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OBJECTIVES: The activated partial thromboplastin time (aPTT) is the most frequently used monitoring assay for bivalirudin in children and young adults on mechanical circulatory support including ventricular assist devices (VADs) and extracorporeal membrane oxygenation (ECMO). However, intrinsic variability of the aPTT complicates management and risks bleeding or thrombotic complications. We evaluated the utility and reliability of a bivalirudin-calibrated dilute thrombin time (Bival dTT) assay for bivalirudin monitoring in this population. DESIGN: Retrospective analysis of clinical data (including aPTT, dilute thrombin time [dTT]) and results of residual plasma samples from VAD patients were assessed in two drug-calibrated experimental assays. One assay (Bival dTT) was validated for clinical use in VAD patients, and subsequently used by clinicians in ECMO patients. Pearson correlation and simple linear regression were used to determine R2 correlation coefficients between the different laboratory parameters using Statistical Package for Social Sciences (Armonk, NY). SETTING: ICUs at Cincinnati Children's Hospital Medical Center. SUBJECTS: Children on VAD or ECMO support anticoagulated with bivalirudin. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: One hundred fifteen plasma samples from 11 VAD patients were analyzed. Both drug-calibrated experimental assays (anti-IIa and Bival dTT) showed excellent correlation with each other ( R2 = 0.94) and with the dTT ( R2 = 0.87), but poor correlation with aPTT ( R2 = 0.1). Bival dTT was selected for validation in VAD patients. Subsequently, clinically ordered results (105) from 11 ECMO patients demonstrated excellent correlation between the Bival dTT and the standard dTT ( R2 = 0.86) but very poor correlation with aPTT ( R2 = 0.004). CONCLUSIONS: APTT is unreliable and correlates poorly with bivalirudin's anticoagulant effect in ECMO and VAD patients. A drug-calibrated Bival dTT offers superior reliability and opportunity to standardize results across institutions. Additional studies are needed to determine an appropriate therapeutic range and correlation with clinical outcomes.
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Antitrombinas , Oxigenación por Membrana Extracorpórea , Corazón Auxiliar , Hirudinas , Fragmentos de Péptidos , Proteínas Recombinantes , Humanos , Hirudinas/administración & dosificación , Oxigenación por Membrana Extracorpórea/métodos , Estudios Retrospectivos , Fragmentos de Péptidos/sangre , Fragmentos de Péptidos/uso terapéutico , Niño , Masculino , Femenino , Preescolar , Proteínas Recombinantes/uso terapéutico , Proteínas Recombinantes/administración & dosificación , Antitrombinas/uso terapéutico , Tiempo de Tromboplastina Parcial , Adolescente , Lactante , Reproducibilidad de los Resultados , Monitoreo de Drogas/métodosRESUMEN
OBJECTIVES: Placement of a ventricular assist device (VAD) improves outcomes in children with advanced heart failure, but adverse events remain important consequences. Preoperative mechanical ventilation (MV) increases mortality, but it is unknown what impact prolonged postoperative MV has. DESIGN: Advanced Cardiac Therapies Improving Outcomes Network (ACTION) and Pediatric Cardiac Critical Care Consortium (PC 4 ) registries were used to identify and link children with initial VAD placement admitted to the cardiac ICU (CICU) from August 2014 to July 2020. Demographics, cardiac diagnosis, preoperative and postoperative CICU courses, and outcomes were compiled. Univariable and multivariable statistics assessed association of patient factors with prolonged postoperative MV. Multivariable logistic regression sought independent associations with outcomes. SETTING: Thirty-five pediatric CICUs across the United States and Canada. PATIENTS: Children on VADs included in both registries. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Two hundred forty-eight ACTION subjects were linked to a matching patient in PC 4 . Median (interquartile) age 7.7 years (1.5-15.5 yr), weight 21.3 kg (9.1-58 kg), and 56% male. Primary diagnosis was congenital heart disease (CHD) in 35%. Pre-VAD explanatory variables independently associated with prolonged postoperative MV included: age (incidence rate ratio [IRR], 0.95; 95% CI, 0.93-0.96; p < 0.01); preoperative MV within 48 hours (IRR, 2.76; 95% CI, 1.59-4.79; p < 0.01), 2-7 days (IRR, 1.82; 95% CI, 1.15-2.89; p = 0.011), and greater than 7 days before VAD implant (IRR, 2.35; 95% CI, 1.62-3.4; p < 0.01); and CHD (IRR, 1.96; 95% CI, 1.48-2.59; p < 0.01). Each additional day of postoperative MV was associated with greater odds of mortality (odds ratio [OR], 1.09 per day; p < 0.01) in the full cohort. We identified an associated greater odds of mortality in the 102 patients with intracorporeal devices (OR, 1.24; 95% CI, 1.04-1.48; p = 0.014), but not paracorporeal devices (77 patients; OR, 1.04; 95% CI, 0.99-1.09; p = 0.115). CONCLUSIONS: Prolonged MV after VAD placement is associated with greater odds of mortality in intracorporeal devices, which may indicate inadequacy of cardiopulmonary support in this group. This linkage provides a platform for future analyses in this population.
