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The use of allogeneic blood products to restore hemostasis during pediatric cardiac surgery is associated with major risks. Consequently, there has been a growing interest in new patient blood management strategies, such as those based on the use of fibrinogen concentrate (FC). Accumulating evidence has shown FC supplementation to be safe and effective. Nevertheless, no guidelines are available on using FC in the pediatric setting, and few objective evaluations have been provided in clinical practice. The endpoint of this monocenter retrospective study was the hemostatic effect of additional FC in infants undergoing complex cardiac surgery with cardiopulmonary bypass to manage persistent clinically relevant bleeding. After weaning from cardiopulmonary bypass and after protamine administration, patients were transfused with conventional allogeneic products such as packed red blood cells, fresh frozen plasma (FFP), and platelets. In the case of redo surgery, according to the institutional protocol, patients also received tranexamic acid. In case of clinically persistent relevant bleeding, according to the anesthesiologist's judgment and thromboelastography, patients received FC supplementation (group with FC) or further FFP transfusions without receiving FC supplementation (group without FC). The primary endpoint was the hemostatic effects of FC. Secondary endpoints were the functional hypofibrinogenemia threshold value (expressed as maximum amplitude fibrinogen, MA-Fib) and postoperative MA-Fib, fibrinogenemia, intraoperative transfusions, and adverse events (AEs). In total, 139 patients who underwent cardiac surgery with CPB and aged less than 2 years were enrolled: 70 patients received allogeneic blood products and FC supplementation (group FC); 69 patients received allogeneic products without FC supplementation (group without FC). Patients that received FC supplementation were characterized by a significantly longer time of extracorporeal circulation (p < 0.001) and aortic cross-clamping (p < 0.001), a significantly lower minimum temperature (p = 0.011), increased use of concentrated prothrombin complex (p = 0.016) and tranexamic acid (p = 0.010), and a significantly higher amount of packed red blood cells, platelets (p < 0.001) and fresh frozen plasma (p = 0.03). Postoperative bleeding and severe bleeding were not statistically different between patients treated with FC and those not treated with FC supplementation (p = 0.786 and p = 0.695, respectively); after adjustment, a trend toward reduced bleeding can be observed with FC (p = 0.064). Overall, 88% of patients with severe bleeding had MA-Fib < 10 mm; a moderate association between severe bleeding and MA-Fib (odds ratio 1.7, 95% CI 0.5-6.5, p = 0.425) was found. Increased MA-Fib and postoperative fibrinogen were higher in the FC group (p = 0.003 and p < 0.001, respectively) than in FFP. AEs in the FC group were comparable to those observed in less complicated surgeries. Our results suggest a potential role of FC in complex surgery in maintaining postoperative bleeding at a level comparable to less complicated surgical procedures and favoring the increase in postoperative MA-Fib and fibrinogen.
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Deranged cerebral autoregulation (CA) is associated with worse outcome in adult brain injury. Strategies for monitoring CA and maintaining the brain at its 'best CA status' have been implemented, however, this approach has not yet developed for the paediatric population. This scoping review aims to find up-to-date evidence on CA assessment in children and neonates with a view to identify patient categories in which CA has been measured so far, CA monitoring methods and its relationship with clinical outcome if any. A literature search was conducted for studies published within 31st December 2022 in 3 bibliographic databases. Out of 494 papers screened, this review includes 135 studies. Our literature search reveals evidence for CA measurement in the paediatric population across different diagnostic categories and age groups. The techniques adopted, indices and thresholds used to assess and define CA are heterogeneous. We discuss the relevance of available evidence for CA assessment in the paediatric population. However, due to small number of studies and heterogeneity of methods used, there is no conclusive evidence to support universal adoption of CA monitoring, technique, and methodology. This calls for further work to understand the clinical impact of CA monitoring in paediatric and neonatal intensive care.
