RESUMEN
BACKGROUND: Pediatric heart transplant candidates on the waitlist have the highest mortality rate among all solid organ transplants. A risk score incorporating a candidate's individual risk factors may better predict mortality on the waitlist and optimize organ allocation to the sickest of those awaiting transplant. METHODS: Using the United Network for Organ Sharing (UNOS) database, we evaluated a total of 5542 patients aged 0-18 years old on the waitlist for a single, first time, heart transplant from January 2010 to June 2019. We performed a univariate analysis on two-thirds (N = 3705) of these patients to derive the factors most associated with waitlist mortality or delisting secondary to deterioration within 1 year. Those with a p <0.2 underwent a multivariate analysis and the resulting factors were used to build a prediction model using the Fine-Grey model analysis. This predictive scoring model was then validated on the remaining one-third of the patients (N = 1852). RESULTS: The Pediatric Risk to OHT (PRO) scoring model utilizes the following unique patient variables: blood type, diagnosis of congenital heart disease, weight, presence of ventilator support, presence of inotropic support, extracorporeal membrane oxygenation (ecmo) status, creatinine level, and region. A higher score indicates an increased risk of mortality. The PRO score had a predictive strength of 0.762 as measured by area under the ROC curve at 1 year. CONCLUSION: The PRO score is an improved predictive model with the potential to better assess mortality for patients awaiting heart transplant.
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Cardiopatías Congénitas , Trasplante de Corazón , Trasplante de Hígado , Obtención de Tejidos y Órganos , Humanos , Niño , Recién Nacido , Lactante , Preescolar , Adolescente , Factores de Riesgo , Listas de Espera , Estudios RetrospectivosRESUMEN
BACKGROUND: A significant number of pediatric heart transplant recipients and their families experience post-traumatic stress symptoms following transplantation, which can impact recipient behavioral and medical health outcomes. Preventive behavioral health interventions may improve outcomes, especially if interventions can be delivered at a distance to decrease barriers to mental health care. This pilot study examined the acceptability and accessibility of an evidence-informed resilience training program delivered using a video telehealth platform. A secondary aim was to assess the preliminary efficacy of the intervention on recipient behavioral health outcomes, perceived barriers to recipient medication adherence, parent behavioral health outcomes, and family functioning. METHODS: Seventeen heart transplant recipients (8-18 years old) and their families were recruited and randomly assigned to a treatment as usual (n = 8) or an intervention group (n = 9). Baseline assessment data collected included demographic information and validated behavioral health measures. Follow-up assessments included the validated measures and acceptability and satisfaction ratings. RESULTS: The study demonstrated that the program has high acceptability by recipients and parents, and a positive impact on recipients and parents, including significant reductions in youth behavioral difficulties as well as parent depression and post-traumatic stress symptoms. CONCLUSIONS: Results of this study are promising and call for further evaluation of hybrid delivery models for behavioral health screening and prevention interventions for pediatric heart transplant recipients and their families.
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Trasplante de Corazón , Telemedicina , Adolescente , Niño , Humanos , Proyectos Piloto , Padres/psicología , Depresión , Trasplante de Corazón/psicologíaRESUMEN
BACKGROUND: Since the earliest clinical successes in solid organ transplantation, the proper method of organ allocation for children has been a contentious subject. Over the past 30-35 years, the medical and social establishments of various countries have favored some degree of preference for children on the respective waiting lists. However, the specific policies to accomplish this have varied widely and changed frequently between organ type and country. METHODS: Organ allocation policies over time were examined. This review traces the reasons behind and the measures/principles put in place to promote early deceased donor transplantation in children. RESULTS: Preferred allocation in children has been approached in a variety of ways and with varying degrees of commitment in different solid organ transplant disciplines and national medical systems. CONCLUSION: The success of policies to advantage children has varied significantly by both organ and medical system. Further work is needed to optimize allocation strategies for pediatric candidates.