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Insuficiencia Cardíaca , Corazón Auxiliar , Sistema de Registros , Respiración Artificial , Humanos , Niño , Masculino , Femenino , Respiración Artificial/estadística & datos numéricos , Preescolar , Lactante , Adolescente , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/mortalidad , Estados Unidos/epidemiología , Canadá/epidemiología , Unidades de Cuidado Intensivo Pediátrico , Resultado del Tratamiento , Complicaciones Posoperatorias/epidemiologíaRESUMEN
Critically ill pediatric patients supported on ventricular assist devices (VADs) are increasingly being anticoagulated on bivalirudin, but with difficulty monitoring anticoagulation. Activated partial thromboplastin time (aPTT) has recently been shown to poorly correlate with bivalirudin plasma concentrations, while dTT had excellent correlation. However, aPTT is the more common monitoring test and dTT testing is rarely used. In addition, effects of frequent clinical VAD scenarios (such as inflammation) on the accuracy of aPTT and dTT testing remains uncertain. We reviewed the effects of clinical scenarios (infection/inflammation, chylothorax, and steroids administration) on anticoagulation monitoring in 10 pediatric VAD patients less than 3 years at Cincinnati Children's Hospital Medical Center from 10/27/2020 to 5/6/2022 using bivalirudin for anticoagulation. There were 16 inflammation/infection, 3 chylothorax, and 6 steroids events. Correlation between dTT and aPTT was significantly lower after infection/inflammation, with dTT increasing prior to inflammation/infection while aPTT remained unchanged. In addition, steroids are administered to VAD patients to reduce inflammation and thus additionally stabilize anticoagulation. However, this anticoagulation stabilization effect was reflected more accurately by dTT compared to aPTT. In children requiring VAD support utilizing bivalirudin anticoagulation, inflammation/infection is a common occurrence resulting in anticoagulation changes that may be more accurately reflected by dTT as opposed to aPTT.
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BACKGROUND: Both gender- and weight-matching between donor and recipient are thought to impact survival in pediatric heart transplantation, with clinical dogma holding that male donor hearts and "ideal" weight-matching yield superior survival. The composite impacts of gender and weight on post-transplant survival (PTS) are understudied. METHODS: All pediatric (age <18) heart recipients between 1989 and 2021 with the complete recipient and donor gender and weight data were identified in the United Network for Organ Sharing database. Patients were grouped by recipient-donor gender (M & F) and donor-to-recipient weight ratio (DRWR; undersized [<0.8], ideal-sized [0.8-1.5], oversized [>1.5]). RESULTS: A total of 10 697 patients were identified. Among male recipients, PTS was greatest with oversized DRWR from either male or female donors (median 22.4 and 20.6 years; p < .001 vs. others) and lowest for undersized DRWR from either male or female donors (median 13.4 and 13.2 years; p < .001 vs. others). The majority (64%) of male recipients received ideal-sized DRWR, among which female donor hearts yielded superior survival to males (median 18.9 vs. 17.4 years, p = .014). No differences in PTS existed for female recipients on the basis of gender-match, DRWR, and gender/DRWR together (all p > .1). CONCLUSIONS: When considered together, gender and DRWR pairings impact PTS in male-but not female-pediatric heart transplant recipients. For males receiving ideal-sized DRWR organs (most common pairing, >60%), male recipients achieve superior survival when female donor hearts are transplanted. These findings suggest that if weight is being used for size-matching, donor gender should also be considered, particularly for male recipients.