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In current clinical practice, transplant clinicians create collaborative working relationships with histocompatibility laboratory scientists to identify the risk of long-term graft failure, which may assist in establishing strategies for treatment and surveillance. Transplant immunology research also focuses on optimizing human leukocyte antibody tissue typing and defines the most effective test for detecting the presence of donor-specific antibodies. Although several studies have been conducted, data on pediatric heart transplant recipients are limited. Epitope load information may be utilized to identify donors with permissible human leukocyte antibody mismatches to increase transplant success. Although current guidelines do not consider human leukocyte antibody epitope-based matching tools, these guidelines could be useful for identifying recipients at a high risk of donor-specific antibody production, which would be appropriate for routine donor-specific antibody screening to initiate early interventions to prevent antibody-mediated rejection. Human leukocyte antibody matching at the epitope level offers an effective approach for identifying acceptable mismatches in sensitized patients and provides information about epitope loads. In the future, eplet matching may be used to define the best immunosuppressive therapy protocol for cardiothoracic organ transplantation. This report provides an overview of the role of human leukocyte antibodies in heart and lung transplantation.
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Anticuerpos , Donantes de Tejidos , Humanos , Niño , Alelos , Selección de Donante , EpítoposRESUMEN
Thromboelastography (TEG) is a point-of-care test (POCT) used to analyze the hemostatic properties of whole blood. TEG® 5000and TEG® 6s (Haemonetics Corp, USA) measure the same parameters describing clot viscoelasticity using different methodologies. The purpose of this study was to evaluate agreement between TEG5000 and TEG6s measurements. We analyzed prospectively collected tests resulting from paired blood samples in cardiac surgery pediatric patients at one hour (T0) and 24 h (T1) postoperatively. Each citrated sample was utilized for TEG® 5000 and TEG ®6s. Six specific TEG parameters were analyzed and compared: R kaolin time (RK), R kaolin heparinase (RKH) time, K kaolin time (KK), K kaolin heparinase time KH (KKH), Maximum Amplitude kaolin (MAK), Maximal Amplitude Kaolin Heparinase (MAKH). We enrolled 30 patients. Median (interquartile range) patients' age was 206 (20-597) days. All surgical patients underwent correction except 5 who were palliated. At T0, RK and RKH showed an average (standard deviation) % bias of 15.8 (31) and 16.1 (28), respectively, with similar results at T1. A % bias of -6 (23) and - 6 [15] in MAK was found at T0 and T1, respectively. Similarly, MAKH % bias was 1.5 (22) and 7.6 (29) at T0 and T1, respectively. At both timepoints, low % biases (< ± 6%) were demonstrated in KK and KKH. All parameters showed improved coagulation from T0 to T1, but without significant interaction between type of device and time. Analysis of the entire pool of 60 paired samples showed no agreement in diagnostic performance (within the range vs. outside the range) in 12 (20%), 5 (9.8%), 1 (1.7%), 4 (7.8%), 9 (15%), and 5 (9.8%) cases for RK, RKH, MAK, MAKH, KK and KKH, respectively. We observed substantial agreement in MAK and KK in a cohort of pediatric patients undergoing uncomplicated cardiac surgery. Our findings suggest that TEG®5000 and TEG®6s are interchangeable for assessing these parameters.
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OBJECTIVES: The aim of this study was to elucidate predictors of death and reintervention after mitral valve (MV) surgery in children. METHODS: A single-centre retrospective study was performed enrolling 142 patients younger than 18 years who underwent primary index surgical mitral repair or replacement at Bambino Gesù Children's Hospital in Rome from July 1982 to April 2020. Patients with complete, transitional or partial atrioventricular septal defect and patients with single ventricle physiology were excluded. Patients were stratified according to the age group: group 1 (<1 year old), group 2 (1-5 years old) and group 3 (>5 years old). The composite primary outcome was freedom from death or transplant. The secondary outcome was freedom from redo MV surgery. RESULTS: Transplant-free survival was 89% at 5 years and 88% at 10 years. Stratified by age, group 1 had poorer outcome in comparison with other groups (log-rank test P = 0.105). Both univariate and multivariate analyses showed that age <1 year was a significant risk factor for death or transplant (P = 0.044). Age <1 year was associated with increased risk of reoperation (aHR = 3.38, P = 0.009), while the presence of genetic syndrome (aHR = 0.22) and preoperative EF% (aHR = 0.97) were protective factors for reoperation. CONCLUSIONS: The overall survival and freedom from reoperation in children undergoing MV surgery still need improvements. Younger age was a significant risk factor for death and reintervention both after repair and replacement of the MV. In particular, infants and neonates have a three-fold risk for death compared to children.
