RESUMO
Background: Infants with hypoplastic left heart syndrome (HLHS) or a variant are at risk of ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) prior to superior cavopulmonary connection (SCPC). Although the impact of these complications in isolation has been described, their effect in combination on attrition is poorly defined. Methods: A retrospective observational study of patients with HLHS or variants undergoing a Norwood procedure between 2008 and 2020 at a single center was performed. VD and AVVR were defined as moderate or severe when seen on 2 sequential echocardiograms outside the perioperative period. Attrition was defined as death, listing for heart transplant, or unsuitability for SCPC or transplant. Descriptive statistics and regression models were used for analysis. Results: A total of 397 patients were included, of whom 75% had HLHS and 57% had received a Blalock-Thomas-Taussig shunt. Isolated VD occurred in 9% of patients, AVVR occurred in 13%, and both occurred in 6%. Attrition prior to SCPC occurred in 19% of the overall cohort, in 52% of patients with combined VD and AVVR (odds ratio [OR], 5.2; 95% confidence interval [CI], 2.3-12.0; P < .01), 26% of those with VD (OR, 1.5; 95% CI, 0.7-3.3; P = .32), 25% of those with AVVR (OR, 1.5; 95% CI, 0.7-2.9; P = .27), and 15% in those with neither (OR, 0.3; 95% CI, 0.2-0.6; P < .01). Other factors associated with attrition included prematurity, total bypass time at Norwood, and extracorporeal membrane oxygenation after Norwood, whereas later year of Norwood was protective (P < .01 for all). Conclusions: The presence of combined VD and AVVR markedly increases the likelihood of attrition prior to SCPC, identifying a high-risk group.
RESUMO
OBJECTIVES: To investigate the prevalence of hearing loss after cardiac surgery in infancy, patient and operative factors associated with hearing loss, and the relationship of hearing loss to neurodevelopmental outcomes. STUDY DESIGN: Audiologic and neurodevelopmental evaluations were conducted on 348 children who underwent repair of congenital heart disease at the Children's Hospital of Philadelphia as part of a prospective study evaluating neurodevelopmental outcomes at 4 years of age. A prevalence estimate was calculated based on presence and type of hearing loss. Potential risk factors and the impact of hearing loss on neurodevelopmental outcomes were evaluated. RESULTS: The prevalence of hearing loss was 21.6% (95% CI, 17.2-25.9). The prevalence of conductive hearing loss, sensorineural hearing loss, and indeterminate hearing loss were 12.4% (95% CI, 8.8-16.0), 6.9% (95% CI, 4.1-9.7), and 2.3% (95% CI, 0.6-4.0), respectively. Only 18 of 348 subjects (5.2%) had screened positive for hearing loss before this study and 10 used a hearing aid. After adjusting for patient and operative covariates, younger gestational age, longer postoperative duration of stay, and a confirmed genetic anomaly were associated with hearing loss (all P < .01). The presence of hearing loss was associated with worse language, cognition and attention (P <.01). CONCLUSIONS: These findings suggest that the prevalence of hearing loss in preschool children after heart surgery in infancy may be 20-fold higher than in the 1% prevalence seen in the general population. Younger gestational age, presence of a genetic anomaly, and longer postoperative duration of stay were associated with hearing loss. Hearing loss was associated with worse neurodevelopmental outcomes.
