RESUMEN

OBJECTIVES: To evaluate patterns and determinants of longitudinal growth among children requiring complex biventricular repair for congenital heart disease, as well as to assess for associations of growth with early feeding modality, comorbidities, postoperative complications, and socioeconomic characteristics. STUDY DESIGN: A single-institution retrospective cohort study was performed in children born February 1999 to March 2009 with complex congenital heart disease who underwent biventricular repair before age 4 years, defined by Risk Adjustment in Congenital Heart Surgery-1 category 3-5. Clinical characteristics, height, weight, and body mass index (BMI) from ages 2-12 years were collected by chart review. Neighborhood-level socioeconomic data were identified using a geographic information system approach. The adjusted association of covariates with growth outcomes was estimated using multivariable linear regression models using generalized estimating equations. RESULTS: Compared with population growth curves, the cohort (n = 150) trended toward early decrease in age-adjusted weight and height. Early tube feeding was significantly associated with decreased BMI before adolescence (-0.539; 95% CI -1.02, -0.054; P = .029). In addition, other clinical and perioperative characteristics had significant associations with growth, including low birth weight, preoperative tube feeds, need for multiple bypass runs, and diagnosis of feeding disorder. CONCLUSIONS: Early childhood growth in children with complex biventricular repair may be impaired. Early tube feeding was associated with decreased BMI over the course of early childhood, which may indicate a need for continued close nutrition follow-up and support even beyond the duration of tube feeds.

Asunto(s)

Nutrición Enteral , Cardiopatías Congénitas , Niño , Adolescente , Humanos , Preescolar , Lactante , Estudios Retrospectivos , Índice de Masa Corporal , Estado Nutricional , Cardiopatías Congénitas/cirugía

RESUMEN

BACKGROUND: In hypoplastic left heart syndrome, tricuspid regurgitation (TR) is associated with circulatory failure and death. We hypothesized that the tricuspid valve (TV) structure of patients with hypoplastic left heart syndrome with a Fontan circulation and moderate or greater TR differs from those with mild or less TR, and that right ventricle volume is associated with TV structure and dysfunction. METHODS: TV of 100 patients with hypoplastic left heart syndrome and a Fontan circulation were modeled using transthoracic 3-dimensional echocardiograms and custom software in SlicerHeart. Associations of TV structure to TR grade and right ventricle function and volume were investigated. Shape parameterization and analysis was used to calculate the mean shape of the TV leaflets, their principal modes of variation, and to characterize associations of TV leaflet shape to TR. RESULTS: In univariate modeling, patients with moderate or greater TR had larger TV annular diameters and area, greater annular distance between the anteroseptal commissure and anteroposterior commissure, greater leaflet billow volume, and more laterally directed anterior papillary muscle angles compared to valves with mild or less TR (all P<0.001). In multivariate modeling greater total billow volume, lower anterior papillary muscle angle, and greater distance between the anteroposterior commissure and anteroseptal commissure were associated with moderate or greater TR (P<0.001, C statistic=0.85). Larger right ventricle volumes were associated with moderate or greater TR (P<0.001). TV shape analysis revealed structural features associated with TR, but also highly heterogeneous TV leaflet structure. CONCLUSIONS: Moderate or greater TR in patients with hypoplastic left heart syndrome with a Fontan circulation is associated with greater leaflet billow volume, a more laterally directed anterior papillary muscle angle, and greater annular distance between the anteroseptal commissure and anteroposterior commissure. However, there is significant heterogeneity of structure in the TV leaflets in regurgitant valves. Given this variability, an image-informed patient-specific approach to surgical planning may be needed to achieve optimal outcomes in this vulnerable and challenging population.

Asunto(s)

Procedimiento de Fontan , Síndrome del Corazón Izquierdo Hipoplásico , Insuficiencia de la Válvula Tricúspide , Humanos , Válvula Tricúspide/diagnóstico por imagen , Válvula Tricúspide/cirugía , Procedimiento de Fontan/efectos adversos , Ventrículos Cardíacos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico por imagen , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Síndrome del Corazón Izquierdo Hipoplásico/complicaciones , Insuficiencia de la Válvula Tricúspide/diagnóstico por imagen , Insuficiencia de la Válvula Tricúspide/etiología , Insuficiencia de la Válvula Tricúspide/cirugía , Estudios Retrospectivos