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Procedimientos Quirúrgicos Cardíacos , Implantación de Prótesis de Válvulas Cardíacas , Insuficiencia de la Válvula Mitral , Niño , Preescolar , Estudios de Seguimiento , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Humanos , Lactante , Recién Nacido , Válvula Mitral/cirugía , Insuficiencia de la Válvula Mitral/cirugía , Reoperación , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Deletions involving the distal portion of the short arm of chromosome 8(8p23.1) show a high phenotypic variability. Congenital heart diseases (CHD) are often described. GATA4 when mutated or deleted is reported to be involved in cardiac morphogenesis. Only twice, left ventricular non compaction (LVNC) was reported in literature in association with 8p23.1 deletion. The present cohort includes five new patients with 8p23.1 deletions including GATA4. The spectrum of CHD is variable. Moreover, in four patients, LV hypertrabeculation was detected and in the fifth LVNC was recognized. Literature revision identified 45 patients with 8p23.1 deletions (encompassing GATA4) and heart involvement. It included wide spectrum of CHD including: heterotaxy spectrum 7/45 (15, 6%), atrioventricular canal 14/45 (balanced 3/45 including two of them with hypoplastic aortic arch; unbalanced 4/45, Fallot-AVC 1/45, partial AVC 3/45, unspecified 3/45), predominant major left heart lesions included 2/45 (4, 4%): interrupted aortic arch and hypoplastic left heart syndrome. Left ventricular hypertrabeculation might be potentially underestimated in patients with 8p23.1 deletion. These might suggest the importance of including microarray analysis in this group of patients. Moreover, 8p23.1 microdeletion or GATA4 variants can be considered in heterotaxy genetic panels.
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Factor de Transcripción GATA4 , Cardiopatías Congénitas , Deleción Cromosómica , Factor de Transcripción GATA4/genética , Corazón , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/genética , Ventrículos Cardíacos , HumanosRESUMEN
Warfarin is prescribed in patients with ventricular assist devices (VADs). Dosage depends on several factors including the underlying genotype. These include polymorphisms of genes encoding cytochrome P450 enzymes, the main ones being CYP2C9, VKORC1, and CYP4F2. The objectives of this study were to evaluate the prevalence of CY2CP9 1*2*3*, VKORC1, and CYP4F2 in children with VADs and the time to reach the target international normalized ratio. We performed a retrospective/prospective study in children with VADs. We recorded polymorphisms, disease, type of VAD, ethnicity, age, gender, height, weight, INR values, bleeding, and thromboembolic episodes. Informed consent was obtained. We enrolled 34 children (19 male, 15 female), with a median age of 2 years (range 0.3-17 years) and median weight of 6.9Kg. The Berlin Heart was the most commonly implanted VAD (22/34; 64%), and the most common diagnosis was dilated cardiomyopathy. Statistical analysis confirmed a significant partial correlation with VKORC1 CC (p = 0.019). The CYP2C9*2 CT genotype showed a late rise in target INR values (p = 0.06), while the CYP2C9*2 CC showed a tendency toward an early INR rise (p = 0.024). We provide new information on the contribution of the warfarin polymorphisms in children with VAD implantation. Pharmacogenomic dosing for children using warfarin has the potential to improve clinical care in VAD patients. Patients with the CYP2C9*2 CT genotype may need more time or higher doses to reach target INR, while clinicians may need to be aware of the potential for a rapid rise in INR in patients with the CYP2C9*2 CC genotype.
