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INTRODUCTION: Pediatric pulmonary hypertension is a rare condition. Survival remains poor in the current management era. There is a lack of data regarding the medical management of pediatric pulmonary hypertension and most pulmonary vasodilators are used off-label in children. AREAS COVERED: Pediatric pulmonary hypertension clinical trials' design and realization face many hurdles, including poor recruitment, limited available pharmacologic and physiologic data in children of various ages, ethical issues, and the lack of validated trial endpoint. Innovative clinical trial designs have emerged and may allow us to overcome some of these issues. Extrapolation of adult data to children, with additional pharmacokinetic and safety data, remains extremely important and valid in etiologies where the pediatric and the adult pathophysiologies are believed to be similar. EXPERT OPINION: Close collaboration between sponsors, regulators, patients, caregivers, physicians and researchers is necessary to develop efficacious and safe drugs for pediatric pulmonary hypertension. The increasing involvement of patients' and caregivers' participation in the development of clinical trials should help shape future research that is feasible and meaningful to the patients.
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PURPOSE: Children with congenital heart disease (CHD) from low- to middle-income countries (LMIC) are suspected to have a high prevalence of antibiotic-resistant microorganisms (ARMOs) carriage, but data are currently lacking. Carriage of ARMOs could impact the post-operative course in pediatric intensive care unit (PICU). The aim of the study was to assess the prevalence of ARMOs carriage in children with CHD from LMIC and its impact on post-operative outcomes. METHODS: This was a retrospective monocentric study from 01/2019 to 12/2022. Included patients were children (0-18 years) from a LMIC admitted after CHD surgery and with AMRO screening performed the week before. Infections and post-operative evolution were compared based on ARMOs carriage status. FINDINGS: Among 224 surgeries (median age 38.5 months (IQR 22-85.5)), ARMOs carriage was evidenced in 95 cases (42.4%). Main organisms isolated were Extended Spectrum Beta-Lactamase (ESBL) producing E. coli (75/224) 33.5%)) and ESBL-K. pneumoniae (30/224) 13.4%)). Median mechanical ventilation duration was 1 day (IQR 0-1), PICU stay 3 days (IQR 2-4) and hospital stay 6.5 days (IQR 5-10). A total of 17 infectious episodes occurred in 15 patients, mostly consisting in hospital-acquired pneumonia (HAP) (12/17). Only two infections were caused by a colonizing ARMO. Occurrence of infections and patients' outcome were similar between ARMO carriers and non-carriers. Higher use of carbapenems (6 (6.3%) vs 1 (0.8%), p = 0.04) and a trend to a higher use of vancomycin (14 (13.7%) vs 9 (6.9%), p = 0.04) in case of ARMOs carriage. Applying current guidelines, negative swab screening could have led to sparing most of empirical vancomycin therapy (11/12) for HAP based on current guidelines. CONCLUSION: Prevalence of AMROs carriage is high in children from LMIC and has a limited impact on patients' outcome. However, ARMOs carriage leads to higher consumption of antibiotics. Screening may help saving use of broad-spectrum antibiotic in non-carrier patients.
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There are minimal data describing use of extracorporeal membrane oxygenation (ECMO) in pediatric patient after a liver transplantation (LT). This study aimed at describing ECMO run in this specific situation using the data from Extracorporeal Life Support Organization (ELSO) Registry between January 1, 2010, to December 31, 2022. We described patients' characteristics at ECMO initiation, outcome and mortality risk factors. We identified 27 patients with a median age of 2.7 years (interquartile range (IQR) = 1.5-9.9). Main indication for ECMO support was respiratory (14/27 [52%]) followed by extracorporeal cardiopulmonary resuscitation (ECPR) (7/27 [26%]) and cardiac (6/27 [22%]). Overall in-hospital mortality was 63% (17/27). Mortality rate according to ECMO indications was 50% for both respiratory and cardiac failure and reached 100% for ECPR patients. Overall, nonsurvivors experienced significantly more complications under ECMO support ( p = 0.007). Main on-ECMO complications were hemorrhagic (11/27 [41%]) and thrombotic (7/27 [26%]). No clinical or biologic factors was predictive of patients' outcome. Our results suggest that ECMO support is a viable option for cardiac and respiratory indications after pediatric LT. Occurrence of complications while on ECMO are associated with unfavorable outcomes. The extremely high mortality rate in ECPR patients merits further research.