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Trasplante de Órganos , Obtención de Tejidos y Órganos , Niño , Humanos , Donantes de Tejidos , Listas de EsperaRESUMEN
BACKGROUND: Fontan associated liver disease (FALD) potentially impacts Fontan patients undergoing heart transplant. This multi-center study sought to identify pre-transplant risk factors and characterize any post-transplant liver recovery in those patients undergoing heart-alone transplant. METHODS: Review of Fontan patients at 12 pediatric institutions who underwent heart transplant between 2001-2019. Radiologists reviewed pre and post-transplant liver imaging for fibrosis. Laboratory, pathology and endoscopy studies were reviewed. RESULTS: 156 patients underwent transplant due to decreased ventricular function (49%), protein losing enteropathy (31%) or plastic bronchitis (10%); median age at transplant was 13.6 years (interquartile range IQR 7.8, 17.2) with a median of 9.3 years (IQR 3.2, 13.4) between the Fontan operation and transplant. Few patients had pre-transplant endoscopy (18%), and liver biopsy (19%). There were 31 deaths (20%). The median time from transplant to death was 0.5 years (95% Confidence Interval CI 0.0, 3.6). The five-year survival was 73% (95% CI 64%, 83%). Deaths were related to cardiac causes in 68% (21/31) and infection in 6 (19%). A pre-transplant elevation in bilirubin was a predictor of death. Higher platelet levels were protective. Immediate post-transplant elevations in creatinine, AST, ALT, and INR were predictive of death. Advanced liver fibrosis identified on ultrasound, computed tomography, or magnetic resonance imaging was not predictive of death. Liver imaging suggested some improvement in liver congestion post-transplant. CONCLUSIONS: Elevated bilirubin, but not fibrosis on liver imaging, was associated with post-heart transplant mortality in Fontan patients in this multicenter retrospective study. Additionally, heart transplant may alter the progression of FALD.
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Procedimiento de Fontan , Cardiopatías Congénitas , Trasplante de Corazón , Hepatopatías , Humanos , Bilirrubina , Procedimiento de Fontan/efectos adversos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/complicaciones , Hígado/patología , Cirrosis Hepática/cirugía , Cirrosis Hepática/complicaciones , Hepatopatías/etiología , Hepatopatías/cirugía , Hepatopatías/patología , Estudios Retrospectivos , AdolescenteRESUMEN
Fluid restriction and diuretic management are mainstays in the postoperative management of cardiac patients, at risk of volume overload and its deleterious effects on primary cardiac function and multi-organ systems. The importance of fluid homeostasis is further emphasized among orthotopic heart transplant recipients (OHT). We sought to investigate the relationship between postoperative volume overload, mortality, and allograft dysfunction among pediatric OHT recipients within 1-year of transplantation. This is a retrospective cohort study from a single pediatric OHT center. Children under 21 years undergoing cardiac transplantation between 2010 and 2018 were included. Cumulative fluid overload (cFO) was assessed as percent fluid accumulation adjusted for preoperative body weight. Greater than 10% cFO defined those with postoperative cFO and a comparison of postoperative cFO vs. no postoperative cFO (< 5%) is reported. 102 pediatric OHT recipients were included. Early cFO at 72 h post-OHT occurred in 14% and overall cFO at 1-week post-OHT occurred in 23% of patients. Risk factors for cFO included younger age, lower weight, and postoperative ECMO. Early cFO was associated with postoperative mortality at 1-year, OR 8.6 (95% CI 1.4, 51.6), p = 0.04, independent of age and weight. There was no significant relationship between cFO and allograft dysfunction, measured by rates of clinical rejection and cardiopulmonary filling pressures within 1-year of transplant. Early postoperative volume overload is prevalent and associated with increased risk of death at 1-year among pediatric OHT recipients. It may be an important postoperative marker of transplant survival, and this relationship warrants further clinical investigation.
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Insuficiencia Cardíaca , Trasplante de Corazón , Trasplantes , Humanos , Niño , Estudios Retrospectivos , Insuficiencia Cardíaca/etiología , Trasplante de Corazón/efectos adversos , Factores de RiesgoRESUMEN
BACKGROUND: Early detection of cardiac allograft rejection is crucial for post-transplant graft survival. Despite the progress made in immunosuppression strategies, acute cellular rejection remains a serious complication during and after the first post-transplant year, and there is a continued lack of consensus regarding its treatment, especially in pediatric transplant patients. METHODS: An open request was placed via the listserv to the membership of the Pediatric Heart Transplant Society (PHTS). Along with a broad literature search, numerous institutional protocols were pooled, analyzed and consolidated. A clinical approach document was generated highlighting areas of consensus and practice variation. RESULTS: The clinical approach document divides cellular rejection by International Society for Heart and Lung Transplantation grades and provides management strategies for each, including persistent cellular rejection. CONCLUSIONS: Cellular rejection treatment can be tailored to the clinical status, graft function, and the grade of cellular rejection. A case of mild and asymptomatic rejection may not require treatment, whereas a higher-grade rejection or rejection with graft dysfunction or hemodynamic compromise may require aggressive intravenous therapies, changes to maintenance immunosuppression therapy and augmented surveillance.
