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IntroductionEx vivo perfusion (EVP) in heart transplantation (HTX) enables transportation of explanted organs over longer distances as well as monitoring of cardiac metabolism under unloaded coronary perfusion, thereby expanding the possible pool of suitable donors for HTX. We aimed at identifying possibly modifiable risk factors predicting impaired outcome after HTX through analysis of perfusion parameters, donor and recipient characteristics.MethodsWe included 64 patients after HTX using EVP (TransMedics Organ Care System) between February 2010 and October 2022 in this retrospective, single-center analysis. Blood gas analyses were taken during EVP in a standardized manner.ResultsHTX recipients were 44.8 ± 16.2 years old, six patients being younger than 18 years at the time of transplantation. High urgency listing status was present in 79.7%, and 67.2% had previous cardiac surgery. Lower base excess during EVP predicted severe primary graft dysfunction (PGD) (-5.55 ± 1.95 vs -3.94 ± 2.38 mmol/L, p = 0.043). With every additional minute of warm ischemia, the risk of PGD was 3.7% higher (OR 1.037, 95% CI [1.004; 1.072], p = 0.028). Low donor serum potassium (3.4 ± 0.76 mmol/L vs 4.2 ± 0.60, p = 0.008), high glucose after EVP priming (207.63 ± 99.05 mg/dL vs 146.45 ± 51.99, p = 0.045), and resuscitation of the donor (55.6% vs 18.2%, p = 0.027) were associated with impaired survival after HTX.ConclusionsEVP provides a unique method to assess graft function during the retrieval of hearts for cardiac transplantation. Low base excess, priming glucose, warm ischemia and donor hypokalaemia influence the frequency of adverse outcomes after HTX. Future studies may address possible strategies to safely modify these risk factors.
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BACKGROUND: Extracorporeal membrane oxygenation (ECMO) can influence pharmacokinetics. We investigated the vancomycin dosage in children on ECMO compared to critically ill children to determine the necessary dosage adjustment on ECMO. METHODS: Eight-year, single-center, retrospective cohort study at a tertiary heart center's pediatric cardiac intensive care unit (ICU) of children undergoing ECMO support. Our control group (non-ECMO) was critically ill children with delayed sternal closure after cardiac surgery. We included consecutively all children undergoing vancomycin administration. The starting dose was 10 to 15 mg/kg BW per dose, every 8 to 12 hours depending on age. The vancomycin trough level was maintained in the 10 to 20 µg/ml range. RESULTS: 85 total courses on ECMO and 99 non-ECMO courses were included. The ECMO group's daily vancomycin dose was significantly lower than non-ECMO's at a median of 33.3 and 38.5 mg/kg/d, respectively (p < 0.001). Vancomycin serum trough levels were similar between groups and within the target range. The ECMO group's daily vancomycin dose dropped faster over time, with a dose on day 3 of 28.7 and 33.7 mg/kg/d, respectively. The impact of renal function on vancomycin dosing was more apparent in the ECMO group. If the renal function was reduced at the start of treatment, the vancomycin dose was lower in the ECMO group compared to the non-ECMO group with renal impairment (22.5 vs. 42.1 mg/kg/d; p < 0.001). When renal function was normal, the doses were similar between groups. CONCLUSION: In children on ECMO with impaired renal function at treatment initiation, lower vancomycin doses were necessary. Early therapeutic drug monitoring, even before reaching a steady state, should be considered.