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Oxigenación por Membrana Extracorpórea , Trasplante de Hígado , Sistema de Registros , Humanos , Oxigenación por Membrana Extracorpórea/métodos , Oxigenación por Membrana Extracorpórea/efectos adversos , Trasplante de Hígado/efectos adversos , Trasplante de Hígado/métodos , Sistema de Registros/estadística & datos numéricos , Preescolar , Niño , Masculino , Lactante , Femenino , Mortalidad Hospitalaria , Estudios RetrospectivosAsunto(s)
Procedimientos Quirúrgicos Cardíacos , Infección Hospitalaria , Países en Desarrollo , Humanos , Infección Hospitalaria/epidemiología , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Lactante , Preescolar , Masculino , Femenino , Adolescente , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiologíaRESUMEN
Inhaled nitric oxide (iNO) is a pulmonary vasodilator that plays an important clinical ICU role. The administration of iNO is usually performed through an endotracheal tube, but spontaneously breathing patients might also benefit from iNO administration. The use of the non-invasive administration of iNO through high-flow nasal cannula (HFNC) allows for NO delivery in spontaneously breathing patients who still need supplemental oxygen and positive airway pressure. A simple method to administer NO through HFNC is described here using standard commercially available NO administration and HFNC.
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Cánula , Óxido Nítrico , Humanos , Óxido Nítrico/uso terapéutico , Cánula/efectos adversos , Pulmón , RespiraciónRESUMEN
Infective endocarditis due to Kingella kingae is a rare but serious invasive infection that occurs mostly in children. Recent advances in nucleic acid amplification testing as well as in cardiac imaging have enabled more accurate diagnosis. A good understanding of the epidemiology and virulence factors remains crucial to guide the therapeutic approach. Here, we synthesize the current state of knowledge on epidemiological features, pathophysiological insights, complications, and therapy regarding Kingella kingae endocarditis in children and adults. Finally, throughout this comprehensive review, knowledge gaps and areas for future research are also identified.
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OBJECTIVES: To determine factors associated with brain death in children treated with extracorporeal cardiopulmonary resuscitation (E-cardiopulmonary resuscitation). DESIGN: Retrospective database study. SETTINGS: Data reported to the Extracorporeal Life Support Organization (ELSO), 2017-2021. PATIENTS: Children supported with venoarterial extracorporeal membrane oxygenation (ECMO) for E-cardiopulmonary resuscitation. INTERVENTION: None. MEASUREMENTS AND MAIN RESULTS: Data from the ELSO Registry included patient characteristics, blood gas values, support therapies, and complications. The primary outcome was brain death (i.e., death by neurologic criteria [DNC]). There were 2,209 children (≥ 29 d to < 18 yr of age) included. The reason for ECMO discontinuation was DNC in 138 patients (6%), and other criteria for death occurred in 886 patients (40%). Recovery occurred in 1,109 patients (50%), and the remaining 76 patients (4%) underwent transplantation. Fine and Gray proportional subdistribution hazards' regression analyses were used to examine the association between variables of interest and DNC. Age greater than 1 year ( p < 0.001), arterial blood carbon dioxide tension (Pa co2 ) greater than 82 mm Hg ( p = 0.022), baseline lactate greater than 15 mmol/L ( p = 0.034), and lactate 24 hours after cannulation greater than 3.8 mmol/L ( p < 0.001) were independently associated with greater hazard of subsequent DNC. In contrast, the presence of cardiac disease was associated with a lower hazard of subsequent DNC (subdistribution hazard ratio 0.57 [95% CI, 0.39-0.83] p = 0.004). CONCLUSIONS: In children undergoing E-cardiopulmonary resuscitation, older age, pre-event hypercarbia, higher before and during ECMO lactate levels are associated with DNC. Given the association of DNC with hypercarbia following cardiac arrest, the role of Pa co2 management in E-cardiopulmonary resuscitation warrants further studies.