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Anticoagulantes/administración & dosificación , Corazón Auxiliar , Warfarina/administración & dosificación , Adolescente , Anticoagulantes/metabolismo , Niño , Preescolar , Sistema Enzimático del Citocromo P-450/genética , Relación Dosis-Respuesta a Droga , Femenino , Humanos , Lactante , Relación Normalizada Internacional , Masculino , Farmacogenética , Polimorfismo Genético , Estudios Prospectivos , Estudios Retrospectivos , Vitamina K Epóxido Reductasas/genética , Warfarina/metabolismoRESUMEN
OBJECTIVES: Management of resternotomy is often a difficult challenge in patients with congenital diseases who have undergone multiple sternotomies. Our goal was to report our single-centre experience with carotid cannulation using a graft interposed during redo sternotomy in complex cardiac reintervention procedures. METHODS: We performed a retrospective review of all patients who had undergone complex redo sternotomies between January 2019 and May 2020 utilizing a cervical cannulation technique with a Gore-Tex graft interposed on the carotid artery. We classified our population study on the basis of the primary diagnosis and the type of surgery. The primary outcomes of our analysis were the evaluation of the safety of the procedure in terms of survival and freedom from neurological events. RESULTS: We analysed 22 patients who had undergone previous complex operations. The median age and weight at the time of reintervention were 130.35 (range 0.46-435) months and 31.5 (range 2.2-85) kg, respectively. Composite graft carotid cannulation provided adequate arterial flow in all patients with a median arterial flow of 3.5 l/min/m2 (range 0.6-6). One major cardiac injury occurred during sternotomy when emergency cardiopulmonary bypass (CPB) was initiated. Moreover, during their hospital stays, all patients had an uneventful recovery without neurological or vascular complications and no cervical wound infections. CONCLUSIONS: Carotid cannulation using interposition of a side graft on the common carotid artery for arterial inflow is a reliable and safe method for initiation of CPB in complex redo surgeries in patients with congenital disease. Complications directly associated with this type of cannulation are uncommon and allow surgical re-entry with overall low risks.
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Procedimientos Quirúrgicos Cardíacos , Esternotomía , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Puente Cardiopulmonar , Arteria Carótida Común , Cateterismo , Humanos , Reoperación , Estudios Retrospectivos , Esternotomía/efectos adversosRESUMEN
OBJECTIVES: The aim of this study was to identify the predictors of death and of reintervention after mitral valve replacement (MVR) in children. METHODS: A single-centre retrospective study was performed including 115 patients under the age of 18 undergoing MVR between 1982 and 2019. For all patients, the ratio of prosthetic valve size (diameter in mm) to weight (kg) at surgery was calculated and long-term result was assessed. The primary outcome was freedom from mitral valve (MV) re-replacement. The composite secondary outcome was freedom from death or transplant. RESULTS: Fifty-four patients had a previous surgical attempt to MV repair. The median age at surgery was 5.5 years (interquartile range 1.21-9.87). Death/transplant-free survival was 77 ± 4% at 5 years and 72 ± 5% at 10 years. Univariate analysis showed a size/weight ratio higher than 2 and age <2 years as significant risk factors for death or transplant. Freedom from MV re-replacement at 5 and 10 years was 90 ± 3% and 72 ± 6%, respectively. Biological prosthesis implanted at first replacement (P = 0.007) and size/weight ratio higher than 2 (P = 0.048) were predictors of reoperation. Significant upsizing (P < 0.0001) of mitral prosthesis was observed at re-replacement. CONCLUSIONS: MVR is a viable strategy in children with unrepairable MV disease. Mortality can be predicted based on size/weight ratio and age <2 years. MV re-replacement can be performed with low morbidity and mortality and a larger-size prosthesis can often be placed at the time of redo.