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Trasplante de Corazón , Humanos , Niño , Rechazo de Injerto/epidemiología , Terapia de Inmunosupresión , Supervivencia de Injerto , HemodinámicaRESUMEN
BACKGROUND: Management of infants with pulmonary atresia/intact ventricular septum (PA/IVS) is variable. Because of higher mortality in more severe forms, heart transplant (HT) is an acceptable approach, but waitlist and post-transplant outcomes are unclear. This study compared outcomes of infants with PA/IVS vs. other single ventricle (SV) anatomies listed for HT. METHODS: Data from the Pediatric Heart Transplant Society (1993-2018) were analyzed for survival and risk factors for mortality. RESULTS: Of 1617 SV infants, 300 had PA/IVS (19%) and 1317 had other SV (81%). Overall, 1-, 5-, and 10-year survival was higher among PA/IVS (74%, 65%, 61%) versus other SV infants (62%, 54%, 50%, p = .004). While waitlist mortality was similar between groups (p = .09), PA/IVS was an independent predictor of improved waitlist survival (HR 0.68, p = .03), and PA/IVS infants had higher incidence of waitlist removal (8% vs. 5.5%, p = .03), most commonly for being "too well." Post-transplant survival was superior among PA/IVS versus other SV infants (1- and 5-year survival 93% and 81% vs. 80% and 71%, p < .0001). Risk factors for PA/IVS waitlist mortality (2008-2018) included extracorporeal membrane oxygenation and mechanical ventilation. Prior aortopulmonary (AP) shunt among PA/IVS infants was associated with improved waitlist survival. CONCLUSIONS: Overall survival among PA/IVS infants listed for HT exceeds that of other SV infants with PA/IVS identified as an independent predictor of improved waitlist and post-transplant survival. Prior AP shunt among listed PA/IVS infants was associated with improved waitlist outcomes, though, which may reflect a listing selection bias.
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Cardiopatías Congénitas , Trasplante de Corazón , Atresia Pulmonar , Tabique Interventricular , Niño , Humanos , Lactante , Atresia Pulmonar/cirugía , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: This document is designed to outline the definition, pathogenesis, diagnostic modalities and therapeutic measures to treat antibody-mediated rejection in children postheart transplant METHODS: Literature review was conducted by a Pediatric Heart Transplant Society (PHTS) working group to identify existing pediatric and adult studies on antibody-mediated rejection (AMR). In addition, the centers participating in PHTS were asked to submit their approach to diagnosis and management of pediatric AMR. This document synthesizes information gathered from both these sources to highlight a practical approach to diagnosing and managing a child with AMR postheart transplant. This document may not represent the practice at all centers in the PHTS and serves as a starting point to understand an approach to this clinical scenario.
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Trasplante de Corazón , Trasplantes , Humanos , Niño , Adulto , Rechazo de Injerto/diagnóstico , Rechazo de Injerto/patología , AnticuerposRESUMEN
Post-transplant lymphoproliferative disorders (PTLD) are a group of lesions that can complicate solid organ or hematopoietic stem cell transplantation and are often associated with Epstein-Barr virus (EBV). The treatment of PTLD is dependent on the type of lesion and includes a wide range of therapies, but chimeric antigen receptor (CAR) T-cell therapy has not previously been reported as a treatment option for PTLD. We present a patient who developed refractory PTLD in her right retroperitoneum, right inguinal and iliac chains, and right axillary region shortly after heart transplantation and was treated with CAR T-cell therapy. She could not tolerate complete discontinuation of immunosuppression due to the risk of rejection of a life-supporting graft. The patient's PTLD responded to CAR T-cell therapy, and her heart was monitored throughout the treatment course without any signs of rejection or ventricular dysfunction. CAR T-cell therapy may be a viable treatment option in patients who develop PTLD after a solid organ transplant.