Assuntos
Perda Auditiva/etiologia , Cardiopatias Congênitas/cirurgia , Complicações Pós-Operatórias/etiologia , Desenvolvimento Infantil , Pré-Escolar , Feminino , Seguimentos , Perda Auditiva/diagnóstico , Perda Auditiva/epidemiologia , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Prevalência , Estudos Prospectivos , Fatores de RiscoRESUMO
OBJECTIVE: To test the hypotheses that an innovative skills-based behavioral family clinic and home-based intervention (LAUNCH) would reduce body mass index z score (BMIz) compared with motivational interviewing and to standard care in preschool-aged children with obesity. STUDY DESIGN: Randomized controlled trial with children between the ages of 2 and 5 years above the 95th percentile for body mass index for age and sex recruited from 27 pediatrician offices across 10 recruitment cycles between March 12, 2012 and June 8, 2015. Children were randomized to LAUNCH (an 18-session clinic and home-based behavioral intervention), motivational interviewing (delivered at the same frequency as LAUNCH), or standard care (no formal intervention). Weight and height were measured by assessors blinded to participant assignment. The primary outcome, BMIz at month 6 after adjusting for baseline BMIz, was tested separately comparing LAUNCH with motivational interviewing and LAUNCH with standard care using regression-based analysis of covariance models. RESULTS: A total of 151 of the 167 children randomized met intent-to-treat criteria and 92% completed the study. Children were 76% White and 57% female, with an average age of 55 months and BMI percentile of 98.57, with no demographic differences between the groups. LAUNCH participants demonstrated a significantly greater decrease in BMIz (mean = -0.32, SD = ±0.33) compared with motivational interviewing (mean = -0.05, SD = ±0.27), P < .001, ω2 = 0.74 and compared with standard care (mean = -0.13, SD = ±0.31), P < .004, ω2 = 0.75. CONCLUSIONS: In preschool-age children, an intensive 6-month behavioral skills-based intervention is necessary to reduce obesity. TRIAL REGISTRATION: Clinicaltrials.gov NCT01546727.
Assuntos
Assistência Ambulatorial/métodos , Terapia Comportamental/métodos , Serviços de Assistência Domiciliar , Obesidade Infantil/terapia , Índice de Massa Corporal , Pré-Escolar , Feminino , Seguimentos , Humanos , Análise de Intenção de Tratamento , Masculino , Entrevista Motivacional , Obesidade Infantil/diagnóstico , Método Simples-Cego , Resultado do TratamentoRESUMO
OBJECTIVE: Extracorporeal membrane oxygenation is an important form of short-term mechanical support in children with cardiac disease, but information on long-term outcomes and quality of life is limited. The primary objective of this study was to determine the long-term outcomes of children previously supported by extracorporeal membrane oxygenation for cardiac etiologies. DESIGN: A retrospective analysis was performed on patients with cardiac disease managed with extracorporeal membrane oxygenation between January 1, 1995, and December 31, 2012, at the Children's Hospital of Philadelphia. Survivors completed patient- and parent-reported verbal and written surveys, and univariate analyses assessed risk factors for long-term outcomes. SETTING: Tertiary-care children's hospital. PATIENTS: Patients with cardiac disease managed with extracorporeal membrane oxygenation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Over 18 years, 396 patients were managed with extracorporeal membrane oxygenation with 43% survival to discharge. The median age at cannulation was 78 days. The majority had congenital heart disease (86%), surgery prior to extracorporeal membrane oxygenation (71%), and cardiopulmonary arrest as the primary extracorporeal membrane oxygenation indication (53%). With 6-year median follow-up, 66% are known to be deceased, including 38 deaths after hospital discharge. Among survivors at discharge, 65 (38%) completed the phone survey, and 33 (19%) completed the written survey. Negative clinical outcomes, defined as having at least significant physical limitations or "fair" or "poor" health, were present in 18% of patients. No patient- or extracorporeal membrane oxygenation-related variables were associated with negative outcomes in univariate analyses. There were significantly lower self-reported and parent-reported written Pediatric Quality of Life Inventory quality of life scores in children compared with healthy individual normative data but no differences in adolescents. CONCLUSIONS: In this series of pediatric cardiac patients supported by extracorporeal membrane oxygenation, mortality was 66% with 6-year median follow-up. The majority reported positive outcomes with respect to health and physical limitations, but children reported lower quality of life compared with healthy individuals.
