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BACKGROUND: Patients with hepatoblastoma (HB) have a higher risk of congenital heart defects (CHD). There is limited literature on the management and outcomes of these patients. The purpose of this study was to identify demographics and outcomes of these patients in a single tertiary referral center. METHODS: An Institutional Review Board (IRB)-approved retrospective chart review of patients with newly diagnosed HB from October 2004 to January 2021 was performed. CHD was defined as the presence of a septal defect, patent ductus arteriosus, pulmonary atresia, or bicuspid aortic valve. Chi-square and t-test were utilized for statistical analyses. RESULTS: Of the 151 patients diagnosed with HB during the study timeframe, 29 patients were found to have CHD. Five-year overall survival (OS) for non-CHD HB patients was 81.9% compared to 68.9% in the CHD cohort (p = .12). The 5-year OS for patients without surgically intervened CHD was 63.6% compared to 70.5% for those with surgically repaired CHD (p = .88). Pre-treatment extent of tumor IV was present more often in patients with HB and CHD who passed away (6/9, 66.7%) compared to those who survived (3/16,18.8%, p = .01). CONCLUSIONS: Patients with HB and CHD have similar survival compared to those without CHD. Our data support that patients with HB and CHD should be treated with curative intent including cardiac surgical intervention, medical oncology therapy, and oncological surgery for their HB.
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BACKGROUND: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with congenital heart disease (CHD). Little is known about the adult HS population with CHD. OBJECTIVE: To describe the outcomes and sociodemographics of the adult CHD population with HS. METHODS: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed. RESULTS: Sixty-two patients met inclusion criteria. Median age was 22.7 [IQR 19.6-30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap in care of >3 years. Median follow-up time in adulthood was 2.9 [IQR 1.3-8.2] years. Forty-three (69%) of patients had single ventricle heart disease, 31 (71%) of whom completed Fontan circulation. A total of 36 interventions occurred in 24 patients which included 16 cardiac catherization interventions, 13 electrophysiology-related procedures, and 18 surgical procedures including 2 orthotopic heart transplants. The median age for death or heart transplant was 45.3 (95%CI 34.3-56.1) years. Heart failure-free survival was 80.8 ± 5.2%, 58.7 ± 11.0%, and 31.1 ± 15.7% at 20, 30, and 40 years old, respectively. Cerebrovascular accident-free survival was 84.3 ± 5.1%, 54.2 ± 11.3%, and 40.6 ± 14.5% at 20, 30, and 40 years old, respectively. Tachyarrhythmia-free survival was 54.0 ± 7.1%, 29.2 ± 8.3%, and 19.5 ± 9.7% at 20, 30, and 40 years old and bradyarrhythmia-free survival was 66.0 ± 6.3%, 41.7 ± 9.4%, and 33.4 ± 10.6% at ages 20, 30, and 40 years, respectively. CONCLUSIONS: At a tertiary referral center, adult patients with CHD and HS have high rates of comorbidities and early death or heart transplant. Longitudinal surveillance and further exploration into factors associated with improved survival in this population are warranted.
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Cateterismo Cardíaco , Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas/terapia , Síndrome de Heterotaxia/complicaciones , Sobrevivientes , Adulto , Factores de Edad , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/mortalidad , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/mortalidad , Causas de Muerte , Comorbilidad , Progresión de la Enfermedad , Estado de Salud , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/mortalidad , Síndrome de Heterotaxia/diagnóstico por imagen , Síndrome de Heterotaxia/mortalidad , Humanos , Persona de Mediana Edad , Supervivencia sin Progresión , Estudios Retrospectivos , Factores de Riesgo , Texas , Factores de Tiempo , Adulto JovenRESUMEN
BACKGROUND: The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure. METHODS: All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both). RESULTS: The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival. CONCLUSIONS: Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.
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Procedimiento de Fontan , Adolescente , Adulto , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/epidemiología , Reoperación , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Insuficiencia del Tratamiento , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: There are limited studies analyzing pulsatile Glenn as a long-term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution. DESIGN: A retrospective review was performed. SETTING: Study performed at a single pediatric hospital. PATIENTS: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included. OUTCOME MEASURES: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk factors were assessed by Cox multivariable competing risk analyses. RESULTS: Seventy-eight patients underwent pulsatile Glenn at age 9 months (interquartile range, 5-14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time-to-palliation of 4 years (interquartile range, 3-5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five- and 10-year transplant-free survival were 91% and 84%, respectively. At a median follow-up of 6 years (interquartile range, 2-8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3-11], oxygen saturation 83% ± 4%). Preoperative moderate-severe atrioventricular valve regurgitation (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80-33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08-6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for covariates. Reaching further palliation was less likely in patients with preoperative moderate-severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08-0.59; P =.002). CONCLUSION: Pulsatile Glenn can be an effective tool to be used in challenging circumstances, these patients can have a favorable long-term prognosis without reducing their suitability for further palliation.