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Tratamiento Basado en Trasplante de Células y Tejidos/métodos , Trasplante de Corazón , Trastornos Linfoproliferativos/inmunología , Trastornos Linfoproliferativos/terapia , Complicaciones Posoperatorias/inmunología , Complicaciones Posoperatorias/terapia , Receptores Quiméricos de Antígenos/inmunología , Adolescente , Femenino , HumanosRESUMEN
Post-transplant lymphoproliferative disorders (PTLD) are the main malignancy seen after pediatric heart transplant and are a significant cause of morbidity and mortality. Prior to the development of detailed guidelines, we sought to identify trends in screening, diagnosis, and treatment of pediatric PTLD. All Pediatric Heart Transplant Society (PHTS) institutions were surveyed. No identifiable patient information was shared. From 56 PHTS centers, 22 responses were received (39.3%). 100% agree PTLD cannot be diagnosed solely based on elevated Epstein-Barr virus (EBV) load. All respondents routinely screen for EBV by blood PCR, but frequency of screening varies. There was intermediate consensus regarding the use of computed tomography (CT) and/or positron emission tomography (PET) in surveillance management for PTLD. Most centers require a diagnostic biopsy before initiating new treatment for PTLD (14 of 18, 77.8%), but many reduce immune suppression based on elevated EBV without pathologic PTLD (16 of 22, 72.7%). Beyond immune modulation, rituximab is most commonly used (9 of 13, 69.2%). Consultation with oncology is common (17 of 17, 100%), but timing varies widely. Our survey highlights significant elements of agreement and significant practice variation among PHTS institutions regarding pediatric PTLD. Reduction of immune suppression prior to pathologic diagnosis of PTLD is a common management strategy. When this fails, rituximab is used, but is most often reserved until after confirmation of the diagnosis. Oncology subspecialists are commonly involved in these cases. Our findings highlight the need to develop improved guidelines for evaluation and treatment of pediatric PTLD.
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Trasplante de Corazón , Trastornos Linfoproliferativos/diagnóstico , Trastornos Linfoproliferativos/terapia , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/terapia , Pautas de la Práctica en Medicina/tendencias , Adolescente , Niño , Preescolar , Consenso , Esquema de Medicación , Quimioterapia Combinada , Femenino , Rechazo de Injerto/prevención & control , Encuestas de Atención de la Salud , Humanos , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Lactante , Recién Nacido , Trastornos Linfoproliferativos/etiología , Masculino , Complicaciones Posoperatorias/etiología , Guías de Práctica Clínica como Asunto , Sistema de RegistrosRESUMEN
Pediatric sHKTx has become an effective therapy for patients with combined cardiac and renal failure. Often, these patients develop human leukocyte antigen antibodies from their previous allografts and are therefore more difficult to re-transplant. We describe the largest case series of a predominantly sensitized pediatric sHKTx with emphasis on medical management and patient outcomes. Demographics, clinical characteristics, antibody, and biopsy data were retrospectively collected from University of California, Los Angeles database and correlated with short- and long-term patient and allograft outcomes of all sHKTx performed between 2002 and 2015. We identified seven pediatric patients who underwent sHKTx at our center. Mean age at time of sHKTx was 13.7 years and 85.7% were re-graft patients. 57.1% were sensitized with cPRA >50% and another 57.1% had preformed donor-specific antibody. Five-year renal allograft survival and patient survival was 85.7% for both end-points. The remaining six patients are all alive (mean follow-up 78.5 months) with good kidney and heart function. sHKTx in a population with increased immunological risk can be associated with good long-term outcomes and offers potential guidance to the pediatric transplant community where data are limited.
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Insuficiencia Cardíaca/cirugía , Trasplante de Corazón/métodos , Trasplante de Riñón/métodos , Insuficiencia Renal/cirugía , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Supervivencia de Injerto , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/mortalidad , Humanos , Masculino , Insuficiencia Renal/complicaciones , Insuficiencia Renal/mortalidad , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi®), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH.
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Acetamidas/uso terapéutico , Antihipertensivos/uso terapéutico , Hipertensión Pulmonar/tratamiento farmacológico , Pirazinas/uso terapéutico , Adolescente , Niño , Epoprostenol/análogos & derivados , Epoprostenol/uso terapéutico , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/tratamiento farmacológico , Humanos , Masculino , Resultado del Tratamiento , Prueba de Paso/métodos , Adulto JovenRESUMEN
Fukuyama congenital muscular dystrophy weakens both skeletal and cardiac muscles, but the rate of cardiomyopathic progression can accelerate faster than that of skeletal muscles. A 14-year-old boy with Fukuyama congenital muscular dystrophy presented with mild skeletal myopathy but severe cardiomyopathy requiring heart transplantation within 1 year of declining heart function. These patients need frequent screening regardless of musculoskeletal symptoms.
