagnóstico por imagen , Biopsia , Presión Venosa Central , Niño , Humanos , Hígado/diagnóstico por imagen , Cirrosis Hepática/patología , Hepatopatías/etiología , Pruebas de Función Hepática , Imagen por Resonancia Magnética , Complicaciones Posoperatorias , Estudios Retrospectivos , Esplenomegalia/diagnóstico por imagen , Trombocitopenia , Várices/diagnóstico por imagen , Remodelación Vascular , Adulto JovenRESUMEN
OBJECTIVE: Assess differences in approaches to and provision of developmental care for infants undergoing surgery for congenital heart disease. STUDY DESIGN: A collaborative learning approach was used to stratify, assess, and compare individualized developmental care practices among multidisciplinary teams at 6 pediatric heart centers. Round robin site visits were completed with structured site visit goals and postvisit reporting. Practices of the hosting site were assessed by the visiting team and reviewed along with center self-assessments across specific domains including pain management, environment, cue-based care, and family based care coordination. RESULTS: Developmental care for infants in the cardiac intensive care unit (CICU) varies at both a center and individual level. Differences in care are primarily driven by variations in infrastructure and resources, composition of multidisciplinary teams, education of team members, and use of developmental care champions. Management of pain follows a protocol in most cardiac intensive care units, but the environment varies across centers, and the provision of cue-based infant care and family-based care coordination varies widely both within and across centers. The project led to proposed changes in clinical care and center infrastructure at each participating site. CONCLUSIONS: A collaborative learning design fostered rapid dissemination, comparison, and sharing of strategies to approach a complex multidisciplinary care paradigm. Our assessment of experiences revealed marked variability across and within centers. The collaborative findings were a first step toward strategies to quantify and measure developmental care practices in the cardiac intensive care unit to assess the association of complex inpatient practices with long-term neurodevelopmental outcomes.
Asunto(s)
Conducta Cooperativa , Cuidados Críticos/organización & administración , Unidades de Cuidado Intensivo Neonatal/organización & administración , Aprendizaje , Modelos Educacionales , Cardiopatías Congénitas/cirugía , Humanos , Recién Nacido , Grupo de Atención al Paciente , Estados UnidosAsunto(s)
Encéfalo/crecimiento & desarrollo , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Trastornos del Conocimiento/etiología , Discapacidades del Desarrollo/etiología , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Hiperglucemia/complicaciones , Femenino , Humanos , MasculinoRESUMEN
OBJECTIVE: To measure neurodevelopment at 3 years of age in children with single right-ventricle anomalies and to assess its relationship to Norwood shunt type, neurodevelopment at 14 months of age, and patient and medical factors. STUDY DESIGN: All subjects in the Single Ventricle Reconstruction Trial who were alive without cardiac transplant were eligible for inclusion. The Ages and Stages Questionnaire (ASQ, n = 203) and other measures of behavior and quality of life were completed at age 3 years. Medical history, including measures of growth, feeding, and complications, was assessed through annual review of the records and phone interviews. The Bayley Scales of Infant Development, Second Edition (BSID-II) scores from age 14 months were also evaluated as predictors. RESULTS: Scores on each ASQ domain were significantly lower than normal (P < .001). ASQ domain scores at 3 years of age varied nonlinearly with 14-month BSID-II. More complications, abnormal growth, and evidence of feeding, vision, or hearing problems were independently associated with lower ASQ scores, although models explained <30% of variation. Type of shunt was not associated with any ASQ domain score or with behavior or quality-of-life measures. CONCLUSION: Children with single right-ventricle anomalies have impaired neurodevelopment at 3 years of age. Lower ASQ scores are associated with medical morbidity, and lower BSID-II scores but not with shunt type. Because only a modest percentage of variation in 3-year neurodevelopmental outcome could be predicted from early measures, however, all children with single right-ventricle anomalies should be followed longitudinally to improve recognition of delays.