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Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Cuidados Paliativos/métodos , Complicaciones Posoperatorias/epidemiología , Cateterismo Cardíaco , Ecocardiografía , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Humanos , Incidencia , Lactante , Masculino , Estudios Retrospectivos , Factores de Riesgo , Texas/epidemiología , Resultado del TratamientoRESUMEN
BACKGROUND: Heterotaxy syndrome (HS) is a rare disorder with complex anatomy involving misarrangements of the cardiac conduction system. Arrhythmias may be related to anatomic variations and contribute to morbidity. OBJECTIVE: The purpose of this study was to investigate the associations between arrhythmias, anatomy, and outcomes in a large HS cohort. METHODS: A single-center retrospective review of patients ≤21 years of age diagnosed with HS was performed. RESULTS: A total of 337 patients were included in the study. During median follow-up of 7 years (interquartile range 2-16 years), 129 patients (38%) had ≥1 clinically significant rhythm disturbance: tachyarrhythmias in 75 (22%), bradyarrhythmias in 29 (9%), and both in 25 (7%). Factors associated with tachyarrhythmia by multivariate analysis were at least moderate atrioventricular valve regurgitation (hazard ratio [HR] 1.66; 95% confidence interval [CI] 1.11-2.50), single ventricle anatomy (HR 2.30; 95% CI 1.09-4.85), and pulmonary venous obstruction (HR 2.33; 95% CI 1.45-3.76). Isomerism subtype was not associated with tachyarrhythmias. In adjusted and unadjusted analyses, bradyarrhythmias (symptomatic sinus/atrial bradycardia and high-grade or complete heart block) were associated with left atrial isomerism (LAI) compared to right atrial isomerism (HR 7.12; 95% CI 3.01-16.9). The overall transplant-free survival of the cohort was 66%. Tachyarrhythmias, but not bradyarrhythmias, were associated with mortality or need for transplant (HR 2.24; 95% CI 1.45-3.46). CONCLUSION: Clinically significant arrhythmias are common in HS. Although bradyarrhythmias are associated with LAI, tachyarrhythmia occurrence may depend more on hemodynamic and anatomic factors than isomerism subtype. Tachyarrhythmias, but not bradyarrhythmias, are associated with death or need for transplant.
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Arritmias Cardíacas/etiología , Electrocardiografía , Sistema de Conducción Cardíaco/fisiopatología , Síndrome de Heterotaxia/complicaciones , Adolescente , Edad de Inicio , Arritmias Cardíacas/epidemiología , Arritmias Cardíacas/fisiopatología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Lactante , Masculino , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Texas/epidemiología , Factores de Tiempo , Adulto JovenRESUMEN
BACKGROUND: There is disagreement over the management of malrotation in children with heterotaxy and congenital heart disease (CHD). We sought to evaluate the outcomes of management with a Ladd procedure compared to observation in this cohort of patients. METHODS: We performed a retrospective review of CHD children with heterotaxy and malrotation identified on radiographs treated from 8/2002 until 4/2014. Primary outcomes evaluated were readmission for small bowel obstruction (SBO) or volvulus. RESULTS: We identified 88 patients with cardiac heterotaxy and malrotation. Sixty-eight (77%) had a Ladd procedure. Eighteen (26%) of the 68 had abdominal symptoms, but only one had an underlying volvulus without ischemia. Twenty (23%) patients died of cardiopulmonary complications, 8 before and 12 after the Ladd procedure. Sixty-eight patients survived to the review date (median: 5years): 56 in the Ladd cohort and 12 observed. Six of the 56 (11%) surviving Ladd patients were readmitted to hospital with an SBO, and 2 required surgical intervention. None of the 12 surviving nonoperative patients developed a volvulus. CONCLUSION: Eleven percent of patients developed SBO after their Ladd procedure. Conversely, no observed patients developed a volvulus. This suggests that complications from a Ladd procedure occur with higher frequency than complications from observing heterotaxy patients with malrotation.