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Antibacterianos , Estado Terminal , Monitoramento de Medicamentos , Oxigenação por Membrana Extracorpórea , Vancomicina , Humanos , Vancomicina/administração & dosagem , Vancomicina/farmacocinética , Vancomicina/efeitos adversos , Vancomicina/sangue , Oxigenação por Membrana Extracorpórea/efeitos adversos , Estudos Retrospectivos , Antibacterianos/administração & dosagem , Antibacterianos/farmacocinética , Antibacterianos/efeitos adversos , Masculino , Feminino , Pré-Escolar , Lactente , Resultado do Tratamento , Criança , Fatores de Tempo , Cálculos da Dosagem de Medicamento , Rim/fisiopatologia , Rim/efeitos dos fármacos , Fatores Etários , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Recém-Nascido , Insuficiência Renal/terapia , Insuficiência Renal/diagnóstico , Insuficiência Renal/complicações , Insuficiência Renal/fisiopatologia , Fatores de Risco , Redução da Medicação , Adolescente , Unidades de Terapia Intensiva PediátricaRESUMO
BACKGROUND: Hypothermia is a neuroprotective strategy during cardiopulmonary bypass. Rewarming entailing a rapid rise in cerebral metabolism might lead to secondary neurological sequelae. In this pilot study, we aimed to validate the hypothesis that a slower rewarming rate would lower the risk of cerebral hypoxia and seizures in infants. METHODS: This is a prospective, clinical, single-center study. Infants undergoing cardiac surgery in hypothermia were rewarmed either according to the standard (+1°C in < 5 minutes) or a slow (+1°C in > 5-8 minutes) rewarming strategy. We monitored electrocortical activity via amplitude-integrated electroencephalography (aEEG) and cerebral oxygenation by near-infrared spectroscopy during and after surgery. RESULTS: Fifteen children in the standard rewarming group (age: 13 days [5-251]) were cooled down to 26.6°C (17.2-29.8) and compared with 17 children in the slow-rewarming group (age: 9 days [4-365]) with a minimal temperature of 25.7°C (20.1-31.4). All neonates in both groups (n = 19) exhibited suppressed patterns compared with 28% of the infants > 28 days (p < 0.05). During rewarming, only 26% of the children in the slow-rewarming group revealed suppressed aEEG traces (vs. 41%; p = 0.28). Cerebral oxygenation increased by a median of 3.5% in the slow-rewarming group versus 1.5% in the standard group (p = 0.9). Our slow-rewarming group revealed no aEEG evidence of any postoperative seizures (0 vs. 20%). CONCLUSION: These results might indicate that a slower rewarming rate after hypothermia causes less suppression of electrocortical activity and higher cerebral oxygenation during rewarming, which may imply a reduced risk of postoperative seizures.
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Ponte Cardiopulmonar , Eletroencefalografia , Hipotermia Induzida , Reaquecimento , Convulsões , Espectroscopia de Luz Próxima ao Infravermelho , Humanos , Lactente , Estudos Prospectivos , Projetos Piloto , Masculino , Fatores de Tempo , Recém-Nascido , Feminino , Resultado do Tratamento , Hipotermia Induzida/efeitos adversos , Fatores de Risco , Convulsões/fisiopatologia , Convulsões/diagnóstico , Convulsões/etiologia , Convulsões/prevenção & controle , Ponte Cardiopulmonar/efeitos adversos , Ondas Encefálicas , Hipóxia Encefálica/prevenção & controle , Hipóxia Encefálica/etiologia , Hipóxia Encefálica/fisiopatologia , Hipóxia Encefálica/diagnóstico , Fatores Etários , Monitorização Neurofisiológica Intraoperatória , Encéfalo/metabolismo , Encéfalo/fisiopatologia , Encéfalo/irrigação sanguínea , Circulação CerebrovascularRESUMO
BACKGROUND: Immunosuppression after heart transplantation (HTX) with mammalian target of rapamycin (mTOR) inhibitors serves as a prophylaxis against rejection and to treat coronary vascular injury. However, there is little data on the early, preventive use of everolimus after pediatric HTX. METHODS: Retrospective study of 61 pediatric HTX patients (48 cardiomyopathy and 13 congenital heart disease), 28 females, median age 10.1 (range 0.1-17.9) years transplanted between 2008 and 2020. We analyzed survival, rejection, renal function, occurrence of lymphoproliferative disorder, and allograft vasculopathy together with adverse effects of early everolimus therapy combined with low-dose calcineurin inhibitors. RESULTS: Everolimus therapy was started at a median 3.9 (1-14) days after HTX. Median follow-up was 4.3 (range 0.5-11.8) years, cumulative 184 patient years. The estimated 1- and 5-year survival probability was 89% (CI 82%:98%) and 87% (CI 78%:97%). Four patients developed rejection (6.6%) (maximum 2R ISHLT criteria). No patient suffered from chronic renal failure. Three patients (4.9%) developed post-transplant lymphoproliferative disorder. Five patients suffered relevant wound-healing disorders after transplantation, four of them carrying relevant risk factors before HTX (mechanical circulatory support (n = 3), delayed chest closure after HTX (n = 3)). No recipient developed cardiac allograft vasculopathy. CONCLUSION: Initiating everolimus within the first 14 days after HTX seems to be well tolerated, enabling a low incidence of rejection, post-transplant lymphoproliferative disorders, renal failure, and reveals no evidence of cardiac allograft vasculopathy as well as good overall survival in pediatric heart transplant recipients.