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Insuficiencia Cardíaca/diagnóstico por imagen , Insuficiencia Cardíaca/etiología , Distrofias Musculares/complicaciones , Adolescente , Ecocardiografía , Insuficiencia Cardíaca/cirugía , Trasplante de Corazón , Humanos , Japón , Masculino , Distrofias Musculares/congénito , Distrofias Musculares/diagnóstico por imagenRESUMEN
Introduction The spatial peaks QRS-T angle accurately distinguishes children with hypertrophic cardiomyopathy from their healthy counterparts. The spatial peaks QRS-T angle is also useful in risk stratification for ventricular arrhythmias. We hypothesised that the spatial peaks QRS-T angle would be useful for the prediction of ventricular arrhythmias in hypertrophic cardiomyopathy patients under 23 years of age. METHODS: Corrected QT interval and spatial peaks QRS-T angles were retrospectively assessed in 133 paediatric hypertrophic cardiomyopathy patients (12.4±6.6 years) with versus without ventricular arrhythmias of 30 seconds or longer. Significance, positive/negative predictive values, and odds ratios were calculated based on receiver operating characteristic curve cut-off values. RESULTS: In total, 10 patients with ventricular arrhythmias were identified. Although the corrected QT interval did not differentiate those with versus without ventricular arrhythmias, the spatial peaks QRS-T angle did (151.4±19.0 versus 116.8±42.6 degrees, respectively, p<0.001). At an optimal cut-off value (124.1 degrees), the positive and negative predictive values of the spatial peaks QRS-T angle were 15.4 and 100.0%, respectively, with an odds ratio of 25.9 (95% CI 1.5-452.2). CONCLUSION: In children with hypertrophic cardiomyopathy, the spatial peaks QRS-T angle is associated with ventricular arrhythmia burden with high negative predictive value and odds ratio.
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Cardiomiopatía Hipertrófica/complicaciones , Taquicardia Ventricular/diagnóstico , Vectorcardiografía , Adolescente , Cardiomiopatía Hipertrófica/diagnóstico , Niño , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Masculino , Oportunidad Relativa , Valor Predictivo de las Pruebas , Curva ROC , Estudios Retrospectivos , Taquicardia Ventricular/etiología , Taquicardia Ventricular/fisiopatología , Adulto JovenRESUMEN
BACKGROUND: Fibroblast growth factor-23 (FGF23) levels are elevated in cardiopulmonary bypass (CPB)-associated acute kidney injury (AKI); however, it is unknown how much of the circulating FGF23 is intact and bioactive. Hypoxia may induce FGF23 production, yet its impact in humans is unknown. Pediatric cardiac surgery patients have both a high incidence of CPB-associated AKI and a high prevalence of chronic hypoxemia. METHODS: We assessed the effects of hypoxemia and CPB-associated AKI on C-terminal FGF23 (cFGF23) and intact FGF23 (iFGF23) levels in 32 pediatric cardiac surgery patients with normal estimated glomerular filtration rate (eGFR). Plasma cFGF23 and iFGF23 were measured preoperatively and serially postoperatively. RESULTS: Despite normal renal and ventricular function, preoperative cFGF23 levels were high and elevated out of proportion to iFGF23 levels. Preoperative oxygen saturation measurements correlated inversely with FGF23 levels. Preoperative cFGF23 and oxygen saturation both predicted postoperative AKI. Postoperatively, cFGF23 and iFGF23 increased by 2 h postreperfusion; iFGF23 then returned to baseline, but cFGF23 remained elevated through 24 h postreperfusion. Group status (AKI vs. non-AKI) modified the effect of time on changes in iFGF23 levels but not cFGF23 levels. CONCLUSIONS: Preoperative cFGF23 may predict CPB-associated kidney dysfunction. Changes over time in cFGF23 and iFGF23 levels post-CPB differ. Chronic hypoxemia may affect FGF23 production in humans.