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Síndrome de Heterotaxia/complicaciones , Síndrome de Heterotaxia/cirugía , Vólvulo Intestinal/complicaciones , Vólvulo Intestinal/cirugía , Procedimientos Quirúrgicos del Sistema Digestivo , Femenino , Síndrome de Heterotaxia/diagnóstico por imagen , Humanos , Recién Nacido , Obstrucción Intestinal/etiología , Vólvulo Intestinal/diagnóstico por imagen , Intestino Delgado , Masculino , Radiografía , Estudios RetrospectivosRESUMEN
BACKGROUND: Heterotaxy syndrome (HTX) is a constellation of defects including abnormal organ lateralization and often including congenital heart defects. HTX has widely divergent population-based estimates of prevalence, racial and ethnic predominance, and mortality in current literature. METHODS: The objective of this study was to use a population-based registry to investigate potential racial and ethnic disparities in HTX. Using the Texas Birth Defects Registry, we described clinical features and mortality of HTX among infants delivered from 1999 to 2006. We calculated birth prevalence and crude prevalence (cPR) ratios for infant sex, maternal diabetes, and sociodemographic factors. RESULTS: A total of 353 HTX cases were identified from 2,993,604 births (prevalence ratio = 1.18 per 10,000 live births. HTX prevalence was approximately 70% higher among infants of Hispanic and non-Hispanic black mothers and 28% higher among female infants (cPR = 1.28; 95% confidence interval,1.04-1.59). There was a twofold higher female preponderance for infants of mothers who were non-Hispanic white or black. Mothers with diabetes were three times more likely to have a child with HTX compared with nondiabetics (cPR = 3.13; 95% confidence interval, 2.12-4.45). Among nondiabetics, HTX cases were 86% more likely to have a Hispanic mother and 72% a non-Hispanic black mother. First-year mortality for live born children with HTX was 30.9%. CONCLUSION: This study represents one of the largest population-based studies of HTX to date, with a novel finding of higher rates of HTX among Hispanic infants of mostly Mexican origin, as well as among female infants of only non-Hispanic white and black mothers. These findings warrant further investigation.
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Etnicidad/estadística & datos numéricos , Disparidades en el Estado de Salud , Síndrome de Heterotaxia/epidemiología , Grupos Raciales/estadística & datos numéricos , Sistema de Registros/estadística & datos numéricos , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Madres , Prevalencia , Texas/epidemiologíaRESUMEN
BACKGROUND: Adverse neurodevelopmental outcomes are observed in up to 50% of infants after complex cardiac surgery. We sought to determine the association of perioperative anesthetic exposure with neurodevelopmental outcomes at age 12 months in neonates undergoing complex cardiac surgery and to determine the effect of brain injury determined by magnetic resonance imaging (MRI). METHODS: Retrospective cohort study of neonates undergoing complex cardiac surgery who had preoperative and 7-day postoperative brain MRI and 12-month neurodevelopmental testing with Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III). Doses of volatile anesthetics (VAA), benzodiazepines, and opioids were determined during the first 12 months of life. RESULTS: From a database of 97 infants, 59 met inclusion criteria. Mean ± sd composite standard scores were as follows: cognitive = 102.1 ± 13.3, language = 87.8 ± 12.5, and motor = 89.6 ± 14.1. After forward stepwise multivariable analysis, new postoperative MRI injury (P = 0.039) and higher VAA exposure (P = 0.028) were associated with lower cognitive scores. ICU length of stay (independent of brain injury) was associated with lower performance on all categories of the Bayley-III (P < 0.02). CONCLUSIONS: After adjustment for multiple relevant covariates, we demonstrated an association between VAA exposure, brain injury, ICU length of stay, and lower neurodevelopmental outcome scores at 12 months of age. These findings support the need for further studies to identify potential modifiable factors in the perioperative care of neonates with CHD to improve neurodevelopmental outcomes.