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Trasplante de Corazón , Donantes de Tejidos , Humanos , Niño , Masculino , Estudios Retrospectivos , Corazón , Receptores de Trasplantes , Supervivencia de InjertoRESUMEN
Objective: Hospitalized children with cardiac disease have the highest rate of cardiac arrest compared to other disease types. Different intensive care unit (ICU) models exist, but it remains unknown whether resuscitation guideline adherence is different between cardiac ICUs (CICU) and general pediatric ICUs (PICU). We hypothesize there is no difference in resuscitation practices between unit types. Design: Retrospective observational study. Setting: The American Heart Association's Get With The Guidelines®-Resuscitation (GWTG-R) registry. Patients: Children < 18 years old with medical or surgical cardiac disease who had cardiopulmonary arrest from 2014 to 2018. Intervention: None. Measurements and Main Results: Events were assessed for compliance with GWTG-R achievement measures of time to first chest compressions ≤ 1 min, time to intravenous/intraosseous epinephrine ≤ 5 min, time to first shock ≤ 2 min for ventricular fibrillation (VF)/pulseless ventricular tachycardia (VT), and confirmation of endotracheal tube placement. Additional practices were evaluated for consistency with Pediatric Advanced Life Support (PALS) recommendations. Eight hundred and eighty-six patients were evaluated, 687 (79%) in CICUs and 179 (21%) in PICUs. 484 (56%) had surgical cardiac disease. There were no differences in GWTG-R achievement measures or PALS recommendations between ICU types in univariable or multivariable models. Amiodarone, lidocaine, and nonstandard medication use did not differ by unit type. Extracorporeal cardiopulmonary resuscitation (ECPR) was more common in CICUs for both medical (16% vs 7%) and surgical (25% vs 2.5%) categories (P < .0001). Conclusions: Resuscitation compliance for patients with cardiac disease is similar between CICUs and PICUs. Patients were more likely to receive ECPR in CICUs. Additional study should evaluate how ICU type affects arrest outcomes in children with cardiac disease.
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Reanimación Cardiopulmonar , Paro Cardíaco , Taquicardia Ventricular , Niño , Humanos , Adolescente , Paro Cardíaco/terapia , Fibrilación Ventricular , Epinefrina , Unidades de Cuidado Intensivo PediátricoRESUMEN
OBJECTIVES: The epidemiology of unplanned extubations (UEs) and associated adverse outcomes in pediatric cardiac ICUs (CICU). DESIGN: Registry data (August 2014 to October 2020). SETTING: Forty-five Pediatric Cardiac Critical Care Consortium hospitals. PATIENTS: Patients receiving mechanical ventilation (MV) via endotracheal tube (ETT). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Fifty-six thousand five hundred eight MV courses occurred in 36,696 patients, with a crude UE rate of 2.8%. In cardiac surgical patients, UE was associated with longer duration of MV, but we failed to find such association in medical patients. In both cohorts, UE was associated with younger age, being underweight, and airway anomaly. In multivariable logistic regression, airway anomaly was associated with UE in all patients. Younger age, higher Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score category, longer duration of MV, and initial oral rather than nasal ETT are associated with UE in the surgical group, but we failed to find such associations in the medical group. UE was associated with a higher reintubation rate compared with elective extubation (26.8 vs 4.8%; odds ratio [OR], 7.35; 95% CI, 6.44-8.39; p < 0.0001) within 1 day of event. After excluding patients having redirection of care, UE was associated with at least three-fold greater odds for each of ventilator-associated pneumonia (VAP), cardiac arrest, and use of mechanical circulatory support (MCS). However, we failed to identify an association between UE and greater odds of mortality (1.2 vs 0.8%; OR, 1.48; 95% CI, 0.86-2.54; p = 0.15), but uncertainty remains. CONCLUSIONS: UE in CICU patients is associated with greater odds of cardiac arrest, VAP, and MCS. Cardiac medical and surgical patients in the CICU appear to have different explanatory factors associated with UE, and perhaps these may be modifiable and tested in future collaborative population research.