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Lesión Renal Aguda/sangre , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Puente Cardiopulmonar/efectos adversos , Factores de Crecimiento de Fibroblastos/sangre , Hipoxia/sangre , Fragmentos de Péptidos/sangre , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/etiología , Biomarcadores/sangre , Niño , Preescolar , Enfermedad Crónica , Femenino , Factor-23 de Crecimiento de Fibroblastos , Humanos , Hipoxia/diagnóstico , Hipoxia/etiología , Lactante , Masculino , Valor Predictivo de las Pruebas , Factores de Riesgo , Factores de Tiempo , Resultado del TratamientoRESUMEN
Mechanical circulatory support has been used for more than 30 yr to allow the heart to recover from ischemia and injury. There are limited pediatric data, however, on the efficacy of ECMO in the setting of post-transplantation support for primary graft dysfunction or rejection. Data from all patients at our university-affiliated, tertiary care children's hospital who underwent OHT between 1998 and 2010 and required subsequent ECMO support were analyzed. The primary outcome measure was survival to hospital discharge. Two hundred and three pediatric patients underwent OHT between 1998 and 2010 at our institution. Twenty-nine of these patients experienced post-transplantation cardiac failure requiring ECMO support, 18 of whom survived to hospital discharge (62%). Survival in the rejection and allograft vasculopathy group was 75%, and survival in patients with primary graft failure was 53% after ECMO support (p = 0.273). Patient survival to hospital discharge was not associated with ischemic time or duration of ECMO. ECMO provides hemodynamic support in the setting of cardiac failure and can be used successfully after pediatric OHT for primary graft dysfunction or rejection.
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Oxigenación por Membrana Extracorpórea , Insuficiencia Cardíaca/terapia , Trasplante de Corazón , Complicaciones Posoperatorias/terapia , Niño , Preescolar , Femenino , Insuficiencia Cardíaca/mortalidad , Trasplante de Corazón/mortalidad , Humanos , Masculino , Alta del Paciente , Complicaciones Posoperatorias/mortalidad , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
INTRODUCTION: The spatial peaks QRS-T angle has been shown to differentiate adult patients with hypertrophic cardiomyopathy (HCM) from controls. We hypothesized that the spatial peaks QRS-T angle would, in isolation, be more accurate than the Italian 12-lead ECG Pre-participation Screening criteria or the Seattle criteria for detecting hypertrophic cardiomyopathy (HCM) in pediatric patients. METHODS: A retrospective study of pediatric patients with HCM compared to age and gender-matched control patients was undertaken. Significance, odds ratios, sensitivity and specificity of HCM detection of the visually derived spatial peaks QRS-T angle were compared to those of traditional 12-lead ECG criteria using: 1) Italy's National Pre-participation Screening Programme criteria; and 2) described criteria from Seattle. RESULTS: ECG results from 130 pediatric HCM patients (14.2±4.4years) were compared to 470 control patients (normal echocardiograms, mean age 13.4±4.6years). Mean±standard deviation (SD) values for spatial peaks QRS-T angles were 120.4±40.7 and 21.3±13.7 degrees for HCM and controls, respectively (P<0.001). A spatial peaks QRS-T angle cutoff value of >54.9 degrees yielded greater sensitivity and specificity (93.1% and 98.7%, respectively) for detecting HCM over ECG criteria from Italy (68.5% and 48.1%, respectively) or Seattle (64.6% and 78.9%, respectively) with odds ratios at 1039.70 (95% CI 363.03 to 2977.67), 2.01 (95% CI 1.33 to 3.04) and 6.84 (4.49-10.44), respectively. CONCLUSION: In our cohort, a visually derived spatial peaks QRS-T angle has increased sensitivity and specificity for detection of HCM in pediatric patients compared to currently utilized Italian or Seattle ECG criteria.