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Anestésicos/efectos adversos , Encefalopatías/inducido químicamente , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Discapacidades del Desarrollo/inducido químicamente , Sistema Nervioso/crecimiento & desarrollo , Anestésicos/administración & dosificación , Encéfalo/patología , Encefalopatías/patología , Encefalopatías/psicología , Puente Cardiopulmonar , Estudios de Cohortes , Discapacidades del Desarrollo/epidemiología , Discapacidades del Desarrollo/fisiopatología , Femenino , Cardiopatías Congénitas/psicología , Humanos , Hipnóticos y Sedantes/administración & dosificación , Hipnóticos y Sedantes/efectos adversos , Lactante , Recién Nacido , Trastornos del Desarrollo del Lenguaje/inducido químicamente , Trastornos del Desarrollo del Lenguaje/epidemiología , Imagen por Resonancia Magnética , Masculino , Sistema Nervioso/efectos de los fármacos , Pruebas Neuropsicológicas , Periodo Perioperatorio , Estudios RetrospectivosRESUMEN
BACKGROUND: In this study we report magnetic resonance imaging (MRI) brain injury and 12-month neurodevelopmental outcomes when regional cerebral perfusion (RCP) is used for neonatal aortic arch reconstruction. METHODS: Fifty-seven neonates receiving RCP during aortic arch reconstruction were enrolled in a prospective outcome study. RCP flows were determined by near-infrared spectroscopy and transcranial Doppler monitoring. Brain MRI was performed preoperatively and 7 days postoperatively. Bayley Scales of Infant Development III was performed at 12 months. RESULTS: Mean RCP time was 71 ± 28 minutes (range, 5 to 121 minutes) and mean flow was 56.6 ± 10.6 mL/kg/min. New postoperative MRI brain injury was seen in 40% of patients. For 35 RCP patients at age 12 months, mean Bayley Scales III Composite standard scores were: Cognitive, 100.1 ± 14.6 (range, 75 to 125); Language, 87.2 ± 15.0 (range, 62 to 132); and Motor, 87.9 ± 16.8 (range, 58 to 121). Increasing duration of RCP was not associated with adverse neurodevelopmental outcomes. CONCLUSIONS: Neonatal aortic arch repair with RCP using a neuromonitoring strategy results in 12-month cognitive outcomes that are at reference population norms. Language and motor outcomes are lower than the reference population norms by 0.8 to 0.9 standard deviations. The neurodevelopmental outcomes in this RCP cohort demonstrate that this technique is effective and safe in supporting the brain during neonatal aortic arch reconstruction.
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Aorta Torácica/cirugía , Procedimientos Quirúrgicos Cardíacos , Circulación Cerebrovascular , Monitoreo Intraoperatorio/métodos , Sistema Nervioso/crecimiento & desarrollo , Perfusión , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/métodos , Humanos , Recién Nacido , Estudios Prospectivos , Espectroscopía Infrarroja Corta , Resultado del Tratamiento , Ultrasonografía Doppler TranscranealRESUMEN
OBJECTIVES: Neonates undergoing complex congenital heart surgery have a significant incidence of neurologic problems. Erythropoietin has antiapoptotic, antiexcitatory, and anti-inflammatory properties to prevent neuronal cell death in animal models, and improves neurodevelopmental outcomes in full-term neonates with hypoxic ischemic encephalopathy. We designed a prospective phase I/II trial of erythropoietin neuroprotection in neonatal cardiac surgery to assess safety and indicate efficacy. METHODS: Neonates undergoing surgery for D-transposition of the great vessels, hypoplastic left heart syndrome, or aortic arch reconstruction were randomized to 3 perioperative doses of erythropoietin or placebo. Neurodevelopmental testing using the Bayley Scales of Infant and Toddler Development III was performed at age 12 months. RESULTS: Fifty-nine patients received the study drug. Safety profile, including magnetic resonance imaging brain injury, clinical events, and death, was not different between groups. Three patients in each group died. Forty-two patients (22 in the erythropoietin group and 20 in the placebo group; 79% of survivors) returned for 12-month follow-up. In the group receiving erythropoietin, mean Cognitive Scale scores were 101.1 ± 13.6, Language Scale scores were 88.5 ± 12.8, and Motor Scale scores were 89.9 ± 12.3. In the group receiving placebo, Cognitive Scale scores were 106.3 ± 10.8 (P = .19), Language Scores were 92.4 ± 12.4 (P = .33), and Motor Scale scores were 92.6 ± 14.1 (P = .51). CONCLUSIONS: Safety profile for erythropoietin administration was not different than placebo. Neurodevelopmental outcomes were not different between groups; however, this pilot study was not powered to definitively address this outcome. Lessons learned suggest optimized study design features for a larger prospective trial to definitively address the utility of erythropoietin for neuroprotection in this population.