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Traumatismos Cardíacos , Transplante de Coração , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Aloenxertos , Everolimo/uso terapêutico , Coração , Estudos Retrospectivos , MasculinoRESUMO
Background: The charity organization Kinderherzen retten e.V. (KHR) enables humanitarian congenital heart surgery for pediatric patients from low- and middle-income countries at the University Heart Center Freiburg, Germany. The aim of this study was to assess periprocedural and mid-term outcomes of these patients for evaluation of KHR sustainability. Methods: Part one of the study comprised retrospective medical chart analyses of the periprocedural course of all KHR-treated children from 2008 to 2017, and part two a prospective evaluation of their mid-term outcome, assessed by questionnaires concerning survival, medical history, mental and physical development, and socioeconomic situation. Results: Of the 100 consecutively presented children from 20 countries (median age 3.25 years), 3 patients were not invasively treatable, 89 underwent cardiovascular surgery, and 8 received a catheter intervention only. There were no periprocedural deaths. Median postoperative duration of mechanical ventilation, intensive care stay, and total hospital stay was 7 (interquartile range [IQR] 4-21) hours, 2 (IQR 1-3) days, and 12 (IQR 10-16) days, respectively. Mid-term postoperative follow-up demonstrated a 5-year survival probability of 94.4%. The majority of patients received continued medical care in their home country (86.2% of patients), were in good mental and physical condition (96.5% and 94.7% of patients, respectively), and able to engage in age-appropriate education/employment (98.3% of patients). Conclusions: Cardiac, neurodevelopmental, and socioeconomic outcomes of patients treated via KHR was satisfactory. Thorough pre-visit evaluation and close contact with local physicians are crucial when providing this high-quality, sustainable, and viable therapeutic option for these patients.
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Cardiopatias Congênitas , Criança , Humanos , Lactente , Pré-Escolar , Cardiopatias Congênitas/cirurgia , Estudos Retrospectivos , Alemanha , Tempo de InternaçãoRESUMO
A 9-month-old girl born with an interrupted aortic arch type B, an arteria lusoria (aberrant right subclavian artery) and a multilevel left ventricular outflow tract obstruction underwent a Yasui completion after an initial interim palliation. After the Damus-Kaye-Stansel procedure was carried out and the Sano shunt had been established as a source of pulmonary flow, the reported procedure comprised closure of the ventricular septal defect and the intraventricular baffling of left ventricular outflow through a malaligned ventricular septal defect, incision and partial resection of a conal septum and establishment of a right ventricle-to-pulmonary artery connection using an autologous left atrial appendage as a free graft. This technique consisted of dissecting and harvesting the left atrial appendage, which was then used as autologous material for an interposition plasty connecting the central pulmonary artery bifurcation segment with the upper rim of the infundibulotomy. Native, autologous tissue thus comprised the backwall of the newly created right ventricle-to-pulmonary artery continuity. Porcine pericardial patch plasty was then used to complete the remaining circumference of the right ventricle-to-pulmonary artery continuity.
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Apêndice Atrial , Comunicação Interventricular , Obstrução do Fluxo Ventricular Externo , Humanos , Animais , Suínos , Artéria Pulmonar/cirurgia , Ventrículos do Coração/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Comunicação Interventricular/cirurgiaRESUMO
BACKGROUND: Pediatric heart transplant (HTx) recipients with congenital heart defects require complex concomitant surgical procedures with the risk of prolonging the allograft's ischemic time. Ex vivo allograft perfusion with the Organ Care System (OCS; Transmedics, Andover, MA) may improve survival of these challenging patients. METHODS: In this retrospective, single-center study a consecutive series of 8 children with allografts preserved using the OCS was compared with 13 children after HTx with cold storage of the donor heart from March 2018 to March 2020. RESULTS: Median recipient age in the control group was 18 months (range, 1-189) versus 155 months (range, 83-214) in the OCS group, and the baseline differences between the 2 groups were not significant. Fifty percent of the children in the OCS group had complex congenital heart defects (vs 15% of the control subjects). Median operation time during HTx in the OCS group was 616 minutes (range, 270-809) versus 329 minutes (range, 283-617). Because of the time of ex vivo allograft perfusion (265 minutes [range, 202-372]) median total ischemia time was significantly shorter in the OCS group: 78 minutes (range, 52-111) versus 222 minutes (range, 74-326). The incidence of primary graft, renal, or hepatic failure did not differ between the groups. Graft function and the occurrence of any treated rejection at follow-up revealed no significant difference between the 2 groups. One-year survival was 88% in the OCS group (vs 85%). CONCLUSIONS: Ex vivo allograft perfusion enabled complex pediatric HTx, yielding outcomes as positive as those of children whose donor hearts were stored in ice-cold solution.