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Paro Cardíaco , Neumonía Asociada al Ventilador , Humanos , Niño , Extubación Traqueal/efectos adversos , Prevalencia , Respiración Artificial/efectos adversos , Unidades de Cuidado Intensivo Pediátrico , Cuidados Críticos , Neumonía Asociada al Ventilador/epidemiología , Neumonía Asociada al Ventilador/etiología , Intubación Intratraqueal/efectos adversos , Paro Cardíaco/etiología , Sistema de Registros , Factores de RiesgoRESUMEN
OBJECTIVE: Extracorporeal Membrane Oxygenation (ECMO) may serve as a life-saving rescue therapy in critically ill children with respiratory failure. While survival rates of ECMO in children with secondary immunodeficiency is considered relatively poor, survival rates in children with primary immunodeficiencies (PID) has yet to be thoroughly investigated. DESIGN: Retrospective analysis of prospectively collected data from children (29 days-18 years old). PID patients were identified by using International Classification of Diseases (ICD) codes. SETTING: Data were retrieved from Extracorporeal Life Support Organization Registry (1989-2018). INTERVENTIONS: ECMO for a pulmonary support indication. The survival-to-discharge rate was calculated and factors influencing outcomes were compared between survivors and non-survivors. MEASUREMENTS AND MAIN RESULTS: A total of 73 eligible ECMO runs were included. The survival-to-discharge rate in pediatric PID patients was 45.2%. No differences were noted in survival based on type of immunodeficiency (p = 0.42) or decade of support (p = 0.98). There was no difference in the rate of pre-ECMO infection in survivors versus non-survivors (p = 0.69). The survival-to-discharge rate in patients with a culture positive infection during the ECMO run was 45.0% versus 45.3% in those with no infection (p = 0.98). In multivariate analysis, only cardiac complications (OR 5.09, 95% CI: 1.15-22.53), pulmonary complications (OR: 13.00, 95% CI: 1.20-141.25), and neurologic complications (OR: 9.86, 95% CI: 1.64-59.21) were independently associated with increased mortality. CONCLUSION: Children with a PID who require extracorporeal life support due to respiratory failure have a reasonable chance of survival and should be considered candidates for ECMO. The presence of a pre-ECMO infection should not be considered an ECMO contraindication.
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Oxigenación por Membrana Extracorpórea , Insuficiencia Respiratoria , Niño , Humanos , Estudios Retrospectivos , Insuficiencia Respiratoria/etiología , Insuficiencia Respiratoria/terapia , Sistema de Registros , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
PURPOSE: Achieving effective anticoagulation during neonatal extracorporeal membrane oxygenation (ECMO) without increasing the risk of hemorrhage remains challenging. The use of antithrombin III (AT-III) for this purpose has been examined, but studies have been limited to intermittent bolus dosing. We aimed to evaluate the efficacy and safety of an institutionally developed AT-III continuous infusion protocol in neonates receiving ECMO for the treatment of congenital diaphragmatic hernia (CDH). METHODS: In this single center, retrospective study, all neonates with a CDH who received ECMO support during the study period were included. Data on anticoagulation labs and therapy, life-threatening bleeding, and circuit changes were analyzed. RESULTS: Eleven patients were divided into two groups: patients with AT-III continuous infusion (n = 5) and without (n = 6). There were no differences in the gestational age (p = 0.29), sex (p = 1.00), ECMO duration (p = 0.59), or initial AT-III levels (p = 0.76) between groups. Patients in the AT-III infusion group had on average 18.5% higher AT-III levels (p < 0.0001). Patients receiving continuous AT-III infusions spent a significantly higher percentage of ECMO time within the therapeutic range, measured using anti-Factor Xa levels (64.9±4.2% vs. 29.1±8.57%, p = 0.008), and required fewer changes to the heparin infusion rate (6.48±0.88 vs 2.38±0.36 changes/day changes/day, p = 0.005). Multivariate analysis revealed continuous infusion of AT-III did not increase the rate of intracranial or surgical bleeding (p = 0.27). CONCLUSION: AT-III as a continuous infusion in CDH neonates on ECMO provides a decreased need to modify heparin infusion and more consistent therapeutic anticoagulation without increasing the risk of life-threatening bleeding.