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Reanimación Cardiopulmonar , Oxigenación por Membrana Extracorpórea , Niño , Humanos , Oxigenación por Membrana Extracorpórea/efectos adversos , Estudios Retrospectivos , Muerte Encefálica , Dióxido de Carbono , Ácido Láctico , Sistema de RegistrosRESUMEN
Introduction: Pulmonary hypertension (PH) is a rare but fatal complication of sickle cell disease (SCD) that is possibly reversible if treated early. Dual-energy computed tomography (DECT) is a valuable tool for diagnosing PH. We attempted to determine if DECT can detect early signs of PH in children with SCD. Methods: This prospective observational pilot study was conducted at the Geneva University Hospitals and was approved by the local human ethics committee (CCER 2019-01975). A written informed consent was obtained from the patients and/or their legal guardian. Eight children (consisting of five girls and three boys) with homozygous SCD were included in the study. They underwent full cardiological workup using transthoracic echocardiography (TTE) and cardiopulmonary exercise test (CPET), as well as DECT. Results: The median age of the children was 11 years old (range 8-12). All patients exhibited a normal biventricular systo-diastolic function using the TTE. The median tricuspid regurgitant jet velocity value was 2.24â m/s (range 1.96-2.98). Four children were found to have signs of vasculopathy detected on DECT. Of them, two had abnormal screening test results. They both had an increased VE/VCO2 slope during CPET and an increased TVR of >2.5â m/s on TTE. Conclusion: DECT is capable of identifying early signs of pulmonary vascular disease in children with SCD. Further studies are needed to understand the correlation between DECT abnormalities and hemodynamic pulmonary circulation better.
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Congenital and acquired heart diseases can cause pulmonary hypertension (PH) in children, either by increasing pulmonary blood flow (PBF), left atrial pressure (LAp), and/or pulmonary vascular resistance (PVR). Pathophysiological process of pulmonary vascular disease (PVD) in different types of congenital heart diseases (CHDs) are reviewed hereafter. As with other types of PH, a rigorous diagnostic evaluation is mandatory to characterize the etiology of the PH, rule out other or additional causes of PH, and establish a risk profile. Cardiac catheterization remains the gold standard exam for PH diagnosis. Treatment of pulmonary arterial hypertension (PAH) associated with CHD (PAH-CHD) can then be started according to the recent guidelines recommendations, although most of the evidence is extrapolated from studies on other causes of PAH. PH in pediatric heart disease is often multifactorial, and sometimes unclassifiable, making the management of these patients complicated. The operability of patients with a prevalent left-to-right shunt and increase of PVR, the management of children with PH associated with left-sided heart disease, the challenges of pulmonary vascular disorders in children with univentricular heart physiology and the role of vasodilator therapy in failing Fontan patients are some of the hot topics discussed in this review.
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Shock is a life-threatening condition, and its timely recognition is essential for adequate management. Pediatric patients with congenital heart disease admitted to a cardiac intensive care unit (CICU) after surgical corrections are particularly at risk of low cardiac output syndrome (LCOS) and shock. Blood lactate levels and venous oxygen saturation (ScVO2) are usually used as shock biomarkers to monitor the efficacy of resuscitation efforts, but they are plagued by some limitations. Carbon dioxide (CO2)-derived parameters, namely veno-arterial CO2 difference (ΔCCO2) and the VCO2/VO2 ratio, may represent a potentially valuable addition as sensitive biomarkers to assess tissue perfusion and cellular oxygenation and may represent a valuable addition in shock monitoring. These variables have been mostly studied in the adult population, with a strong association between ΔCCO2 or VCO2/VO2 ratio and mortality. In children, particularly in CICU, few studies looked at these parameters, while they reported promising results on the use of CO2-derived indices for patients' management after cardiac surgeries. This review focuses on the physiological and pathophysiological determinants of ΔCCO2 and VCO2/VO2 ratio while summarizing the actual state of knowledge on the use of CO2-derived indices as hemodynamical markers in CICU.