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Transplante de Coração/métodos , Preservação de Órgãos/métodos , Perfusão/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fatores de TempoRESUMO
OBJECTIVES: Bleeding signs can become life-threatening complications in patients on mechanical circulatory support (MCS). Clinical phenotyping and comprehensive analyses of the cause of bleeding are, therefore, essential, especially when risk-stratifying patients during MCS workup. We conducted coagulation analyses and determined von Willebrand factor (VWF) parameters in a paediatric cohort on temporary extracorporeal life support, extracorporeal membrane oxygenation or long-term ventricular assist device support. METHODS: We carried out an observational single-centre study including 30 children with MCS (extracorporeal life support, n = 13; extracorporeal membrane oxygenation, n = 5; and ventricular assist device, n = 12). We also assessed the acquired von Willebrand parameters of each study participant: collagen binding capacity (VWF:CB), the ratio of collagen-binding capacity to VWF antigen (VWF:CB/VWF:Ag) and high-molecular-weight VWF multimers. We also documented bleeding events, transfusion requirement, haemolysis parameters and surgical interventions. RESULTS: All children developed AVWS (acquired von Willebrand syndrome) during MCS, usually during the early postoperative course. They presented no AVWS after device explantation. We detected a loss of high-molecular-weight VWF multimers, decreased VWF:CB/VWF:Ag ratios and reduced VWF:CB levels. Twenty of the 30 patients experienced bleeding complications; approximately 53% of them required surgical revision. There were no deaths due to bleeding during support. CONCLUSIONS: The AVWS prevalence in paediatric patients on MCS is 100% regardless of the types of devices tested in this study. The bleeding propensity of AVWS patients widely varies.
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Oxigenação por Membrana Extracorpórea/efeitos adversos , Insuficiência Cardíaca/terapia , Coração Auxiliar/efeitos adversos , Hemorragia/etiologia , Doenças de von Willebrand/complicações , Fator de von Willebrand/metabolismo , Adolescente , Biomarcadores/sangue , Criança , Pré-Escolar , Feminino , Seguimentos , Alemanha/epidemiologia , Hemorragia/epidemiologia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Adulto Jovem , Doenças de von Willebrand/sangue , Doenças de von Willebrand/epidemiologiaRESUMO
Extracorporeal life support (ECLS) weaning is a complex interdisciplinary process with no clear guidelines. To assess ventricular and pulmonary function as well as hemodynamics including end-organ recovery during ECLS weaning, we developed a standardized weaning protocol. We reviewed our experience 2 years later to assess its feasibility and efficacy. In 2015 we established an inter-professional, standardized, stepwise protocol for weaning from ECLS. If the patient did not require further surgery, weaning was conducted bedside in the intensive care unit (ICU). Most of the weaning procedures are guided via echocardiography. Data acquisition began at baseline level, followed by four-step course (each step lasting 10 min), entailing flow-reduction and ending 30 min after decannulation. Moreover, data from the preprotocol era are presented. Between May 2015 and 2017, 26 consecutive patients (18 male), median age 177 days (2 days-20 years) required ECLS with median support of 4 (2-11) days. Excluding eight not weanable patients, 21 standardized weaning procedures were protocolled in the remaining 18 children. Our generally successful protocol-guided weaning rate (with at least 24-h survival) was 89%, with a discharge home rate of 58%. Practical application of the novel standard protocol seems to facilitate ECLS weaning and to improve its success rate. The protocol can be administered as part of standard bedside ICU assessment.