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Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Niño , Preescolar , Humanos , Válvula Mitral/cirugía , Reoperación , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVES: Ventricular-arterial coupling represents the interaction between the left ventricle and the arterial system. Ventricular-arterial coupling is measured as the ratio between arterial elastance and ventricular end-systolic elastance. Scant information is available in critically ill children about these variables. The aim of this study was to prospectively assess ventricular-arterial coupling after pediatric cardiac surgery and evaluate its association with other commonly recorded hemodynamic parameters. DESIGN: Single-center retrospective observational study. SETTING: Pediatric cardiac surgery operating room. PATIENTS: Children undergoing corrective cardiac surgery. INTERVENTIONS: Hemodynamic monitoring with transesophageal echocardiography. MEASUREMENTS AND MAIN RESULTS: Twenty-seven patients with biventricular congenital heart disease, who underwent elective cardiac surgery with cardiopulmonary bypass, were enrolled before operating room discharge. Chen single-beat modified method was applied to calculate ventricular-arterial coupling. The median arterial elastance and end-systolic elastance values were 5.9 mm Hg/mL (2.2-9.3 mm Hg/mL) and 4.3 mm Hg/mL (1.9-8.3 mm Hg/mL), respectively. The median ventricular- arterial coupling was 1.2 (1.1-1.6). End-systolic elastance differences between patients with a ventricular-arterial coupling below (low ventricular-arterial coupling) and above (high ventricular-arterial coupling) the median value were -5.2 (95% CI, -6.28 to -0.7; p = 0.008). Differently, arterial elastance differences were -2.1 (95% CI, -5.7 to 1.6; p = 0.19). Ventricular-arterial coupling showed a significant association with pre-ejection time (r, 0.44; p = 0.02), total ejection time (r, -0.41; p = 0.003), cardiac cycle efficiency (r, -0.46; p = 0.02), maximal delta pressure over delta time (r, -0.44; p = 0.02), ejection fraction (r, -0.57; p = 0.01), and systemic vascular resistances indexed (0.56; p = 0.003). After adjustment, total ejection time (p = 0.001), pre-ejection time (p = 0.02), and ejection fraction (p = 0.001) remained independently associated with ventricular-arterial coupling. CONCLUSIONS: Median ventricular-arterial coupling values in children after cardiac surgery appear high (above 1). Uncoupling was particularly evident in high ventricular-arterial coupling patients who showed the lowest end-systolic elastance values (but not significantly different arterial elastance values) compared with low ventricular-arterial coupling. Ventricular-arterial coupling appears to be inversely proportional to pre-ejection time, total ejection time, and ejection fraction.
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Puente Cardiopulmonar/efectos adversos , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/diagnóstico por imagen , Rigidez Vascular , Gasto Cardíaco Bajo/diagnóstico , Preescolar , Ecocardiografía , Ventrículos Cardíacos/cirugía , Humanos , Lactante , Recién Nacido , Estudios RetrospectivosAsunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Diafragma/diagnóstico por imagen , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Diafragma/fisiopatología , Humanos , Lactante , Complicaciones Posoperatorias/diagnóstico por imagen , Complicaciones Posoperatorias/etiología , Respiración ArtificialRESUMEN
The aim of this study was to describe the echocardiographic trend of left ventricular (LV) and right ventricular (RV) function after implantation of a pulsatile flow left ventricular assist device (LVAD) in children. From 2013 to 2016, we prospectively evaluated 13 consecutive pediatric Berlin Heart EXCOR LVAD patients. Clinical and echocardiographic data were collected at baseline, within 24 h after implantation and monthly until LVAD explant. Median age and weight at the implantation was 8 (4-23) months and 5 (4.6-8.3) kg at the time of implantation, respectively. All were affected by dilated cardiomyopathy. Average LVAD support time was 226.2 ± 121.2 days. Nine (70%) were transplanted, 4 (30%) died. LV end-systolic and end-diastolic volumes were reduced until the follow up of two months (P = 0.019 and P = 0.001). A progressive increase in RV dimensions was observed. After 4 months of follow up, RV fractional area change worsening was statistically related with the deterioration of LV unloading (P = 0.0036). Four patients needed prolonged inotropic support for RV failure. Pulsatile LVAD in pediatrics is followed by an early and mid-term LV unloading, as expressed by a decrease in LV volumes and diameters at echocardiogram. The effects of unloading do not remain stable at long term follow up. RV function improved in the acute phase, but a progressive dilatation of RV was noted over time. In some patients, RV failure might lead to the need of an increase of inotropic support at long term follow up.