RESUMEN

OBJECTIVES: To test whether youth with Down syndrome have aortic stiffness indices, as measured by pulse wave velocity (PWV), that differ from youth without Down syndrome and to compare reference-based age-adjusted (age-PWV-Z) and height-adjusted (Ht-PWV-Z) in youth with and without Down syndrome. STUDY DESIGN: Cross-sectional study of PWV in 129 adolescents with Down syndrome and 97 youth of comparable age, sex, race/ethnicity, and body mass index (BMI). PWV, age-PWV-Z, and Ht-PWV-Z were compared. Regression models were developed to test for associations with PWV. RESULTS: Youth with Down syndrome and controls were comparable in BMI-Z (1.4 [-1.5 to 2.8] vs 1.2 [-2.0 to 2.8], P = .57) but not Ht-Z (-2.3 [-4.7 to 0.8] vs 0.4 [-2.0 to 2.6], P < .0001). PWV (m/s, 5.0 [3.1-7.9] vs 5.0 [3.6-8.0], P = .5) and mean arterial pressure (MAP, mm Hg) (78 [61-102] vs 74 [64-97], P = .09) were not different between groups. In adjusted analyses confined to Down syndrome, PWV was associated only with BMI, but not age, black race, or MAP (R2 = 0.11). In contrast, BMI, age, black race, and MAP were all positively associated with and better explained PWV in controls (R2 = 0.50). PWV was not associated with height in youth with or without Down syndrome. Although age-PWV-Z was not different in Down syndrome (-0.36 [-2.93 to 3.49]) vs -0.15 [-2.32 to 3.22]), Ht-PWV-Z was greater in Down syndrome (0.32 [-2.28 to 4.07] vs -0.08 [-2.64 to 2.64], P = .002), and Ht-PWV-Z was greater than age-PWV-Z in Down syndrome (P < .0001). CONCLUSIONS: The lack of relationship of PWV, an independent predictor of adult cardiovascular events, with its traditional determinants including MAP suggests Down syndrome-specific phenomena may alter such relationships in this population. In youth with Down syndrome, Ht-adjusted PWV may overestimate aortic stiffness. TRIAL REGISTRATION: Clinicaltrials.gov: NCT01821300.

Asunto(s)

Síndrome de Down/fisiopatología , Rigidez Vascular , Adolescente , Adulto , Estudios de Casos y Controles , Niño , Estudios Transversales , Femenino , Humanos , Masculino , Análisis de la Onda del Pulso/métodos , Adulto Joven

RESUMEN

BACKGROUND: Intramural ventricular septal defects (VSDs), residual interventricular communications occurring after repair of conotruncal defects, are associated with poor postoperative outcomes. The ability of intraoperative transesophageal echocardiography (TEE) to identify intramural VSDs has not yet been evaluated. METHODS: Intraoperative TEE and postoperative transthoracic echocardiography (TTE) data in all patients undergoing all biventricular repair of conotruncal anomalies in our hospital between January 1, 2006, and June 30, 2013, were reviewed. The ability of TEE to accurately identify residual defects was assessed using postoperative TTE as the reference imaging modality. RESULTS: Intramural VSDs occurred in 34 of 337 patients evaluated; 19 were identified by both TTE and TEE, and 15 were identified by TTE only. Sensitivity was 56% and specificity was 100% for TEE to identify intramural VSDs. Peripatch VSDs were identified in 90 patients by both TTE and TEE, in 53 by TTE only, and in 15 by TEE only, yielding a sensitivity of 63% and specificity of 92%. Of the VSDs requiring catheterization or surgical reintervention, 6 of 7 intramural VSDs and all 5 peripatch VSDs were identified by intraoperative TEE. TEE guided the intraoperative decision to return to cardiopulmonary bypass (CPB) in an attempt to close residual defects in 12 patients with intramural VSDs and in 4 patients with peripatch VSDs seen after initial CPB; of these, 10 intramural VSDs and all 4 peripatch VSDs resolved or became smaller on final intraoperative TEE. CONCLUSIONS: TEE has modest sensitivity but high specificity for identifying intramural VSDs and can detect most defects requiring reintervention. Repeat attempts at closure in the index operation may successfully correct intramural VSDs identified by TEE.