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Oxigenação por Membrana Extracorpórea/normas , Cuidados para Prolongar a Vida/normas , Choque Cardiogênico/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Protocolos Clínicos , Ecocardiografia , Oxigenação por Membrana Extracorpórea/instrumentação , Oxigenação por Membrana Extracorpórea/métodos , Estudos de Viabilidade , Feminino , Humanos , Lactente , Recém-Nascido , Cuidados para Prolongar a Vida/instrumentação , Cuidados para Prolongar a Vida/métodos , Masculino , Guias de Prática Clínica como Assunto , Estudos Retrospectivos , Choque Cardiogênico/diagnóstico por imagem , Choque Cardiogênico/mortalidade , Taxa de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are major causes of long-term mortality after Fontan operation. The objective of this study was to determine early clinical risk factors before the onset of PLE and PB. In a cohort study, 106 Fontan patients between 2005 and 2013 were examined. A median of 5.3 (1.5-8.5) years later, follow-up questionnaires were used to group the patients in a PLE or PB group (n = 14) and a non-PLE/PB group (n = 92). Prevalence of PLE was 9.4% (n = 10) and of PB 3.8% (n = 4). At follow-up, five patients (4.7%) died of PLE or PB. Median age at death was 6.2 years (IQR 10.5, 95% CI 5.3-23.4). We observed no significant group differences in gender distribution (p = 0.73), ventricular morphology (p = 0.87), surgical technique (p = 0.64), conduit fenestration (p = 0.34), age at Fontan operation (p = 0.54), and need for diuretics (p = 0.56). Hypoplastic left heart syndrome was more frequent in the PLE/PB group 50 vs. 22.8% (p = 0.03) OR 3.4 (95% CI 1.1-10.8). The modified Glenn procedure was performed at a median age of 4 months (IQR 4.0) in the PLE/PB group versus 8 months (IQR 8.0) in the non-PLE/PB group (p = 0.01). The early Glenn procedure and hypoplastic left heart syndrome may be associated with the development of PLE and PB.
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Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Enteropatias Perdedoras de Proteínas/etiologia , Bronquite/etiologia , Criança , Pré-Escolar , Feminino , Técnica de Fontan/reabilitação , Cardiopatias Congênitas/reabilitação , Humanos , Síndrome do Coração Esquerdo Hipoplásico/reabilitação , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Lactente , Masculino , Estudos Retrospectivos , Fatores de Risco , Inquéritos e Questionários , Fatores de Tempo , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Procedimentos Cirúrgicos Vasculares/reabilitaçãoRESUMO
OBJECTIVES: The No-React® Injectable BioPulmonic™ valve (BioIntegral) was introduced for minimally invasive off-pump replacement of the pulmonary valve almost 10 years ago. We present our mid- to long-term follow-up results. METHODS: We conducted a retrospective analysis of all 7 patients treated at our institution at the median age of 9 (range 1-24) years. The children underwent cardiac catheterization when worsening strain on the right heart was suspected after examining their medical history and/or observing significant changes on echocardiography. RESULTS: After a median follow-up period lasting 5.2 (range 0.7-6.7) years, all patients presented the indication for recatheterization, particularly because the maximum instantaneous velocity measured by Doppler had revealed systolic gradients of a median 63 (dP 18-74) mmHg across the right ventricular outflow. Catheterization confirmed severe stenosis in 2, and moderate stenosis together with moderate insufficiency in 4 patients. We observed two principal failure mechanisms: technical problems resulting from poor alignment to the right ventricular outflow tract and structural problems leading to neointimal proliferation even in cases with appropriate prosthesis positioning. At median of 5.7 (0.7-7) years after implantation, 6 of the 7 patients underwent valve rereplacement. Redo surgery was necessary in 3, and percutaneous valve-in-valve implantation in the remaining 3 patients. Histological analysis of two explanted valves confirmed significant neointima proliferation and thickened valve cusps leading to stenosis of the graft. CONCLUSIONS: These mid-term results after implantation of the No-React® Injectable BioPulmonic™ valve are disappointing. Graft failure was mainly due to neointimal formation and valve malposition.
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Implante de Prótese de Valva Cardíaca/métodos , Próteses Valvulares Cardíacas/efeitos adversos , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Ecocardiografia , Feminino , Seguimentos , Humanos , Lactente , Masculino , Desenho de Prótese , Falha de Prótese , Insuficiência da Valva Pulmonar/diagnóstico , Estenose da Valva Pulmonar/diagnóstico , Estudos Retrospectivos , Fatores de Tempo , Adulto JovemRESUMO
Propofol is a short-acting, intravenously administered hypnotic agent which is used in procedural sedation in children. Propofol is known to decrease systemic vascular resistance, arterial blood pressure and can lead to desaturations and decreased systemic perfusion in children with cardiac shunting. This may result in a reduction in cerebral blood flow and oxygenation. Near-infrared spectroscopy (NIRS) can monitor cerebral tissue oxygenation in the frontal neocortex. The objective of our study was to measure the changes in cerebral oxygen and blood supply after Propofol infusion in children with congenital heart disease. Propofol infusion may reduce cerebral oxygenation in children with congenital heart disease. The study group consisted of 32 children (f:m = 18:14), with median age of 49 (5-112) months and median weight of 15 (5-34) kg. We performed NIRS derived continuous measurement of cerebral oxygenation and cardiac output using Electrical velocimetry for 5 min before and after sedation with Propofol (1-2 mg/kg i.v.) for cardiac catheterization. Simultaneously, non-invasive arterial blood pressure and transcutaneous oxygen saturation were measured. Propofol sedation led to a significant decrease in mean arterial pressure (79 ± 16 vs. 67 ± 12 mmHg) (p = 0.01) and cardiac index (3.2 ± 0.8 vs. 2.9 ± 0.6 ml/min/m(2)) (p = 0.03). In contrast, cerebral tissue oxygenation index, increased significantly from 57 ± 11 to 59 ± 10 % (p < 0.05). Sedation with Propofol increased cerebral tissue oxygenation despite a decrease in cardiac index and arterial blood pressure. This may be caused by a decreased oxygen consumption of the sedated brain with intact cerebral auto-regulation.