Asunto(s)
Discapacidades del Desarrollo/etiología , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Sistema Nervioso/crecimiento & desarrollo , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios ProspectivosRESUMEN
OBJECTIVES: Screening for critical congenital heart disease with pulse oximetry requires healthcare providers to decipher a previously published algorithm, a feature that raises concerns about quality of interpretation of pulse oximetry results. We hypothesized that this method would be prone to error and a computer-based tool would lead to a more accurate interpretation of the screening results. STUDY DESIGN: In this randomized crossover study, healthcare providers with prior experience using pulse oximetry received 2 sets of 10 mock screening scenarios and were asked to interpret the results of each scenario as "pass," "fail," or "retest." Participants were randomized to use either the paper algorithm or computer-based tool for the first set of 10 scenarios and the alternative method for the second set. We used Wilcoxon rank sum tests to compare the accuracy of interpretation using the 2 methods. RESULTS: The 102 participants answered 81.6% of the scenarios correctly when manually interpreting the algorithm vs 98.3% correct when using the computer-based tool (P < .001). These differences were most pronounced for the "fail" scenarios (65.4% manual vs 96.7% computer, P < .001) and the "retest" scenarios (80.7% manual vs 98.7% computer, P < .001), but were also significant for the "pass" scenarios (94.1% manual vs 99.0% computer, P < .001). CONCLUSIONS: Use of a manual algorithm for the interpretation of results in screening for critical congenital heart disease with pulse oximetry is susceptible to human error. Implementation of a computer-based tool to aid in the interpretation of the results may lead to improved accuracy and quality.
Asunto(s)
Algoritmos , Cardiopatías Congénitas/diagnóstico , Tamizaje Neonatal/métodos , Mejoramiento de la Calidad , Estudios Cruzados , Interpretación Estadística de Datos , Femenino , Humanos , Recién Nacido , Masculino , Oximetría , Reproducibilidad de los ResultadosRESUMEN
OBJECTIVE: To determine whether abdominal magnetic resonance imaging (MRI) detects hepatic abnormalities before clinical or biochemical perturbations in patients after the Fontan procedure. STUDY DESIGN: Thirty-nine children and adolescents who underwent the Fontan procedure and were referred to a pediatric hepatologist by cardiology services between 2011 and 2012 were reviewed retrospectively. Physical examination findings, routine laboratory tests of liver function, evaluation for chronic liver disease, and abdominal MRI findings were recorded. MRI findings were evaluated relative to time elapsed since surgery by 2 radiologists (blinded). RESULTS: Assessment for coexisting chronic liver disease was negative in all patients. All patients had a normal serum albumin level and International Normalized Ratio. Twenty-six of the 39 patients (67%) underwent abdominal MRI, 4 had MRI-incompatible hardware, and 9 did not undergo MRI because of insurance denial. All MRI scans demonstrated morphologic liver changes with varying degrees of reticular contrast enhancement compatible with fibrosis and congestion. Reticular contrast enhancement was often nonuniform, and 9 patients (35%) had multifocal arterially enhancing lesions. CONCLUSION: MRI can identify hepatic abnormalities in patients after Fontan surgery that go undetected by standard clinical and laboratory assessments. These abnormalities are not uniformly distributed throughout the liver, and thus assessment by liver biopsy analysis is subject to sampling error.
Asunto(s)
Procedimiento de Fontan , Hepatopatías/diagnóstico , Imagen por Resonancia Magnética , Complicaciones Posoperatorias/diagnóstico , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECTIVES: This study assessed racial/ethnic disparities in post-operative mortality after surgery for congenital heart disease (CHD) and explored whether disparities persist after adjusting for access to care. STUDY DESIGN: We used the Pediatric Health Information System database to perform a retrospective cohort study of 44,017 patients with 49,833 CHD surgery encounters in 2004-2008 at 41 children's hospitals. We used χ(2) analysis to compare unadjusted mortality rates by race/ethnicity (non-Hispanic white, non-Hispanic black, Hispanic) and constructed Poisson regression models to determine adjusted mortality risk ratios (RRs) and 95% CIs. RESULTS: In-hospital post-operative mortality rate was 3.4%; non-Hispanic whites had the