Asunto(s)

Ecocardiografía Transesofágica , Cardiopatías Congénitas/cirugía , Defectos del Tabique Interventricular/diagnóstico por imagen , Complicaciones Posoperatorias/diagnóstico por imagen , Puente Cardiopulmonar , Ecocardiografía , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Reoperación , Estudios Retrospectivos , Sensibilidad y Especificidad

RESUMEN

OBJECTIVE: Bedside ultrasound for hemodynamic evaluation in critically ill children is increasingly recognized as an important skill for pediatric critical care medicine providers. Our institution implemented a training curriculum leading to institutional credentialing for pediatric critical care providers in nonprocedural bedside ultrasound core applications. We hypothesized that hemodynamic studies performed or supervised by credentialed providers (credentialed providers group) have better image quality and greater accuracy in interpretation than studies performed by non-credentialed providers without supervision (non-credentialed providers group). DESIGN: Retrospective descriptive study. SETTING: Single-center tertiary non-cardiac 55-bed PICU in a children's hospital. PATIENTS: Patients from October 2013 to January 2015, with hemodynamic bedside ultrasound performed and interpreted by pediatric critical care providers exposed to bedside ultrasound training. INTERVENTIONS: A cardiologist blinded to performer scored hemodynamic bedside ultrasound image quality for five core cardiac views (excellent = 3, good = 2, fair = 1, unacceptable = 0; median = quality score) and interpretation within 5 hemodynamic domains (agreement = 3, minor disagreement = 2, major disagreement = 1; median = interpretation score), as well as a global assessment of interpretation. MEASUREMENTS AND MAIN RESULTS: Eighty-one studies (45 in the credentialed providers group and 36 in the non-credentialed providers group) were evaluated. There was no statistically significant difference in quality score between groups (median: 1.4 [interquartile range: 0.8-1.8] vs median: 1.2 [interquartile range: 0.75-1.6]; p = 0.14]. Studies in the credentialed providers group had higher interpretation score than those in the non-credentialed providers group (median: 3 [interquartile range: 2.5-3) vs median: 2.67 [interquartile range: 2.25-3]; p = 0.04). Major disagreement between critical care provider and cardiology review occurred in 25 of 283 hemodynamic domains assessed (8.8%), with no statistically significant difference between credentialed providers and non-credentialed providers groups (6.1% vs 11.9%; p = 0.12). CONCLUSION: Hemodynamic bedside ultrasound performed or supervised by credentialed pediatric critical care providers had more accurate interpretation than studies performed by unsupervised non-credentialed providers. A rigorous pediatric critical care medicine bedside ultrasound credentialing program can train intensivists to attain adequate images and interpret those images appropriately.

Asunto(s)

Cuidados Críticos/métodos , Curriculum , Educación de Postgrado en Medicina/métodos , Hemodinámica , Pediatría/educación , Sistemas de Atención de Punto , Ultrasonografía , Adolescente , Niño , Preescolar , Competencia Clínica , Femenino , Humanos , Lactante , Recién Nacido , Unidades de Cuidado Intensivo Pediátrico , Masculino , Philadelphia , Estudios Retrospectivos , Adulto Joven

RESUMEN

BACKGROUND: Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies. We assessed the prevalence of residual intramural VSDs and their effect on postoperative course. METHODS AND RESULTS: Children who underwent biventricular repair of a conotruncal anomaly from January 1, 2006, to June 30, 2013, and had a postoperative transthoracic echocardiogram were included. Images were reviewed for residual intramural or nonintramural VSDs. The primary outcome was a composite of mortality, extracorporeal membrane oxygenation use, and need for subsequent catheter or surgical VSD closure. The secondary outcome was postoperative hospital length of stay. A residual VSD was present in 256 of the 442 subjects (58%), of which 231 (90%) were <2 mm in size. Forty-nine patients (11%) had intramural VSDs, and 207 (47%) had nonintramural VSDs. Patients with intramural VSDs were more likely to reach the primary composite outcome compared with those with nonintramural VSDs or no residual VSD (14 of 49 [29%] versus 15 of 207 [7%] versus 6 of 186 [3%]; P<0.0001). In addition, those with intramural VSDs had longer postoperative hospital length of stay compared with those with nonintramural VSDs or no residual VSD (20 days [interquartile range, 11-42 days] versus 7 days [interquartile range, 5-14 days] versus 6 days [interquartile range, 4-11 days]; P=0.0001). These associations remained significant after adjustment for known risk factors for poor outcomes, including residual VSD size and operative complexity. CONCLUSIONS: Among residual VSDs after repair of conotruncal anomalies, intramural VSDs are uniquely associated with postoperative morbidity, mortality, and longer postoperative hospital length of stay. It is important to recognize intramural VSDs in the postoperative period.