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Oxigenación por Membrana Extracorpórea , Hernias Diafragmáticas Congénitas , Recién Nacido , Humanos , Antitrombina III , Oxigenación por Membrana Extracorpórea/métodos , Estudios Retrospectivos , Anticoagulantes/uso terapéutico , Hemorragia , Heparina/uso terapéuticoRESUMEN
INTRODUCTION: In children with myocarditis or dilated cardiomyopathy (DCM) on extracorporeal membrane oxygenation (ECMO) for cardiogenic shock, it is often necessary to decompress the left heart to minimize distension and promote myocardial recovery. We compare outcomes in those who underwent balloon atrial septostomy (BAS) versus direct left atrial (LA) drainage for left heart decompression in this population. METHODS: Retrospective study of the Extracorporeal Life Support Organization (ELSO) multicenter registry of patients ≤ 18 years with myocarditis or DCM on ECMO who underwent LA decompression. Descriptive and univariate statistics assessed association of patient factors with decompression type. Multivariable logistic regression sought independent associations with outcomes. RESULTS: 369 pediatric ECMO runs were identified. 52% myocarditis, 48% DCM, overall survival 74%. 65% underwent BAS and 35% LA drainage. Patient demographics including age, weight, gender, race/ethnicity, diagnosis, pre-ECMO pH, mean airway pressure, and arrest status were similar. 89% in the BAS group were peripherally cannulated onto ECMO, versus 3% in the LA drainage group (p < .001). On multivariable analysis, LA drainage (OR 3.96; 95% CI, 1.47-10.711; p = .007), renal complication (OR 2.37; 95% CI, 1.41-4.01; p = .001), cardiac complication (OR 3.14; 95% CI, 1.70-5.82; p < .001), and non-white race/ethnicity (OR 1.75; 95% CI, 1.04-2.94; p = .035) were associated with greater odds of mortality. There was a trend toward more episodes of pulmonary hemorrhage in BAS (n = 17) versus LA drainage group (n = 3), p = .08. Comparing only those with central cannulation, LA drainage group was more likely to be discontinued from ECMO due to recovery (72%) versus the BAS group (48%), p = .032. CONCLUSIONS: In children with myocarditis or DCM, there was a three times greater likelihood for mortality with LA drainage versus BAS for LA decompression. When adjusted for central cannulation groups only, there was better recovery in the LA drainage group and no difference in mortality. Further prospective evaluation is warranted.
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BACKGROUND: The approach to vascular access in children with CHD is a complex decision-making process that may have long-term implications. To date, evidence-based recommendations have not been established to inform this process. METHODS: The RAND/UCLA Appropriateness Method was used to develop miniMAGIC, including sequential phases: definition of scope and key terms; information synthesis and literature review; expert multidisciplinary panel selection and engagement; case scenario development; and appropriateness ratings by expert panel via two rounds. Specific recommendations were made for children with CHD. RESULTS: Recommendations were established for the appropriateness of the selection, characteristics, and insertion technique of intravenous catheters in children with CHD with both univentricular and biventricular physiology. CONCLUSION: miniMAGIC-CHD provides evidence-based criteria for intravenous catheter selection for children with CHD.
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Cardiopatías Congénitas , Catéteres , Niño , Humanos , MichiganRESUMEN
The article Nonsurgical Risk Factors for the Development of Chylothorax in Children after Cardiac SurgeryDoes Fluid Matter?, written by Tanya Perry, Kelly Bora, Adnan Bakar, David B. Meyer and Todd Sweberg, was originally published electronically on the publisher's internet portal (currently SpringerLink) on November 2019 with open access. With the author(s)' decision to step back from Open Choice, the copyright of the article changed on November 2019 to © Springer Science+Business Media, LLC, part of Springer Nature 2019 and the article is forthwith distributed under the terms of copyright.
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We hypothesize that there are post-operative, non-surgical risk factors that could be modified to prevent the occurrence of chylothorax, and we seek to determine those factors. Retrospective chart review of 285 consecutive patients < 18 years who underwent cardiac surgery from 2015 to 2017 at a single institution pediatric intensive care unit. Data was collected on patient demographics, cardiac lesion, surgical and post-operative characteristics. Primary outcome was development of chylothorax. Of 285 patients, median age was 189 days, median weight was 6.6 kg, 48% were female, and 10% had trisomy 21. 3.5% of patients developed upper extremity DVTs, and 8% developed chylothorax. At 24 h following surgery, a majority were in the 0-10% fluid overload category or had a negative fluid balance (63% and 34%, respectively), and a positive fluid balance was rare at 72 h (16%). In univariate analysis, age, weight, bypass time, DVT, arrhythmia, and trisomy 21 were significantly associated with chylothorax and adjusted for in logistic regression. Presence of an upper extremity DVT (OR 49.8, p < 0.001) and trisomy 21 (OR 5.8, p < 0.001) remained associated with chylothorax on regression modeling. The presence of an upper extremity DVT and trisomy 21 were associated with the development of chylothorax. Fluid overload was rare in our population. The presence of positive fluid balance, fluid overload, elevated central venous pressure, and early initiation of fat containing feeds were not associated with chylothorax.