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Procedimientos Quirúrgicos Cardíacos , Eritropoyetina/uso terapéutico , Cardiopatías Congénitas/cirugía , Enfermedades del Sistema Nervioso/prevención & control , Fármacos Neuroprotectores/uso terapéutico , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Humanos , Lactante , Recién Nacido , Enfermedades del Sistema Nervioso/etiología , Estudios Prospectivos , Método Simple CiegoRESUMEN
BACKGROUND: Expectations for outcomes after the neonatal arterial switch operation (ASO) continue to change. This cohort study describes neurodevelopmental outcomes at age 12 months after neonatal ASO, and analyzes both modifiable and nonmodifiable factors for association with adverse outcomes. METHODS: Patients who underwent an ASO (n=30) were enrolled in a prospective outcome study, with comprehensive clinical data collection during the first 12 months of life. Brain magnetic resonance imaging was done preoperatively and 7 days postoperatively, and the Bayley Scales of Infant Development III was performed at age 12 months. RESULTS: Ten of 30 patients (33%) had preoperative magnetic resonance imaging injury; 13 of 30 patients (43%) had new postoperative magnetic resonance imaging injury. Twenty patients (67%) had Bayley Scales of Infant Development III: Cognitive Composite standard score mean was 104.8±15.0, Language Composite standard score median was 90.0 (25th to 75th percentile, 83 to 94), and Motor Composite standard score mean was 92.3±14.2. Best subsets multivariable analysis found associations between lower preoperative and intraoperative cerebral oxygen saturation, preoperative magnetic resonance imaging brain injury, total bypass time, and total midazolam dose and lower Bayley Scales of Infant Development III scores at age 12 months. CONCLUSIONS: At 12 months after ASO, neurodevelopmental outcome means were within normal population ranges. The new associations reported in this study between potentially modifiable perioperative factors and outcomes require investigations in larger patient cohorts. Beyond survival, which was 100% in this cohort, factors influencing quality of life including neurodevelopmental outcomes should be routinely investigated in studies of ASO patients.
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Procedimientos Quirúrgicos Cardíacos/efectos adversos , Desarrollo Infantil , Discapacidades del Desarrollo/etiología , Transposición de los Grandes Vasos/cirugía , Encéfalo/patología , Procedimientos Quirúrgicos Cardíacos/métodos , Discapacidades del Desarrollo/diagnóstico , Discapacidades del Desarrollo/epidemiología , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Lactante , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Pruebas Neuropsicológicas , Estudios Prospectivos , Factores de Riesgo , Texas/epidemiología , Resultado del TratamientoRESUMEN
BACKGROUND: Potassium supplementation is a common practice in critically ill children, especially those with heart disease. Intravenous potassium supplementation is the standard route of administration in most intensive care units. Although the enteral route is safer and thus may be a reasonable alternative, data on the efficacy of enteral potassium administration are lacking. METHODS: A change of practice to encourage use of enteral potassium was instituted in the cardiac intensive care unit at Texas Children's Hospital, and a review of this practice change was undertaken. The primary outcome of interest was the comparable efficacy of enteral and intravenous potassium administration. Patient demographic data, including urine output, diuretic use, route of potassium administration, and adverse events were documented and analyzed. RESULTS: Seventy-six patients met inclusion criteria and received 399 bolus doses of potassium (166 intravenous and 233 enteral). No patients became hyperkalemic after either route of administration. The increase in serum potassium was similar in both groups of patients. Side effects of the two routes of administration were not different. CONCLUSIONS: The efficacy of enteral potassium is comparable to intravenous potassium for potassium replacement in pediatric patients after congenital heart surgery.
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Nutrición Enteral/normas , Cardiopatías/cirugía , Unidades de Cuidado Intensivo Pediátrico , Potasio/administración & dosificación , Suplementos Dietéticos , Humanos , Pautas de la Práctica en Medicina , Texas , Resultado del TratamientoRESUMEN
BACKGROUND: Postoperative electroencephalographic (EEG) seizures are reported to occur in 14% to 20% of neonates after cardiac surgery with cardiopulmonary bypass (CPB). EEG seizures are associated with prolonged deep hypothermic circulatory arrest and with adverse long-term neurodevelopmental outcomes. We performed video/EEG monitoring before and for 72 hours after neonatal cardiac surgery, using a high-flow CPB protocol and cerebral oxygenation monitoring, to ascertain incidence, severity, and factors associated with EEG seizures. METHODS: The CPB protocol included 150 mL/kg/min flows, pH stat management, hematocrit >30%, and high-flow antegrade cerebral perfusion. Regional cerebral oxygen saturation (rSo(2)) was monitored, with a treatment protocol for rSo(2) <50%. EEG was assessed for seizures. RESULTS: Sixty-eight patients (36 single ventricle [SV] and 32 2-ventricle [2V]) were monitored for a total of 4824 hours. The total midazolam dose was 2.4 mg/kg (1.5-7.3 mg/kg) (median, 25th-75th percentile) for the SV group and 1.3 mg/kg (1.0-2.7 mg/kg) for the 2V group (P = 0.009). One SV patient experienced 2 brief EEG seizures postoperatively (1.5% incidence; 95% confidence interval: 0.3%-7.9%). The SV patients experienced a significant incidence of cerebral desaturation (rSo(2) <45% for >240 minutes total) perioperatively (18 of 36 SV vs 0 of 32 2V patients, P < 0.001). This difference did not affect electrographic seizure occurrence or other EEG characteristics. CONCLUSIONS: EEG seizures are infrequent in neonates undergoing surgery with high-flow CPB. Cerebral desaturation did not affect EEG seizure occurrence; however, benzodiazepines may play a role in suppressing postoperative seizures caused by cerebral hypoxemia in this patient population. Using this anesthetic and surgical protocol, EEG seizures are a poor surrogate marker for acute neurological injury in this population.