Asunto(s)

Cardiopatías Congénitas/cirugía , Defectos del Tabique Interventricular/etiología , Complicaciones Posoperatorias/etiología , Tronco Arterial/anomalías , Cateterismo Cardíaco/estadística & datos numéricos , Oxigenación por Membrana Extracorpórea/estadística & datos numéricos , Femenino , Defectos del Tabique Interventricular/diagnóstico por imagen , Defectos del Tabique Interventricular/epidemiología , Defectos del Tabique Interventricular/cirugía , Tabiques Cardíacos/diagnóstico por imagen , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro , Enfermedades del Prematuro/epidemiología , Enfermedades del Prematuro/cirugía , Tiempo de Internación/estadística & datos numéricos , Masculino , Complicaciones Posoperatorias/diagnóstico por imagen , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/cirugía , Prevalencia , Reoperación/estadística & datos numéricos , Factores de Riesgo , Resultado del Tratamiento , Tronco Arterial/cirugía , Ultrasonografía

RESUMEN

OBJECTIVES: To determine the cardiovascular effects of obesity on patients with tetralogy of Fallot (TOF) repair. STUDY DESIGN: Ventricular performance measures were compared between obese (body mass index [BMI] ≥95%), overweight (85% ≤BMI <95%), and normal weight subjects (BMI <85%) in a retrospective review of patients with TOF who underwent cardiac magnetic resonance from 2005-2010. Significance was P < .05. RESULTS: Of 260 consecutive patients with TOF, 32 were obese (12.3%), 48 were overweight (18.5%), and 180 were normal weight (69.2%). Biventricular mass was increased in obese compared with normal weight patients with right ventricular mass more affected than left ventricular mass. Obese patients demonstrated decreased biventricular end-diastolic volume (EDV) and stroke volume (SV) when indexed to body surface area (BSA) with an increased heart rate when compared with normal weight patients; cardiac index, ejection fraction, and pulmonary regurgitation fraction were similar. When indexed to ideal BSA, biventricular EDV and SV were similar. EDV and SV for overweight patients were nearly identical to normal weight patients with ventricular mass in between the other 2 groups. CONCLUSIONS: Approximately 12% of patients after TOF repair referred for cardiac magnetic resonance in a tertiary referral center are obese with increased biventricular mass. Obese patients and normal weight patients have similar cardiac indices, however, when indexed to actual BSA, obese patients demonstrate decreased EDV and SV with increased heart rate and similar cardiac indices. When indexed to ideal BSA, no differences in biventricular volumes were noted.

Asunto(s)

Frecuencia Cardíaca/fisiología , Ventrículos Cardíacos/patología , Ventrículos Cardíacos/fisiopatología , Obesidad/complicaciones , Volumen Sistólico/fisiología , Tetralogía de Fallot/cirugía , Adolescente , Índice de Masa Corporal , Niño , Preescolar , Femenino , Humanos , Imagen por Resonancia Cinemagnética , Masculino , Obesidad/epidemiología , Obesidad/fisiopatología , Prevalencia , Estudios Retrospectivos , Tetralogía de Fallot/complicaciones , Tetralogía de Fallot/fisiopatología , Función Ventricular/fisiología

RESUMEN

OBJECTIVE: An apparent life-threatening event (ALTE) is a common diagnosis in pediatrics, but there is no standardized method to evaluate these patients. We sought to determine the prevalence of electrocardiogram (ECG) use in patients presenting to children's hospitals with an ALTE. METHODS: The data from the Pediatric Health Information System database from 43 children's hospitals were collected during a 15-month period between October 2009 and December 2010. Patients were included if they were younger than 1 year at the time of presentation. Demographic data, including age, length of hospital stay, second ALTE, and survival, were recorded, along with the prevalence of ECGs and International Classification of Diseases, Ninth Revision, cardiac diagnoses. RESULTS: There were 2179 patients with an ALTE, with a mean age of 65.7 days old and length of stay of 3.4 days. A total of 947 (43%) of these patients received an ECG. The prevalence of ECG use and cardiac diagnoses were variable among the participating hospitals. Depending on the institution, 0% to 93% of patients had an ECG, and 4% to 39% of patients had an International Classification of Diseases, Ninth Revision, cardiac diagnosis. CONCLUSIONS: Electrocardiograms are performed in fewer than half of patients with ALTE presenting to children's hospitals. There is wide variation in the prevalence of ECG use as a diagnostic tool for infants presenting with an ALTE.