lowest mortality rate (2.8%), followed by non-Hispanic blacks (3.6%) and Hispanics (3.9%) (P < .0001). After adjusting for age, sex, genetic syndrome, and surgery risk category, the RR of death was 1.32 for non-Hispanic blacks (CI, 1.14-1.52) and 1.21 for Hispanics (CI, 1.07-1.37), both compared with non-Hispanic whites. After adjusting for access to care (insurance type and hospital of surgery), these estimates did not appreciably change (non-Hispanic blacks: RR, 1.27; CI, 1.09-1.47; Hispanics: RR, 1.22; CI, 1.05-1.41). CONCLUSIONS: There are notable racial/ethnic disparities in post-operative mortality after CHD surgery that do not appear to be explained by differences in access to care.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Etnicidad , Cardiopatías Congénitas/etnología , Grupos Raciales , Preescolar , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/cirugía , Humanos , Lactante , Recién Nacido , Masculino , Periodo Posoperatorio , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Estados Unidos/epidemiologíaRESUMEN
OBJECTIVE: To evaluate the outcomes associated with isolated bicuspid aortic valve (BAV) during childhood and adolescence. STUDY DESIGN: Analysis of a large single institutional cohort of children (n=981) with isolated BAV was undertaken to determine the prevalence of significant ascending aortic dilation and risk of cardiac events. Subjects with known genetic disorders, critical aortic stenosis (intervention required in infancy), or additional lesions such as coarctation of the aorta were excluded. Aortic dimensions were derived from echocardiography, and values were plotted as Z scores. Clinical outcomes included death, aortic dissection, balloon aortic valvuloplasty, or cardiac surgery. RESULTS: The median age of the subjects at diagnosis was 8.3 years. At the time of the last pediatric follow-up, 7% of the subjects had moderate aortic regurgitation or greater, and the median Z score for the ascending aorta was +2.31. There were 9427 patient years of follow-up. Primary cardiac events occurred in 38 subjects, yielding an event rate of 0.004 per patient year. Eleven subjects (1.1%) underwent aortic valve surgery. Thirty subjects (3.0%) underwent balloon dilatation of the aortic valve. There was a single case of endocarditis. There were no cardiac-related deaths and no cases of aortic dissection. CONCLUSIONS: The incidence of primary cardiac events in children with BAV is relatively low, approximately 3-fold lower than in young adults, and is generally related to aortic stenosis amenable to balloon dilatation. Although mild ascending aortic dilation is common in children, the clinical course is relatively benign. In this series, aortic dissection did not occur. Whether elective surgery for the dilated aorta has a role in children remains unknown.
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Enfermedades de la Aorta/epidemiología , Válvula Aórtica/anomalías , Enfermedades de las Válvulas Cardíacas/epidemiología , Adolescente , Aorta , Enfermedades de la Aorta/etiología , Niño , Preescolar , Estudios de Cohortes , Femenino , Enfermedades de las Válvulas Cardíacas/etiología , Humanos , Lactante , MasculinoRESUMEN
OBJECTIVE: To determine an accurate estimate of the prevalence of congenital heart defects (CHD) using current standard diagnostic modalities. STUDY DESIGN: We obtained data on infants with CHD delivered during 1998 to 2005 identified by the Metropolitan Atlanta Congenital Defects Program, an active, population-based, birth defects surveillance system. Physiologic shunts in infancy and shunts associated with prematurity were excluded. Selected infant and maternal characteristics of the cases were compared with those of the overall birth cohort. RESULTS: From 1998 to 2005 there were 398 140 births, of which 3240 infants had CHD, for an overall prevalence of 81.4/10 000 births. The most common CHD were muscular ventricular septal defect, perimembranous ventricular septal defect, and secundum atrial septal defect, with prevalence of 27.5, 10.6, and 10.3/10 000 births, respectively. The prevalence of tetralogy of Fallot, the most common cyanotic CHD, was twice that of transposition of the great arteries (4.7 vs 2.3/10 000 births). Many common CHD were associated with older maternal age and multiple-gestation pregnancy; several were found to vary by sex. CONCLUSIONS: This study, using a standardized cardiac nomenclature and classification, provides current prevalence estimates of the various CHD subtypes. These estimates can be used to assess variations in prevalence across populations, time, or space.