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Procedimientos Quirúrgicos Cardíacos/efectos adversos , Puente Cardiopulmonar/efectos adversos , Electroencefalografía , Complicaciones Posoperatorias/epidemiología , Convulsiones/epidemiología , Convulsiones/etiología , Anestesia , Anestésicos/uso terapéutico , Química Encefálica/fisiología , Circulación Cerebrovascular/fisiología , Femenino , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Humanos , Hipnóticos y Sedantes/uso terapéutico , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Oxígeno/sangre , Consumo de Oxígeno/fisiología , Dolor Postoperatorio/tratamiento farmacológico , Perfusión , Cuidados Posoperatorios , Grabación en VideoRESUMEN
OBJECTIVES: The purpose of this study was to determine the incidence of renal insufficiency in children hospitalized with acute decompensated heart failure and whether worsening renal function is associated with adverse cardiovascular outcome. DESIGN: Prospective observational cohort study. SETTING: Single-center children's hospital. PATIENTS: All pediatric patients from birth to age 21 yrs admitted to our institution with acute decompensated heart failure from October 2003 to October 2005. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Acute decompensated heart failure was defined as new-onset or acute exacerbation of heart failure signs or symptoms requiring hospitalization and inpatient treatment. We required that heart failure be attributable to ventricular dysfunction only. Worsening renal function was defined as an increase in serum creatinine by > or = 0.3 mg/dL during hospitalization. Sixty-three patients (35 male, 28 female) comprised 73 patient hospitalizations. Median age at admission was 10 yrs (range 0.1-20.3 yrs). Median serum creatinine at admission was 0.6 mg/dL (range 0.2-3.5 mg/dL), and median creatinine clearance was 103 mL/min/1.73 m2 (range 22-431 mL/min/1.73 m2). Serum creatinine increased during 60 of 73 (82%) patient hospitalizations (median increase 0.2 mg/dL, range 0.1-2.7 mg/dL), and worsening renal function occurred in 35 of 73 (48%) patient hospitalizations. Clinical variables associated with worsening renal function included admission serum creatinine (p = .009) and blood urea nitrogen (p = .04) and, during hospitalization, continuous infusions of dopamine (p = .028) or nesiritide (p = .007). Worsening renal function was independently associated with the combined end point of in-hospital death or need for mechanical circulatory support (adjusted odds ratio 10.2; 95% confidence interval 1.7-61.2, p = .011). Worsening renal function was also associated with longer observed length of stay (33 +/- 30 days vs. 18 +/- 25 days, p < .03). CONCLUSIONS: These data suggest that an important cardiorenal interaction occurs in children hospitalized for acute decompensated heart failure. Renal function commonly worsens in such patients and is associated with prolonged hospitalization and in-hospital death or the need for mechanical circulatory assistance.