Asunto(s)

Enfermedades Cardiovasculares/diagnóstico , Electrocardiografía/estadística & datos numéricos , Hospitales Pediátricos/estadística & datos numéricos , Enfermedades Cardiovasculares/epidemiología , Niño , Niño Hospitalizado , Enfermedad Crítica , Bases de Datos Factuales , Femenino , Humanos , Lactante , Clasificación Internacional de Enfermedades , Tiempo de Internación , Masculino , Pediatría , Prevalencia , Estados Unidos

RESUMEN

BACKGROUND: Abnormal height and adiposity are observed after the Fontan operation. These abnormalities may be associated with worse functional outcome. METHODS: We analyzed data from the National Heart, Lung, and Blood Institute Pediatric Heart Network cross-sectional study of Fontan patients. Groups were defined by height (z-score<-1.5 or≥-1.5) and body mass index (body mass index [BMI] z-score<-1.5 or -1.5 to 1.5 or≥1.5). Associations of anthropometric measures with measurements from clinical testing (exercise, echocardiography, magnetic resonance imaging) were determined adjusting for demographics, anatomy, and pre-Fontan status. Relationships between anthropometric measures and functional health status (FHS) were assessed using the Child Health Questionnaire. RESULTS: Mean age of the cohort (n=544) was 11.9±3.4 years. Lower height-z patients (n=124, 23%) were more likely to have pre-Fontan atrioventricular valve regurgitation (P=.029), as well as orthopedic and developmental problems (both P<.001). Lower height-z patients also had lower physical and psychosocial FHS summary scores (both P<.01). Higher BMI-z patients (n=45, 8%) and lower BMI-z patients (n=53, 10%) did not have worse FHS compared to midrange BMI-z patients (n=446, 82%). However, higher BMI-z patients had higher ventricular mass-to-volume ratio (P=.03) and lower % predicted maximum work (P=.004) compared to midrange and lower BMI-z patients. CONCLUSIONS: Abnormal anthropometry is common in Fontan patients. Shorter stature is associated with poorer FHS and non-cardiac problems. Increased adiposity is associated with more ventricular hypertrophy and poorer exercise performance, which may have significant long-term implications in this at-risk population.

Asunto(s)

Estatura , Peso Corporal , Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Hipertrofia Ventricular Izquierda/etiología , Sobrepeso/complicaciones , Niño , Estudios Transversales , Progresión de la Enfermedad , Femenino , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/fisiopatología , Humanos , Hipertrofia Ventricular Izquierda/diagnóstico , Hipertrofia Ventricular Izquierda/fisiopatología , Imagen por Resonancia Magnética , Masculino , Sobrepeso/fisiopatología , Periodo Posoperatorio , Factores de Riesgo , Encuestas y Cuestionarios

RESUMEN

OBJECTIVE: We sought to assess outcome in patients with CDH and HD to determine if LHR is also predictive of outcome in this subset of patients. STUDY DESIGN: We carried out a retrospective review (April 1996-October 2000) of patients with isolated CDH (n = 143, 82.2%) and patients with HD (n = 31, 17.8%) to determine the incidence of additional anomalies, survival to term, CDH repair, cardiac repair, and survival to discharge. Survival based on LHR was analyzed in a subset of fetuses. RESULTS: The risk of death from birth to last follow-up was 2.9 times higher for patients with CDH plus HD than for patients with CDH alone (P <.0001). Of 11 patients with CDH plus HD who had CDH repair (5 of whom also had HD repair), 5 survived. All 10 patients with an LHR <1.2 died; 3 of 6 with an LHR >1.2 survived (Fisher exact test, P =.04). CONCLUSION: Heart disease remains a significant risk factor for death in infants with CDH. The LHR helps predict survival in this high-risk group of patients.

Asunto(s)

Anomalías Múltiples/mortalidad , Cardiopatías Congénitas , Hernia Diafragmática/mortalidad , Hernias Diafragmáticas Congénitas , Femenino , Hernia Diafragmática/diagnóstico por imagen , Humanos , Recién Nacido , Pulmón/patología , Pennsylvania/epidemiología , Embarazo , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Riesgo , Tasa de Supervivencia , Ultrasonografía Prenatal

RESUMEN

Left ventricular hypoplasia is associated with a variety of congenital heart defects, including critical aortic stenosis, unbalanced atrioventricular canal, and total anomalous pulmonary venous connection, and is almost uniformly fatal without surgical or catheter-directed intervention. Accurately determining whether the left ventricle can adequately support the systemic circulation can be challenging and may be approached in a variety of ways, depending on the cardiac defect. The decision is more difficult in the present era of pediatric cardiology and cardiothoracic surgery because other options, such as the Norwood procedure and cardiac transplantation, are available to infants with left ventricular hypoplasia with improving survival. This report is a review of the present understanding of left ventricular hypoplasia and gives suggestions about how to stratify these complex patients to single versus two-ventricle repair. Copyright 1999 by W.B. Saunders Company