Asunto(s)
Cardiopatías Congénitas/epidemiología , Vigilancia de la Población/métodos , Femenino , Georgia/epidemiología , Cardiopatías Congénitas/clasificación , Humanos , Recién Nacido , Masculino , Edad Materna , Registros Médicos , Estudios Multicéntricos como Asunto , Prevalencia , Población UrbanaRESUMEN
OBJECTIVE: Children born with hypoplastic left heart syndrome (HLHS) may experience cardiac dysfunction after staged surgery or transplantation, which may worsen with age. We examined the hypothesis that exercise testing can address cardiovascular capacity and suggest interventions to improve quality of life. STUDY DESIGN: Children with HLHS > or = 8 years old performed treadmill or bicycle ergometric testing at 4 centers. Results were compared with norms for age and sex. RESULTS: Of the 42 participants, the mean age was 12.9 years (range, 8.5-17.0 years), 64% were boys, 20 had staged surgery, and 34 completed metabolic assessment. The percent of predicted maximal oxygen uptake (mVO2) was higher in younger children. Children aged 8 to 12 years achieved 70% of predicted mVO2; children aged 13 to 17 years achieved 60% of predicted mVO2 (P = .02). The percent of predicted peak heart rate trended higher in younger patients (83% versus 75%, P = .07). Electrocardiographic changes were more common in older children. In treadmill testing, patients who had a transplant had better exercise performance than patients who underwent staged surgery in percent of predicted exercise time (82% versus 54%, P < .0001) and peak rate-pressure product (241 x 10(3) versus 195 x 10(3), P = .02). The percent of predicted mVO2 did not differ between patients who had a transplant (66%) and patients who underwent staged surgery (61%, P = .25). CONCLUSION: Children with HLHS showed considerable age-related decline in exercise performance, regardless of surgical strategy.
Asunto(s)
Tolerancia al Ejercicio , Procedimiento de Fontan , Trasplante de Corazón/fisiología , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Adolescente , Análisis de Varianza , Presión Sanguínea , Estudios de Casos y Controles , Niño , Electrocardiografía , Prueba de Esfuerzo , Femenino , Frecuencia Cardíaca , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/metabolismo , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Masculino , Consumo de Oxígeno , Calidad de Vida , Valores de ReferenciaRESUMEN
OBJECTIVES: To determine the incidence, characteristics, and outcomes for adolescents diagnosed with acute myocardial infarction (AMI). STUDY DESIGN: We analyzed data from the Nationwide Inpatient Sample to determine the incidence of AMI in adolescents (age 13 to 18 years). Data from 1998 to 2001 were analyzed. Patients with primary cardiomyopathies, congenital heart lesions, and previous heart transplantation were excluded. RESULTS: The estimated incidence of AMI admissions of adolescents in the United States is 157 per year, or 6.6 events per 1 million patient-years. AMI occurs predominantly in males (80%). Reported incidences of substance abuse and smoking are significantly higher in the adolescents with AMI than in adolescents admitted to the hospital for other conditions (P < .001 for both). Of the 123 subjects with AMI that we studied, 36 underwent coronary angiography (29%) and 2 underwent coronary artery bypass graft surgery (1%). The hospital mortality rate was 0.8%. CONCLUSIONS: AMI is extremely rare in adolescents. Patient characteristics associated with AMI include substance abuse, tobacco use, and male sex. The hospital survival for AMI in adolescents is excellent, and the need for catheter or surgical coronary artery intervention is uncommon.
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Infarto del Miocardio/epidemiología , Infarto del Miocardio/terapia , Revascularización Miocárdica/métodos , Terapia Trombolítica/métodos , Adolescente , Factores de Edad , Estudios de Cohortes , Intervalos de Confianza , Angiografía Coronaria , Femenino , Humanos , Incidencia , Modelos Logísticos , Masculino , Infarto del Miocardio/diagnóstico , Oportunidad Relativa , Probabilidad , Pronóstico , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Factores Sexuales , Análisis de Supervivencia , Estados Unidos/epidemiologíaRESUMEN
OBJECTIVE: To examine the relationship of black race to graft survival after heart transplantation in children. STUDY DESIGN: United Network for Organ Sharing records of heart transplantation for subjects <18 years of age from 1987 to 2004 were reviewed. Analysis was performed using proportional hazards regression controlling for other potential risk factors. RESULTS: Of the 4227 pediatric heart transplant recipients, 717 (17%) were black. The 1-year graft survival rate did not differ among groups; however, the 5-year graft survival rate was significantly lower for black recipients, 51% versus 69%, P < .001. The median graft survival for black recipients was 5.3 years as compared with 11.0 years for other recipients. Black recipients had a greater number of human leukocyte antigen mismatches, lower median household income, and a greater percentage with Medicaid as primary insurance, P < .001, P < .001, and P < .001. After adjusting for economic disparities, black race remained significantly associated with graft failure, odds ratio = 1.67 (95% CI 1.47 to 1.87), P < .001. CONCLUSIONS: Median graft survival after pediatric heart transplantation for black recipients is less than half that of other racial groups. These differences do not appear to be related primarily to economic disparities.