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Cardiomiopatía Dilatada/cirugía , Insuficiencia Cardíaca/terapia , Ventrículos Cardíacos/fisiopatología , Corazón Auxiliar/efectos adversos , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/mortalidad , Cardiomiopatía Dilatada/fisiopatología , Cardiotónicos/uso terapéutico , Ecocardiografía , Femenino , Estudios de Seguimiento , Insuficiencia Cardíaca/diagnóstico por imagen , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Lactante , Masculino , Estudios Prospectivos , Flujo Pulsátil , Función Ventricular Izquierda/fisiología , Función Ventricular Derecha/fisiologíaRESUMEN
OBJECTIVE: We report our experience with the use of a left ventricular assist device (LVAD) as destination therapy (DT) for the management of patients with cardiac end-stage dystrophinopathies. METHODS: From February 2011 to February 2016, 7 patients with dystrophinopathies and dilated cardiomyopathy (DCM) were treated with LVADs at our institution. Median age at surgery was 16.5 years (range, 14.2-23.4 years). All patients were preoperatively evaluated by a multidisciplinary team approach. RESULTS: All patients survived to hospital discharge. The early postoperative course was characterized by abdominal bleeding (1 patient) and retropharyngeal bleeding (1 patient). Because of abdominal or retropharyngeal bleeding, both required postoperative heparin infusion discontinuation for 35 and 33 days, respectively. Among the late complications, 1 child developed osteolysis and infection at the pedestal site of the device, which required surgical displacement; 1 patient required gastrostomy as a result of poor feeding, and another had a cerebral stroke, which was treated with percutaneous thrombus aspiration. The other 2 patients did not show early or late complications. At a median follow-up time of 21.7 months (range, 3-45 months) there have been 3 deaths: 1 patient died of a lung infection after 45 months, 1 died of tracheal bleeding after 29 months, and 1 died of cerebral hemorrhage after 14 months. CONCLUSIONS: Our experience indicates that the use of an LVAD as DT in patients with dystrophinopathies with end-stage DCM is feasible, suggesting that it may be suitable as a palliative therapy for the treatment of these patients with no other therapeutic options.
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Cardiomiopatía Dilatada/cirugía , Insuficiencia Cardíaca/cirugía , Ventrículos Cardíacos/fisiopatología , Corazón Auxiliar , Esternotomía/métodos , Volumen Sistólico/fisiología , Adolescente , Cateterismo Cardíaco , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/fisiopatología , Ecocardiografía , Femenino , Estudios de Seguimiento , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Imagen por Resonancia Cinemagnética , Masculino , Cuidados Paliativos , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Función Ventricular Izquierda/fisiología , Adulto JovenRESUMEN
OBJECTIVE: To analyze the effectiveness and the results of the use of a vacuum-assisted closure (VAC) system for the treatment of complex sternal wounds in newborns after cardiac surgery. METHODS: From May 2008 until December 2012, six patients developed post-sternotomy wound problems (large defects of epithelialization or mediastinitis), which were treated with a VAC system. Median age at the time of institution of VAC was 24.5 days (range 16 to 65 days). Median time of treatment was 14 days (range 3 to 42 days). RESULTS: All patients were newborns and all underwent delayed sternal closure after cardiac surgery. The indications for using the VAC system were: mediastinitis in two patients (33.3%) and impairment of healing without signs of infection in four (66.7%). All children after VAC therapy achieved healing of the sternal wound. VAC therapy was started with high negative pressures (-125 mmHg) continuously then switched to an intermittent modality in all patients. CONCLUSION: VAC system with high negative pressure is safe, effective, and is a well-tolerated therapy in newborns with complex sternal wounds.