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The population of patients with congenital heart disease is constantly growing with an increasing number of individuals reaching adulthood. A significant proportion of these children and young adults will suffer from tachyarrhythmias due to the abnormal anatomy, the hemodynamic burden, or as a sequela of surgical treatment. Depending on the underlying mechanism, arrhythmias may arise in the early postoperative period (hours to days after surgery) or in the late postoperative period (usually years after surgery). A good understanding of the electrophysiological characteristics and pathophysiological mechanisms is therefore crucial to guide the therapeutic approach. Here, we synthesize the current state of knowledge on epidemiological features, risk factors, pathophysiological insights, electrophysiological features, and therapy regarding tachyarrhythmias in children and young adults undergoing reparative surgery for congenital heart disease. The evolution and latest data on treatment options, including pharmacological therapy, ablation procedures, device therapy decision, and thromboprophylaxis, are summarized. Finally, throughout this comprehensive review, knowledge gaps and areas for future research are also identified.
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Ablación por Catéter , Cardiopatías Congénitas , Tromboembolia Venosa , Humanos , Niño , Adulto Joven , Anticoagulantes , Cardiopatías Congénitas/complicaciones , Taquicardia/cirugía , Arritmias Cardíacas/etiología , Ablación por Catéter/métodosRESUMEN
BACKGROUND: Infective endocarditis (IE) in pediatric patients is a severe cardiac disease and its actual epidemiology and clinical outcome in Switzerland is scarcely studied. METHODS: Retrospective nationwide multicenter data analysis of pediatric IE in children (<18 years) between 2011 and 2020. RESULTS: 69 patients were treated for definite (40/69;58%) or possible IE (29/69;42%). 61% (42/69) were male. Diagnosis was made at median 6.4 years (IQR 0.8-12.6) of age with 19 patients (28%) during the first year of life. 84% (58/69) had congenital heart defects. IE was located on pulmonary (25/69;35%), mitral (10/69;14%), tricuspid (8/69;12%) and aortic valve (6/69;9%), and rarely on ventricular septal defect (VSD;4/69;6%) and atrial septal defect (ASD;1/69;1%). In 22% (16/69) localization was unknown. 70% (48/69) had postoperative IE, with prosthetic material involved in 60% (29/48; right ventricular to pulmonary artery conduit (24), VSD (4), ASD (1)). Causative organisms were mostly Staphylococci spp. (25;36%) including Staphylococcus aureus (19;28%), and Streptococci spp. (13;19%). 51% (35/69) suffered from severe complications including congestive heart failure (16;23%), sepsis (17;25%) and embolism (19;28%). Staphylococcus aureus was found as a predictor of severe complications in univariate and multivariate analysis (p = 0.02 and p = 0.033). In 46% (32/69) cardiac surgery was performed. 7% (5/69) died. CONCLUSIONS: IE in childhood remains a severe cardiac disease with relevant mortality. The high morbidity and high rate of complications is associated with Staphylococcus aureus infections. Congenital heart defects act as a risk factor for IE, in particular the high number of cases associated with prosthetic pulmonary valve needs further evaluation and therapeutic alternatives.