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Quilotórax/etiología , Síndrome de Down/complicaciones , Cardiopatías Congénitas/complicaciones , Trombosis de la Vena/complicaciones , Preescolar , Femenino , Cardiopatías Congénitas/cirugía , Humanos , Lactante , Modelos Logísticos , Masculino , Estudios Retrospectivos , Factores de Riesgo , Equilibrio Hidroelectrolítico/fisiologíaRESUMEN
OBJECTIVES: Ventricular assist devices (VADs) are associated with a mortality benefit in children. Database-driven analyses have associated VADs with reduction of modifiable risk factors (MRFs), but validation with institutional data is required. The authors studied MRF reduction on VAD and the influence of persistent MRFs on survival after heart transplant. METHODS: All patients at the authors' institution requiring a VAD at transplant (2011-2022) were retrospectively identified. MRFs included renal dysfunction (estimated glomerular filtration rate <60 mL/min/1.73 m2), hepatic dysfunction (total bilirubin ≥1.2 mg/dL), total parenteral nutrition dependence, sedatives, paralytics, inotropes, and mechanical ventilation. RESULTS: Thirty-nine patients were identified. At time of VAD implantation, 18 patients had ≥3 MRFs, 21 had 1 to 2 MRFs, and 0 had 0 MRFs. At time of transplant, 6 patients had ≥3 MRFs, 17 had 1 to 2 MRFs, and 16 had 0 MRFs. Hospital mortality occurred in 50% (3 out of 6) patients with ≥3 MRFs at transplant vs 0% of patients with 1 to 2 and 0 MRFs (P = .01 for ≥3 vs 1-2 and 0 MRFs). MRFs independently associated with hospital mortality included paralytics (1.76 [range, 1.32-2.30]), ventilator (1.59 [range, 1.28-1.97]), total parenteral nutrition dependence (1.49 [range, 1.07-2.07]), and renal dysfunction (1.31 [range, 1.02-1.67]). Two late mortalities occurred (3.6 and 5.7 y), both in patients with 1 to 2 MRFs at transplant. Overall posttransplant survival was significantly worse for ≥3 versus 0 MRFs (P = .006) but comparable between other cohorts (P > .1). CONCLUSIONS: VADs are associated with MRF reduction in children, yet those with persistent MRFs at transplant experience a high burden of mortality. Transplanting VAD patients with ≥3 MRFs may not be prudent. Time should be given on VAD support to achieve aggressive pre-transplant optimization of MRFs.
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Insuficiencia Cardíaca , Trasplante de Corazón , Corazón Auxiliar , Enfermedades Renales , Niño , Humanos , Corazón Auxiliar/efectos adversos , Estudios Retrospectivos , Trasplante de Corazón/efectos adversos , Factores de Riesgo , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/cirugía , Resultado del TratamientoRESUMEN
BACKGROUND: Children who undergo cardiac surgery may require post-cardiotomy extracorporeal membrane oxygenation (ECMO). Although morbidities are considerable, our understanding of outcome determinants is limited. We evaluated associations between patient and peri-operative factors with outcomes. METHODS: The STS Congenital Heart Surgery Database was queried for patients <18yo who underwent post-cardiotomy ECMO from 1/2016-6/2021. PRIMARY OUTCOME: survival to hospital discharge. SECONDARY OUTCOME: survival without neurologic injury. Logistic regression for binary outcomes and competing risk analysis for survival were used to identify the most important predictors. Variables were selected by stepwise procedure using entry level p=0.35. Those with p≤0.1 were kept in the final model. RESULTS: A total of 3,181 patients were supported with post-cardiotomy ECMO during the same hospitalization as cardiac surgery: (A) intra-operative initiation of ECMO, n=1206; (B) early post-operative (≤48 hours), n=936; (C) late post-operative (>48 hours), n=1039. Most common primary procedure of index operation was the Norwood procedure. 57% intra-op survived to discharge, versus 59% early post-op and 42% late post-op group (χ2 (2) = 64, p<0.0001, V = 0.14). In all groups, post-op septicemia, cardiac arrest, and new neurologic injury had the strongest association with mortality, while post-operative reintubation and unplanned non-cardiac reoperation were associated with higher survival. CONCLUSIONS: Multiple risk factors impact survival in children who undergo cardiac surgery and post-cardiotomy ECMO. ECMO initiated >48 hours after surgery is associated with the poorest outcomes. This is the first step in creating a predictive tool to educate clinicians and families regarding expectations in this high-risk population.