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Encéfalo/metabolismo , Cardiopatias Congênitas/cirurgia , Oxigênio/metabolismo , Propofol/farmacologia , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Anestesia/métodos , Pressão Sanguínea/efeitos dos fármacos , Encéfalo/efeitos dos fármacos , Débito Cardíaco/efeitos dos fármacos , Criança , Pré-Escolar , Feminino , Humanos , Hipnóticos e Sedativos/farmacologia , Lactente , Masculino , Monitorização Fisiológica/métodos , Oximetria/métodos , Propofol/administração & dosagem , Reologia/métodosRESUMO
Mutations in genes encoding for desmosomal components are associated with a broad spectrum of phenotypes comprising skin and hair abnormalities and account for 45-50% of cases of arrhythmogenic right ventricular cardiomyopathy. Today, more than 120 dominant and recessive desmoplakin (DSP) gene mutations have been reported to be associated with skin, hair and/or heart defects. Here we report on 3 cases with yet unreported DSP mutations, c.7566_7567delAAinsC, p.R2522Sfs*39, c.7756C>T, p.R2586*, c.2131_2132delAG and c.1067C>A, p.T356K, that were associated with variable woolly hair or hypotrichosis, palmoplantar keratoderma, and cardiac manifestations. In addition, we review and summarise the clinical features and DSP mutations of the patients described in the literature, which illustrates the complexity of this group of disorders and of their genotype-phenotype correlations, which cannot be easily predicted. Early diagnosis is crucial and cardiac examinations have to be performed on a regular basis.
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Cardiomiopatias/genética , Desmoplaquinas/genética , Doenças do Cabelo/congênito , Ceratodermia Palmar e Plantar/genética , Mutação , Adolescente , Cardiomiopatias/diagnóstico , Criança , Pré-Escolar , Análise Mutacional de DNA , Feminino , Predisposição Genética para Doença , Doenças do Cabelo/diagnóstico , Doenças do Cabelo/genética , Hereditariedade , Humanos , Ceratodermia Palmar e Plantar/diagnóstico , Masculino , Linhagem , Fenótipo , Fatores de RiscoRESUMO
OBJECTIVES: Mechanical circulatory support (MCS) is a rescue therapy for infants and children suffering from severe cardiorespiratory failure with specific system-related complications like bleeding, thromboembolism and device failure. Novel circuit components for temporary MCS with improved haemodynamic properties may improve patients' outcome and reduce system-related morbidities. The Deltastream DP3 (Medos Medizintechnik AG, Stolberg, Germany) is a newly designed rotational pump with a diagonally streamed impeller that can be used in children of all ages (priming volume 16 ml, flow 0-8 l/min). The aim of this study was to analyse the feasibility and safety of the DP3 pump system in children. METHODS: We retrospectively investigated a consecutive series of 16 children [median age 0.9 months (0.1-55 months), median weight 3.2 kg (2.5-14 kg)]. The DP3 circuit was used 22 times in these children for different indications: (I) extracorporeal life support (ECLS) in post-cardiotomy heart failure (n = 11), (II) ECLS in cardiopulmonary resuscitation (CPR) (n = 7) and (III) extracorporeal membrane oxygenation (ECMO) in acute respiratory distress syndrome (ARDS) (n = 4). RESULTS: Median duration of MCS was 4 days (0-18 days), 12 patients (75%) were weaned successfully from MCS, 4 of these children (25%) died after weaning, with a median survival time of 15 days (6-28 days). Overall survival rate was 50% and all 8 survivors were discharged home without neurological injury. There was no case of severe bleeding, thromboembolic complications or device failure. Mean lactate dehydrogenase (LDH) before MCS was 700 (±384) U/l, and increased to a maximum of 2279 (±2635) U/l during MCS (P = 0.04). Baseline D-dimer values were 3.4 (±3.0) mg/l and rose significantly to 19.5 (±11.5) mg/l during MCS (P < 0.001). The mean of the highest plasma-free haemoglobin during MCS was 21.0 (±42.9) mg/dl. The increase in plasma-free haemoglobin correlated moderately with the duration of MCS (Pearson's r: 0.78). CONCLUSION: The use of the Deltastream DP3 seems to be safe and effective for MCS in children and may show a low degree of haemolysis. We observed no system-related complications and an overall good outcome in this demanding patient cohort.