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Endocarditis Bacteriana , Endocarditis , Cardiopatías Congénitas , Defectos del Tabique Interventricular , Infecciones Estafilocócicas , Adolescente , Niño , Humanos , Masculino , Femenino , Estudios Retrospectivos , Endocarditis Bacteriana/diagnóstico , Endocarditis Bacteriana/epidemiología , Endocarditis Bacteriana/cirugía , Endocarditis/diagnóstico , Endocarditis/epidemiología , Infecciones Estafilocócicas/diagnóstico , Infecciones Estafilocócicas/epidemiología , Staphylococcus aureus , Cardiopatías Congénitas/cirugía , Factores de Riesgo , Defectos del Tabique Interventricular/diagnóstico , Defectos del Tabique Interventricular/epidemiología , Defectos del Tabique Interventricular/cirugíaRESUMEN
Background: Eosinophilic myocarditis (EM) is a relatively rare form of myocarditis that could progress to restrictive cardiomyopathy and might be fatal if left untreated. Although myocardial biopsy is considered to be the gold standard for the diagnosis of myocarditis, its use in paediatrics remains controversial and not easily applicable in routine practice. Case summary: A 10-year-old girl with no prior medical history presented to the emergency department for fever, odynophagia, and gastrointestinal symptoms despite 48 h of antibiotics (Cefaclor). Physical examination revealed diffuse petechiae and abdominal tenderness but was otherwise unremarkable. Her vital signs were normal. She was found to have hypereosinophilia and increased cardiac markers on laboratory testing. Echocardiography showed diffuse left ventricular (LV) myocardial infiltrates, moderate LV dilatation, and mild systolic dysfunction. Bone marrow biopsy confirmed B cell acute lymphoblastic leukaemia. The diagnosis of EM was made. High doses of steroids and chemotherapy were initiated. Cardiac magnetic resonance imaging (MRI) identified eosinophilic infiltrates and sub-endocardial enhancement strongly suggestive of EM. Left ventricular function was slightly decreased. Intra-ventricular micro-thrombi were suspected, and warfarin was started. The outcome was favourable. Leucocyte and eosinophil counts were normalized within a month. At 6 months, cardiac MRI demonstrated a significant decrease in eosinophilic infiltration and micro-thrombi, normalization of LV function, and of sub-endocardial enhancement. Discussion: This case demonstrates that non-invasive multi-modality imaging along with typical laboratory and clinical findings allow for appropriate diagnosis of EM while avoiding biopsy. It also highlights that an early diagnosis, timely treatment, and rigorous follow-up improve disease progression and outcome.
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Kingella kingae is a gram-negative coccobacillus belonging to the HACEK group (Haemophilus species, Aggregatibacter actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens, and Kingella species) and is a common oropharyngeal colonizer of healthy young children. Osteoarticular infection is the most commonly reported invasive Kingella kingae infection in children, usually presenting a mild clinical picture. However, it can also cause severe invasive infections, especially infective endocarditis, with a high complication rate. We report the case of a 6-year-old girl, with no past medical history, who presented with fulminant infective endocarditis due to Kingella kingae. She received emergency venoarterial extracorporeal membrane oxygenation support, rapidly underwent cardiac surgery, and was then treated using ceftriaxone for 4 weeks as recommended by the American Heart Association. The patient's postoperative course was marked by a cerebral ischemic stroke consistent with septic embolism. She also presented with a para-aortic pseudoaneurysm that required a secondary surgical procedure, with a good postoperative result. This report illustrates a case of fulminant infective endocarditis due to Kingella kingae and responsible for two major complications. We also describe the preventive valve surgery performed to ensure the preservation of valve function and its capacity for growth.
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Portopulmonary hypertension is a rare but serious complication of portal hypertension or portosystemic shunting. Portopulmonary hypertension is an indication for liver transplantation or shunt closure. However, liver transplantation is contraindicated in patients with severe pulmonary arterial hypertension. Reported mortality rates are high in children with portopulmonary hypertension and there are scarce recommendations on its management. Our aim was to report on our real-world experience of managing portopulmonary hypertension in a specialised centre. We describe a series of 6 children with portopulmonary hypertension. Their median age at diagnosis was 13 years (range 10-15). The underlying liver conditions were cirrhosis of unknown origin (1), congenital portocaval shunts (3), biliary atresia (1), and portal vein cavernoma with surgical mesenterico-caval shunt (1). Median mean pulmonary arterial pressure was 47 mmHg (range 32-70), and median pulmonary vascular resistance was 6.6 Wood units (range 4.3-15.4). All patients except one were treated with a combination of pulmonary arterial hypertension-specific therapy (phosphodiesterase type 5 inhibitors and/or endothelin receptor antagonists and/or prostacyclin analogues). Three patients then benefited from shunt closure and the others underwent liver transplantation. Five patients showed improvement or stabilisation of pulmonary arterial hypertension with no deaths after a mean follow-up of 39 months. Based on our limited experience, early and aggressive treatment with a combination of pulmonary arterial hypertension-specific therapy significantly improves patients' haemodynamic profile and enables the performance of liver transplantation and shunt closure with satisfactory outcomes.