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INTRODUCTION: Outcomes of conventional cardiopulmonary resuscitation are improved when the initial rhythm is shockable (ventricular fibrillation or pulseless ventricular tachycardia). In children, the first documented rhythm is typically asystole or pulseless electrical activity. We evaluate the role the initial rhythm plays in outcomes for children undergoing extracorporeal cardiopulmonary resuscitation (ECPR) for in-hospital cardiac arrest. METHODS: Consecutive patients < 18 years with in-hospital ECPR events ≥ 10 minutes reported to the American Heart Association Get With The Guidelines® - Resuscitation registry from 2014 to 2019 were included. Primary outcome was survival to hospital discharge. Logistic regression modeling was used to compute propensity score matching based on patient, cardiac arrest event and hospital characteristics; patients with initial shockable rhythm were matched to patients with initial non-shockable rhythm. RESULTS: The final cohort included 466 patients, of which 82 (18%) had a shockable, and 384 (82%) had a non-shockable initial rhythm. After propensity score matching of 287 (62%) patients, there was no difference in survival to hospital discharge (risk ratio [RR] 1.2, 95% CI, 0.95-1.53, p = 0.13) or favorable neurologic outcome, defined as Pediatric Cerebral Performance Category (PCPC) of 1 or 2, or no decline from baseline (RR 1.28, 95% CI, 0.84-1.96, p = 0.25) between patients with and without shockable initial rhythm. CONCLUSIONS: In children with in-hospital cardiac arrest undergoing ECPR, there was no significant difference in survival or favorable neurologic outcome between those with initial shockable rhythm compared to non-shockable rhythm. Further investigation to evaluate ECPR patient characteristics and outcomes is warranted to help guide eligibility and ECMO deployment practices.
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Reanimación Cardiopulmonar , Paro Cardíaco , Humanos , Niño , American Heart Association , Paro Cardíaco/terapia , Sistema de Registros , Hospitales , Estudios RetrospectivosRESUMEN
Ventricular assist devices are increasingly used for patients with single ventricle physiology. We describe the use of durable, continuous flow, single ventricular assist device (SVAD) therapy in Fontan circulatory failure patients. Retrospective, single-center review of patients with Fontan circulation implanted with a SVAD between 2017 and 2022. Patient characteristics and outcomes were obtained by chart review. Nine patients underwent SVAD implantation (median age 24 years). Most patients had a total cavopulmonary connection; one had an atriopulmonary Fontan. Five patients had a systemic right ventricle. SVAD was most often utilized as bridge to candidacy (67%). Eight patients had at least moderate systemic ventricular systolic dysfunction. SVAD support continued for a median of 65 days (longest duration, 1,105 days) and one patient remains on support at time of submission. Of five patients discharged home, median length of stay after SVAD was 24 days. Six patients were transplanted (median 96 days from SVAD). Two patients died from pretransplant multisystem organ failure before transplant. All transplanted patients remain alive (median time since transplant 593 days). Continuous flow SVAD therapy can be effective for patients with Fontan circulatory failure and systolic dysfunction. Further studies should investigate feasibility and optimal SVAD timing with more advanced Fontan associated end-organ dysfunction.
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Meningococcal disease is associated with high mortality despite aggressive antibiotic therapy and intensive care support. Patients may develop refractory hypotension and acute respiratory distress syndrome in which extracorporeal membrane oxygenation (ECMO) could serve as a life-saving rescue therapy. However, there is limited data regarding the outcomes of ECMO support in the setting of meningococcal disease. This retrospective analysis of prospectively collected data from Extracorporeal Life Support Organization registry (1989-2019) enrolled children (29 days-18 years old) with Neisseria meningitidis infection receiving ECMO for any support type and mode. A total of 122 patients underwent a single course of ECMO support, equating to 122 ECMO runs. The overall survival-to-discharge rate was 46.7%. Patients receiving pulmonary venovenous (VV) ECMO had the highest survival-to-discharge of 85.7%, while those receiving venoarterial (VA) ECMO for pulmonary indications had a survival of 32.4%. Patients receiving VA ECMO support for cardiac indications had a survival-to-discharge rate of 60.9%. Those needing extracorporeal cardiopulmonary resuscitation (ECPR) had a poor survival (14.3%). Hemorrhagic complications were common, occurring in 43.4% of patients, but not found to be associated with mortality (complication was present in 47.7% of deceased and 38.6% of survivors, p = 0.31). Multivariable logistic regression analysis revealed that neurologic complications were associated with increased odds of mortality (odds ratio: 44.11; 95% confidence interval: 4.95-393.08). ECMO can be utilized as rescue therapy in children with refractory cardiopulmonary failure in setting of meningococcemia. Patients who require pulmonary VV or cardiac ECMO have the best ECMO outcomes. However, the use of ECMO in those suffering cardiac arrest (ECPR) should be undertaken with caution.