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Circulação Extracorpórea/métodos , Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/cirurgia , Cateterismo Cardíaco , Pré-Escolar , Circulação Extracorpórea/efeitos adversos , Circulação Extracorpórea/instrumentação , Feminino , Cardiopatias Congênitas/fisiopatologia , Insuficiência Cardíaca/fisiopatologia , Hemodinâmica , Hemólise , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
BACKGROUND: The Cardio QP™ oesophageal Doppler monitor measures the velocity time integral of the blood flow in the descending aorta. Based on system integrated normograms of the aortic cross-sectional area of a paediatric population, the cardiac output is calculated and displayed. OBJECTIVE: Evaluation of the capability of the Cardio QP™ to detect changes in cardiac output during desynchronizing ventricular pacing (VVI) in children after cardiac surgery. PATIENTS: Eleven children (6 female, 5 male) with epicardial pacemaker electrodes admitted to the paediatric intensive care unit (PICU) after corrective surgery for congenital heart defects. Mean age: 6.3 (2.1-15.0) months, mean body weight: 5.3 (3.5-7.8) kg. INTERVENTIONS: After baseline measurements of cardiac output (base I), we performed 3 steps, each lasting 5 min: (1) ventricular pacing (VVI), (2) baseline (base II) recording, (3) atrial pacing (AOO). We measured the effects on haemodynamic parameters and blood gases as well as on the measured cardiac output. RESULTS: Ventricular pacing, with atrio-ventricular dyssynchrony, led to a significant drop in blood pressure and central venous saturation. Cardiac output parameters showed a decrease in stroke volume (SV) from 4.9±2.2 to 4.2±2.1 ml (P = 0.005) and cardiac index (CI) (2.6±1.1-2.1±0.8 ml/min/m(2)) (P = 0.009) during ventricular pacing. Cardiac index and haemodynamic parameters during atrial stimulation did not show significant changes from baseline. CONCLUSION: The Cardio QP™ seems to be capable of detecting slight changes in cardiac output.
Assuntos
Débito Cardíaco , Procedimentos Cirúrgicos Cardíacos , Monitorização Intraoperatória/instrumentação , Pediatria/instrumentação , Reologia/instrumentação , Ultrassonografia Doppler/instrumentação , Desenho de Equipamento , Análise de Falha de Equipamento , Feminino , Humanos , Lactente , Masculino , Cuidados Pós-Operatórios/instrumentação , Reprodutibilidade dos Testes , Sensibilidade e EspecificidadeRESUMO
BACKGROUND: The brain of children in the early period after repair of congenital heart defects with cardiopulmonary bypass (CPB) may be more vulnerable to hemodynamic changes because of impaired cerebral autoregulation. During postoperative testing of the external temporary safety pacer, we performed desynchronizing ventricular pacing (VVI) while monitoring cerebral oxygenation using near-infrared spectroscopy (NIRS). METHODS: We prospectively investigated 11 children (6 girls, 5 boys). Mean age was 6.1 months (+/-3.8 months) and mean weight: 5.3 kg (+/-1.5 kg). We performed measurements at four study steps: baseline I, VVI pacing, baseline II and atrial pacing (AOO) to exclude effects of higher heart rate. We continuously measured the effects on hemodynamic and respiratory parameters as well as on cerebral tissue oxygenation index (TOI). Hemoglobin difference (HbD) was calculated as a parameter for cerebral blood flow (CBF). RESULTS: Ventricular pacing leads to a significant decrease in arterial blood pressure and central venous saturation accompanied by an immediate and significant decrease in TOI (63.3% +/- 7.6% to 61.5% +/- 8.4% [P < 0.05]) and HbD (0.51 +/- 1.8 micromol.l(-1) to -2.9 +/- 4.7 micromol.l(-1) [P < 0.05]). CONCLUSION: Cardiac desynchronization after CPB seems to reduce CBF and cerebral oxygenation in children.