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Cardiomiopatía Hipertrófica/diagnóstico , Diagnóstico por Computador/métodos , Diagnóstico por Computador/normas , Electrocardiografía/métodos , Electrocardiografía/normas , Guías de Práctica Clínica como Asunto , Adolescente , Cardiomiopatía Hipertrófica/clasificación , Femenino , Humanos , Italia , Masculino , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Estados UnidosRESUMEN
BACKGROUND: Neonatal pulse oximetry screening (POS) algorithms for critical congenital heart disease (CCHD) have contributed towards decreasing neonatal mortality but cannot be applied at high altitudes. New POS algorithms at high altitudes are needed. METHODS: This observational, prospective study included newborns born at different altitudes from 0 to 4380 meters above the sea level in Peru. Healthy newborns underwent neonatal preductal and postductal oximetry, echocardiography and telephonic follow-up up to 12 months of age. Newborns with CCHD underwent preductal and postductal oximetry at the time of telemedicine evaluation while located at the high-altitude hospital where they were born, and their diagnoses were confirmed with echocardiography locally or after arriving to the referral center. Two new algorithms were designed using clinically accepted neonatal oximetry cutoffs or the 5th and 10th percentiles for preductal and postductal oximetry values. RESULTS: A total of 502 healthy newborns and 15 newborns with CCHD were enrolled. Echocardiography and telephonic follow-up were completed in 227 (45%) and 330 healthy newborns (65%), respectively. The algorithm based on clinically accepted cutoffs had a sensitivity of 92%, specificity of 73% and false positive rate of 27% The algorithm based on the 5th and 10th percentiles had a sensitivity of 80%, specificity of 88% and false positive rate of 12%. CONCLUSIONS: Two algorithms that detect CCHD at different altitudes had adequate performance but high false positive rates.
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Altitud , Cardiopatías Congénitas , Humanos , Recién Nacido , Estudios Prospectivos , Cardiopatías Congénitas/diagnóstico por imagen , Oximetría , Tamizaje Neonatal , AlgoritmosRESUMEN
HTx in neonates is mainstay therapy for those with severe cardiomyopathies and congenital heart disease. Fetal listing for HTx has been proposed as a way to increase the potential window for a donor with outcomes predicted to be similar to the neonatal population. Data from the PHTS, a prospective multicenter study, were used to examine the outcomes of fetuses listed between 1993 and 2009. Four thousand three hundred and sixty-five children were listed for HTx during this period. Fetuses comprised 1% and neonates 19.8% of listed patients. In those patients listed as fetus and transplanted, the median wait time from listing to HTx was 55 days (range 4-255), with a median of 25 days (range 0-233) after birth. By six months post-listing, a higher proportion of fetal listed patients had undergone HTx with a lower waitlist mortality when compared with neonate. There was no significant difference in survival following HTx between the two group (p = 0.4). While the results of this study may be less applicable to current practice due to changes in referrals for fetal listing, they do indicate that fetal listing can be a reasonable option. These results are of particular interest at the present time given the ongoing public discourse on the proposed elimination of fetal listing within UNOS.
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Cardiomiopatías/cirugía , Cardiopatías Congénitas/cirugía , Trasplante de Corazón , Listas de Espera , Factores de Edad , Cardiomiopatías/diagnóstico , Bases de Datos Factuales , Femenino , Corazón Fetal , Cardiopatías Congénitas/diagnóstico , Humanos , Recién Nacido , Masculino , Embarazo , Estudios Prospectivos , Factores de Tiempo , Resultado del TratamientoRESUMEN
BACKGROUND: To date, no studies evaluated implicit bias among clinicians caring for children with advanced heart failure. OBJECTIVES: This study aims to evaluate implicit racial and socioeconomic bias among pediatric heart transplant clinicians. METHODS: A cross-sectional survey of transplant clinicians from the Pediatric Heart Transplant Society was conducted between June and August 2021. The survey consisted of demographic questions along with explicit and validated race and socioeconomic status (SES) implicit association tests (IATs). Implicit and explicit biases among survey group members were studied and associations were tested between implicit and explicit measures. RESULTS: Of 500 members, 91 (18.2%) individuals completed the race IAT and 70 (14%) completed the SES IAT. Race IAT scores indicated moderate levels of implicit bias (mean = 0.33, d = 0.76; P < 0.001; ie, preference for White individuals). SES IAT scores indicated strong implicit bias (mean = 0.52, d = 1.53; P < 0.001; ie, preference for people from upper SES). There were weak levels of explicit race and wealth bias. There was a strong level of explicit education bias (mean = 5.22, d = 1.19; P < 0.001; ie, preference for educated people). There were nonsignificant correlations between the race and the SES IAT and explicit measures (P > 0.05 for all). CONCLUSIONS: As observed across other health care disciplines, among a group of pediatric heart transplant clinicians, there is an implicit preference for individuals who are White and from higher SES, and an explicit preference for educated people. Future studies should evaluate how implicit biases affect clinician behavior and assess the impact of efforts to reduce such biases.