Asunto(s)
Insuficiencia Cardíaca/fisiopatología , Enfermedades Renales/complicaciones , Adolescente , Adulto , Niño , Preescolar , Estudios de Cohortes , Femenino , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/terapia , Humanos , Incidencia , Lactante , Recién Nacido , Enfermedades Renales/fisiopatología , Enfermedades Renales/terapia , Pruebas de Función Renal , Masculino , Síndrome , Resultado del TratamientoRESUMEN
OBJECTIVE: Tight glycemic control improves outcomes in critically ill adults. There are limited data regarding the effect of glycemic profiles in infants after cardiac operations. The aim of this study was to evaluate the association of hyperglycemia and hypoglycemia on adverse events in infants undergoing the arterial switch operation. METHODS: From 2000 through 2005, 93 infants underwent the arterial switch operation (mean age, 2.5 +/- 5.9 weeks; mean weight, 3.4 +/- 0.8 kg). All serum glucose values during the first 24 postoperative hours were documented. The effect of time spent in specific glycemic bands on adverse events was determined. RESULTS: Twenty-three (25%; group 1) infants spent more than 50% of the time with glucose values between 80 and 110 mg/dL, and 13 (14%; group 2) spent more than 50% of the time with glucose values of greater than 200 mg/dL. A total of 71 adverse events was documented in 45 (48%) of 93 infants. Group 1 infants were more likely to have any adverse event (P = .001) and renal insufficiency (P < .001). Group 2 infants were not more likely to have adverse events. When controlling for preoperative and operative factors, being in group 1 was an independent predictor of postoperative adverse events (P = .004). CONCLUSION: Hyperglycemia does not appear to be detrimental in postoperative infants with congenital heart disease. Infants who spent the majority of the time with glucose values between 80 and 110 mg/dL were at increased risk for adverse events. The ideal glycemic profile in the postoperative cardiac infant has yet to be defined.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/efectos adversos , Cardiopatías Congénitas/cirugía , Hiperglucemia/complicaciones , Glucemia/análisis , Femenino , Humanos , Hipoglucemia/complicaciones , Lactante , Recién Nacido , Masculino , Periodo PosoperatorioRESUMEN
BACKGROUND: Pediatric patients with heart disease are often treated with high doses of diuretics, which can lead to hypochloremic metabolic alkalosis. There are no data in children regarding the efficacy and safety of acetazolamide to treat hypochloremic metabolic alkalosis. METHODS: Patients from January 2004 to June 2005 who received acetazolamide were identified. Inclusion criteria were: age less than 18 years, being a cardiology patient, diuretics use, and had received a 3-day course of acetazolamide. Demographic information was collected along with serum electrolytes, serum creatinine/blood urea nitrogen, urine output, pH, acid-base excess, concurrent medications, cardiac lesion/surgery, and incidence of adverse effects. Efficacy of acetazolamide was determined by comparing variables before and after the 3-day course. Statistical comparisons were made using Student's t-test. RESULTS: A total of 28 patients were identified, 7 of whom received oral acetazolamide, 21 intravenous acetazolamide. Patients were a median of 2.5 (range, 0.3-20) months of age, and 57% (17/28) were female. Seventy-one percent of the cohort received acetazolamide after cardiac surgery. There was no significant difference in any electrolyte, blood urea nitrogen, or serum creatinine from baseline, except for serum bicarbonate, which decreased (36.2 +/- 4.6 vs. 30.9 +/- 4.5 mmol/L, P < 0.001), and chloride, which increased (91.1 +/- 6.8 vs. 95.4 +/- 6.2, P < 0.03). Acid-base excess values and pH decreased during therapy in patients who had the laboratory values drawn (n = 22). No change in urine output at 8 hours (5.2 +/- 2.3 vs. 4.9 +/- 2.3 mL/kg/hr, P = 0.6) or 24 hours (4.7 +/- 1.5 vs. 4.3 +/- 1.4 mL/kg/hr, P = 0.18) occurred after administration of acetazolamide. CONCLUSION: Acetazolamide was safely used in pediatric patients with heart disease to lower serum bicarbonate and acid-base excess values and raise chloride values in hypochloremic metabolic alkalosis.
Asunto(s)
Acetazolamida/uso terapéutico , Alcalosis/tratamiento farmacológico , Inhibidores de Anhidrasa Carbónica/uso terapéutico , Cloruros/sangre , Diuréticos/uso terapéutico , Cardiopatías/tratamiento farmacológico , Alcalosis/inducido químicamente , Análisis Químico de la Sangre , Preescolar , Diuréticos/efectos adversos , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Bicarbonato de Sodio/sangreRESUMEN
OBJECTIVES: It is known that levels of brain natriuretic peptide predict outcomes of treatment for adults with decompensated heart failure. We hypothesized that it could predict outcomes in children with this condition. METHODS: We divided retrospectively 82 patients with serial measurements of brain natriuretic peptide into 3 groups: those who survived and did not need readmission within less than 60 days; those who survived but needed readmission within less than 60 days; and those who died in hospital or within less than 60 days. Initial and final levels of the peptide correlated with adverse outcomes. RESULTS: The percent change in level of the peptide was minus 78 percent, minus 38 percent, and 138 percent in the readmission-free group, the readmitted, and nonsurviving groups, respectively. Final levels were significantly lower in the readmission-free group than in the readmitted and nonsurviving groups (p equals 0.013 and p is less than 0.00001, respectively) and in the readmitted group than in the nonsurvivors (p equals 0.013). On univariate analysis, the final level, the change in level, and the percentage change in level significantly predicted outcomes (p equals 0.0002, 0.0072 and 0.0005, respectively). On multivariate analysis, only the final level of the peptide significantly predicted outcomes (p equals 0.01). CONCLUSIONS: A final level of brain natriuretic peptide of greater than or equal to 760 picograms per millilitre strongly predicted an adverse outcome. Patients with higher final levels may be at higher risk of death and readmission, suggesting that this variable effectively predicts the response to treatment and prognosis in children with heart failure.