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Mechanical circulatory support (MCS) is a key therapy in the management of patients with severe cardiac disease or respiratory failure. There are two major forms of MCS commonly employed in the pediatric population-extracorporeal membrane oxygenation (ECMO) and ventricular assist device (VAD). These modalities have overlapping but distinct roles in the management of pediatric patients with severe cardiopulmonary compromise. The use of ECMO to provide circulatory support arose from the development of the first membrane oxygenator by George Clowes in 1957, and subsequent incorporation into pediatric cardiopulmonary bypass (CPB) by Dorson and colleagues. The first successful application of ECMO in children with congenital heart disease undergoing cardiac surgery was reported by Baffes et al. in 1970. For the ensuing nearly two decades, ECMO was performed sparingly and only in specialized centers with varying degrees of success. The formation of the Extracorporeal Life Support Organization (ELSO) in 1989 allowed for the collation of ECMO-related data across multiple centers for the first time. This facilitated development of consensus guidelines for the use of ECMO in various populations. Coupled with improving ECMO technology, these advances resulted in significant improvements in ECMO utilization, morbidity, and mortality. This article will review the use of ECMO in children with congenital heart disease.
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The SynCardia temporary total artificial heart (TAH-t) is a pneumatically driven pulsatile device that replaces a patient's ventricles and all four valves. It is now available in two sizes and can be used in patients with sufficient chest size as a bridge to transplant or destination therapy. We describe our experience at a children's hospital with the TAH-t. Seven patients implanted with a TAH-t at Cincinnati Children's Hospital Medical Center from November 2012 to March 2021 were included. Duration of support ranged from 10 to 414 days with a median of 27 days. There was a trend toward decreased time to extubation and intensive care unit (ICU) length of stay, with the three most recent patients being extubated on postoperative day 1. Seventy-one percent of the patients were successfully transplanted following their TAH-t placement. Children and young adults with transplant graft failure requiring durable, biventricular support with a TAH-t had superior outcomes (75% survival to retransplant) than previously described.
Asunto(s)
Insuficiencia Cardíaca , Trasplante de Corazón , Corazón Artificial , Adulto Joven , Niño , Humanos , Insuficiencia Cardíaca/terapia , HospitalesRESUMEN
BACKGROUND: Defects in the RYR1 (OMIM#180901) gene lead to Ryanodine receptor type 1-related myopathies (RYR1-RM); the most common subgroup of congenital myopathies. METHODS: Congenital myopathy presents a diagnostic challenge due to the need for multiple testing modalities to identify the many different genetic etiologies. In this case, the patient remained undiagnosed after whole-exome sequencing (WES), chromosomal microarray, methylation analysis, targeted deletion and duplication studies, and targeted repeat expansion studies. Clinical whole-genome sequencing (WGS) was then pursued as part of a research study to identify a diagnosis. RESULTS: WGS identified compound heterozygous RYR1 intronic variants, RNA sequencing confirmed both variants to be pathogenic causing RYR1-RM in a phenotype of severe congenital hypotonia with respiratory failure from birth, neonatal brain hemorrhage, and congenital heart disease involving transposition of the great arteries. CONCLUSION: While there is an ongoing debate about the clinical superiority of WGS versus WES for patients with a suspected genetic condition, this scenario highlights a weakness of WES as well as the added cost and delay in diagnosis timing with having WGS follow WES or even ending further genetic testing with a negative WES. While knowledge gaps still exist for many intronic variants, transcriptome analysis provides a way of validating the resulting dysfunction caused by these variants and thus allowing for appropriate pathogenicity classification. This is the second published case report of a patient with pathogenic intronic variants in RYR1-RM, with clinical RNA testing confirming variant pathogenicity and therefore the diagnosis suggesting that for some patients careful analysis of a patient's genome and transcriptome are required for a complete genetic evaluation. The diagnostic odyssey experienced by this patient highlights the importance of early, rapid WGS.