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Procedimientos Quirúrgicos Cardíacos/métodos , Mediastinitis/terapia , Terapia de Presión Negativa para Heridas/métodos , Complicaciones Posoperatorias/terapia , Esternotomía , Dehiscencia de la Herida Operatoria/terapia , Factores de Edad , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Factores de Tiempo , Resultado del Tratamiento , Cicatrización de HeridasRESUMEN
Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder, characterized by progressive skeletal muscle weakness, loss of ambulation, and death secondary to cardiac or respiratory failure. End-stage dilated cardiomyopathy (DCM) is a frequent finding in DMD patients, they are rarely candidates for cardiac transplantation. Recently, the use of ventricular assist devices as a destination therapy (DT) as an alternative to cardiac transplantation in DMD patients has been described. Preoperative planning and patient selection play a significant role in the successful postoperative course of these patients. We describe the preoperative, intraoperative and postoperative management of Jarvik 2000 implantation in 4 DMD pediatric (age range 12-17 years) patients. We also describe the complications that may occur. The most frequent were bleeding and difficulty in weaning from mechanical ventilation. Our standard protocol includes: 1) preoperative multidisciplinary evaluation and selection, 2) preoperative and postoperative non-invasive ventilation and cough machine cycles, 3) intraoperative use of near infrared spectroscopy (NIRS) and transesophageal echocardiography, 4) attention on surgical blood loss, use of tranexamic acid and prothrombin complexes, 5) early extubation and 6) avoiding the use of nasogastric feeding tubes and nasal temperature probes. Our case reports describe the use of Jarvik 2000 as a destination therapy in young patients emphasizing the use of ventricular assist devices as a new therapeutic option in DMD.
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Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/cirugía , Corazón Auxiliar , Distrofia Muscular de Duchenne/complicaciones , Adolescente , Cardiomiopatía Dilatada/etiología , Cardiomiopatía Dilatada/cirugía , Niño , Ventrículos Cardíacos/cirugía , Humanos , Masculino , Resultado del TratamientoRESUMEN
OBJECTIVES: The aim of this study was to compare high-flow nasal cannula (HFNC) and conventional O2 therapy (OT) in paediatric cardiac surgical patients; the primary objective of the study was to evaluate whether HFNC was able to improve PaCO2 elimination in the first 48 h after extubation postoperatively. METHODS: We conducted a randomized, controlled trial in pediatric cardiac surgical patients under 18 months of age. At the beginning of the weaning of ventilation, patients were randomly assigned to either of the following groups: OT or HFNC. Arterial blood samples were collected before and after extubation at the following time points: 1, 6, 12, 24 and 48 h. The primary outcome was comparison of arterial PaCO2 postextubation; secondary outcomes were PaO2 and PaO2/fractional inspired oxygen (FiO2) ratio, rate of treatment failure and need of respiratory support, rate of extubation failure, rate of atelectasis, simply to complications and the length of paediatric cardiac intensive care unit stay. RESULTS: Demographic and clinical variables were comparable in the two groups. Analysis of variance for repeated measures showed that PaCO2 was not significantly different between the HFNC and OT groups (P = 0.5), whereas PaO2 and PaO2/FiO2 were significantly improved in the HFNC group (P = 0.01 and P = 0.001). The rate of reintubation was not different in the two groups (P = 1.0), whereas the need for noninvasive respiratory support was 15% in the OT group and none in the HFNC group (P = 0.008). CONCLUSIONS: HFNC had no impact on PaCO2 values. The use of HFNC appeared to be safe and improved PaO2 in paediatric cardiac surgical patients.