Asunto(s)
Cuidados Críticos , Insuficiencia Cardíaca/sangre , Insuficiencia Cardíaca/terapia , Péptido Natriurético Encefálico/sangre , Adolescente , Adulto , Niño , Preescolar , Femenino , Estudios de Seguimiento , Insuficiencia Cardíaca/mortalidad , Hospitalización , Humanos , Lactante , Recién Nacido , Masculino , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
BACKGROUND: Limited data exist regarding the perioperative morbidity profile of adults who have cardiac operations at pediatric facilities. METHODS: A retrospective review (January 2000 to December 2004) of patients (aged 18 years or older) who underwent cardiac surgery at our pediatric institution was performed. RESULTS: There were 149 cardiac operations performed in 135 patients. There were 2 early deaths. There were 70 preoperative noncardiac morbidities in 49 patients (36%) and 140 preoperative cardiac morbidities in 78 patients (58%). Preoperative arrhythmia (n = 76) and moderate or greater ventricular systolic dysfunction (n = 24) were most common. There were 51 postoperative adverse noncardiac events in 32 patients (24%). Renal insufficiency (> 0.5 mg/dL baseline change; n = 8) was most common. There were 53 postoperative adverse cardiac events in 44 patients (33%). Ventricular tachycardia (n = 13) was most common. Risk factors for postoperative adverse noncardiac events included preoperative histories of New York Heart Association (NYHA) class III or greater (p < 0.001), seizure (p = 0.04), and psychiatric disorder (p = 0.002). Risk factors for postoperative adverse cardiac events included older patient age (p = 0.001), preoperative functional single ventricle (p = 0.006), NHYA class III or greater (p = 0.003), atrial fibrillation/flutter (p < 0.001), and ventricular tachycardia or fibrillation (p = 0.04). CONCLUSIONS: Postoperative adverse events occur frequently when adults undergo cardiac operations at children's hospitals. Older patient age, preoperative arrhythmias, and preoperative NHYA class are predictors of postoperative adverse cardiac events.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/efectos adversos , Hospitales Pediátricos/estadística & datos numéricos , Complicaciones Intraoperatorias/epidemiología , Complicaciones Posoperatorias/epidemiología , Adulto , Factores de Edad , Arritmias Cardíacas/complicaciones , Estudios de Cohortes , Femenino , Cardiopatías/complicaciones , Cardiopatías/fisiopatología , Cardiopatías/cirugía , Humanos , Incidencia , Masculino , Registros Médicos , Trastornos Mentales/complicaciones , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Convulsiones/complicaciones , Índice de Severidad de la EnfermedadRESUMEN
Staged single-ventricle palliation is used to treat many cyanotic congenital heart diseases. Hemoglobin sickle cell disease is associated with anemia and significant vascular sickling sequelae, which increase the risk associated with single-ventricle palliation. To our knowledge, there are no reports in the English-language medical literature of single-ventricle palliation having been performed on a patient who had either sickle cell anemia or sickle cell-hemoglobin C disease. Herein, we discuss our clinical and surgical management of an infant with tricuspid atresia type IA and hemoglobin sickle cell disease who survived single-ventricle palliative procedures through the 2nd stage of a bidirectional Glenn anastomosis.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Ventrículos Cardíacos/cirugía , Enfermedad de la Hemoglobina SC/complicaciones , Cuidados Paliativos/métodos , Atresia Tricúspide/cirugía , Anastomosis Quirúrgica/métodos , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Atresia Tricúspide/complicacionesRESUMEN
Congenital heart disease is a risk factor for the development of necrotizing enterocolitis, although the exact mechanism of development remains unclear. Herein, we report the case of an infant with pulmonary atresia, an intact ventricular septum, and multiple aortopulmonary collateral vessels. At 4 weeks of age, the infant developed necrotizing enterocolitis in association with significant mesenteric oxygen desaturation, as measured by means of near-infrared spectroscopy. With bowel rest and antibiotic therapy, the patient's mesenteric oxygen saturation and clinical status improved. This case highlights the importance of impaired mesenteric oxygen delivery consequential to congenital heart disease as a possible risk factor for necrotizing enterocolitis, and the use of near-infrared spectroscopy to measure tissue perfusion noninvasively in high-risk patients. To our knowledge, this is the 1st report of mesenteric oxyhemoglobin desaturation in association with necrotizing enterocolitis in a patient who also had congenital heart disease.