Assuntos
Química Encefálica/fisiologia , Estimulação Cardíaca Artificial , Procedimentos Cirúrgicos Cardíacos , Consumo de Oxigênio/fisiologia , Anestesia Geral , Gasometria , Circulação Cerebrovascular , Feminino , Hemodinâmica/efeitos dos fármacos , Hemoglobinas/metabolismo , Humanos , Lactente , Masculino , Oxigênio/sangue , Estudos Prospectivos , Espectroscopia de Luz Próxima ao InfravermelhoRESUMO
Sildenafil (Viagra) has been shown to be an effective pulmonary vasodilator and is increasingly used in patients with pulmonary hypertension. Its effects on the cerebral circulation are unclear and have not yet been described. We investigated the effect of i.v. sildenafil treatment on cerebral oxygenation in 13 children with elevated pulmonary vascular resistance due to congenital heart defects after cardiac surgery using near-infrared spectroscopy (NIRS). Median age was 4.5 mo, and median weight was 5.5 +/- 1.8 kg. Sildenafil was administered in three steps of 15 min each with cumulative doses of 0.025, 0.1, and 0.25 mg/kg. We examined the changes of oxygenated hemoglobin (HbO2), deoxygenated hemoglobin (HHb), total hemoglobin (tHb) concentration, cytochrome oxidase (CytOx) oxygenation, and cerebral tissue oxygenation index (TOI) in 13 children. A significant increase in cerebral HbO2 and tHb at the beginning of i.v. sildenafil administration with a decrease in HHb was observed. These changes led to a significant elevation in cerebral TOI from 63.4 +/- 2.5% to 65.7 +/- 2.8%, whereas mean systemic arterial pressure and arterial oxygen partial pressure tended to decrease. In conclusion, we observed a reversible increase of HbO2, tHb, and hemoglobin oxygen saturation in the scanned tissue section after i.v. sildenafil administration. These findings may be clinically relevant because they indicate that after cardiac surgery, sildenafil may increase cerebral blood flow (CBF), probably due to general endothelial dysfunction after cardiopulmonary bypass (CPB).
Assuntos
Encéfalo , Procedimentos Cirúrgicos Cardíacos , Circulação Cerebrovascular/efeitos dos fármacos , Oxigênio/metabolismo , Piperazinas/farmacologia , Espectroscopia de Luz Próxima ao Infravermelho , Vasodilatadores/farmacologia , Animais , Encéfalo/anatomia & histologia , Encéfalo/efeitos dos fármacos , Feminino , Hemoglobinas/metabolismo , Humanos , Lactente , Masculino , Consumo de Oxigênio , Piperazinas/administração & dosagem , Estudos Prospectivos , Purinas , Citrato de Sildenafila , SulfonasRESUMO
OBJECTIVE: To evaluate the relationship between the cerebral tissue oxygenation index measured by near-infrared spectroscopy and central venous oxygen saturation (SvO2) after corrective surgery of congenital heart defects in children. DESIGN: Prospective observational clinical study. SETTING: A tertiary neonatal and paediatric intensive care unit for paediatric cardiology. PATIENTS: Neonates and children consecutively admitted to the paediatric cardiology intensive care unit after corrective surgery of non-cyanotic congenital heart defects. MEASUREMENTS AND RESULTS: Forty-three children were studied. Cerebral tissue oxygenation index, measured non-invasively by near-infrared spectroscopy, was compared to SvO2, measured by a catheter placed in the right atrium, and to haemodynamic and respiratory parameters. Pearson's correlation coefficients and p values were calculated. Simultaneously measured values for SvO2 (62.2+/-9.8%, 39.8-80.4%) and cerebral tissue oxygenation index (56.7+/-8.8%, 35.8-71.2%) showed a significant correlation ( r=0.52, p<0.001). CONCLUSION: Cerebral tissue oxygenation index and SvO2 are not interchangeable parameters, but cerebral tissue oxygenation index reflects the haemodynamic influence on cerebral oxygenation after cardiovascular surgery. Further work is necessary to confirm the clinical role of continuous non-invasive measurement of cerebral tissue oxygenation index with regard to the variations of global systemic oxygen consumption after cardiac surgery in children.