Assuntos
Oxigenação por Membrana Extracorpórea , Cardiopatias/terapia , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Indicadores Básicos de Saúde , Cardiopatias/mortalidade , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVES: Despite improved survival in children with hypoplastic left heart syndrome (HLHS), significant concern persists regarding their neurodevelopmental (ND) outcomes. Previous studies have identified patient factors, such as prematurity and genetic syndromes, to be associated with worse ND outcomes. However, no consistent relationships have been identified among modifiable management factors, including cardiopulmonary bypass strategies, and ND outcomes after cardiac surgery in infancy. Studies in immature animals, including primates, have demonstrated neurodegeneration and apoptosis in the brain after certain levels and extended durations of anesthetic exposure. Retrospective human studies have also suggested relationships between adverse ND effects and anesthetic exposure. METHODS: Cumulative minimum alveolar concentration hours (MAC-hrs) of exposure to volatile anesthetic agents (VAA) (desflurane, halothane, isoflurane, and sevoflurane) were collected from an anesthetic database and medical record review for 96 patients with HLHS or variants. ND testing was performed between ages 4 and 5 years, including full-scale IQ, verbal IQ, performance IQ, and processing speed. Four generalized linear modes were hypothesized a priori and tested using a Gaussian (normal) distribution with an identity link. RESULTS: Cumulative VAA exposure ranged from 0 to 35.3 MAC-hrs (median 7.5 hours). Using specified covariates identified previously as significant predictors of ND outcomes, statistically significant relationships were identified between total MAC-hrs exposure and worse full-scale IQ and verbal IQ scores (P's < .05) alone and after adjusting for relevant covariates. CONCLUSIONS: Increased cumulative MAC-hrs exposure to VAA is associated with worse ND outcomes in certain domains in children with HLHS and variants.
Assuntos
Anestesia por Inalação/efeitos adversos , Anestésicos Inalatórios/efeitos adversos , Procedimentos Cirúrgicos Cardíacos , Comportamento Infantil/efeitos dos fármacos , Desenvolvimento Infantil/efeitos dos fármacos , Deficiências do Desenvolvimento/induzido quimicamente , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Sistema Nervoso/efeitos dos fármacos , Fatores Etários , Anestésicos Inalatórios/administração & dosagem , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Pré-Escolar , Bases de Dados Factuais , Deficiências do Desenvolvimento/diagnóstico , Deficiências do Desenvolvimento/fisiopatologia , Deficiências do Desenvolvimento/psicologia , Relação Dose-Resposta a Droga , Função Executiva , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Inteligência , Modelos Lineares , Masculino , Prontuários Médicos , Sistema Nervoso/crescimento & desenvolvimento , Testes Neuropsicológicos , Estudos Retrospectivos , Fatores de Risco , Comportamento VerbalRESUMO
OBJECTIVES: To describe congenital heart disease death rates in infants born between 34 and 40 weeks, estimate the relationship between gestational age and congenital heart disease infant death rates, and compare congenital heart disease death rates across 1- and 2-week intervals in gestational age. STUDY DESIGN: The 2000 to 2003 national linked birth/infant death cohort datasets were obtained. Congenital heart disease deaths were identified by using International Statistical Classification of Diseases, 10th Revision codes. Proportional death rates were calculated by using congenital heart disease deaths and all live births. The relationship between congenital heart disease death rates and gestational age was determined. Death rates were compared across intervals. RESULTS: A total of 14.9 million records were analyzed. Congenital heart disease deaths occurred in 4736 infants (0.04%) born between 34 and 40 weeks. There was a significant, negative linear relationship between congenital heart disease death rate and gestational age (R(2) = 0.97). Comparisons across 1-week intervals varied (P = .02-.23). All 2-week intervals were statistically significant (P < .01). CONCLUSIONS: Congenital heart disease death rates decrease as gestational age approaches 40 weeks. These results should be considered before elective delivery for the sole indication of prenatally diagnosed congenital heart disease.