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Extubación Traqueal , Procedimientos Quirúrgicos Cardíacos , Catéteres , Cardiopatías Congénitas/cirugía , Pulmón/fisiopatología , Terapia por Inhalación de Oxígeno/instrumentación , Respiración , Desconexión del Ventilador , Factores de Edad , Análisis de los Gases de la Sangre , Diseño de Equipo , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/fisiopatología , Humanos , Lactante , Unidades de Cuidado Intensivo Pediátrico , Italia , Tiempo de Internación , Terapia por Inhalación de Oxígeno/efectos adversos , Cuidados Posoperatorios , Estudios Prospectivos , Recuperación de la Función , Factores de Tiempo , Resultado del TratamientoRESUMEN
OBJECTIVES: Haemolysis is known to occur during surgery on cardiopulmonary bypass (CPB) and to be responsible for kidney injury. The aim of this study was to assess, in a cohort of infants, the reference levels of free haemoglobin (fHb) and their change over time postoperatively; the predicting variables of haemolysis in the intraoperative phase; and the association between fHb and renal function. METHODS: A retrospective analysis in infants undergoing surgery on CPB was conducted. Children with preoperative renal dysfunction and need for extracorporeal membrane oxygenation support were excluded. fHb was sampled before and after CPB and on the first 2 postoperative days (POD). RESULTS: Twenty-two patients with a median (interquartile) age of 111 (63-184) days and Aristotle score of 8 (6.4-9) were enrolled. fHb had a baseline value of 29 (24-41) mg/dl, peaked to 75 (65-109) mg/dl at CPB weaning and returned to 35 (30-55) mg/dl on POD 2 (P <0.0001). The median normalized index of haemolysis was 0.15 (0.09-0.19) g of fHb per 100 l of pumped blood. A multivariable regression model showed that, at CPB weaning, fHb levels were independently associated with left atrial venting flow (P = 0.02), and that CPB time remained the only independent variable (P = 0.034), when left atrial venting was excluded from the analysis. Acute kidney injury (AKI) occurred in 10 patients (45%). fHb levels in the 48 post-CPB hours were not significantly different between AKI and non-AKI patients: However, a significant correlation was present between creatinine on POD1 and CPBw-fHb (r = 0.48; P = 0.045); and between cystatin C on POD1 and CPBw-fHb (r = 0.58; P = 0.02). CONCLUSIONS: A high rate of fHb is released during paediatric surgery with CPB in infants. fHb mainly depends on the left atrial venting flow rate and CPB duration. However, such peaks of fHb levels were not associated with renal dysfunction.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/efectos adversos , Puente Cardiopulmonar/efectos adversos , Hemoglobinas/metabolismo , Hemólisis , Lesión Renal Aguda/sangre , Lesión Renal Aguda/etiología , Factores de Edad , Biomarcadores/sangre , Creatinina/sangre , Cistatina C/sangre , Femenino , Humanos , Lactante , Recién Nacido , Modelos Lineales , Masculino , Análisis Multivariante , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Regulación hacia ArribaRESUMEN
Bleeding during and after cardiac surgery is a major issue in pediatric patients. A prospective cohort study was conducted to evaluate the effect of a commercially available prothrombin complex (Confidex) administered in cardiac surgery after weaning from cardiopulmonary bypass of infants with nonsurgical bleeding. In this study, 14 patients younger than 1 year received a Confidex bolus and were matched with 11 patients of a similar age who did not receive the drug. The preoperative coagulation profile was similar in the two groups. No side effects, including anaphylaxis or thrombotic events, were observed. The numbers of units of packed red blood cells and fresh frozen plasma administered both intra- and postoperatively were similar. The postoperative coagulation examination results and thromboelastographic parameters did not differ significantly between the two groups. However, the Confidex patients bled significantly less than the control subjects during the first 24 postoperative hours. The median volume of drained blood was 0.0 ml/kg h (range 0-1.9 ml/kg h) compared with 1.9 ml/kg h (range 1-3 ml/kg h) (p = 0.009). At least one unit of packed red blood cells in the postoperative phase was required by 2 patients (14 %) in the Confidex group and six patients (54 %) in the control group (odds ratio [OR], 0.13; 95 % confidence interval [CI], 0.02-0.9; p = 0.03). The median duration of mechanical ventilation was 3 days (range 2-4 days) in the Confidex group and 4 days (range 0-8 days) in the control group (p = 0.66). The median stay in the intensive care unit was 6 days (range 5-9 days) in the Confidex group and 7 days (range 4-12 days) in the control group (p = 0.88). The use of Confidex for infants undergoing cardiac surgery was safe and effective. It reduced postoperative bleeding and allowed fewer units of packed red blood cells to be infused in the postoperative phase without major side effects.