Imaging of cerebral complications of extracorporeal membrane oxygenation in infants with congenital heart disease - ultrasound with multimodality correlation.

Cranial ultrasound on neonatal intensive care units is generally performed by intensive care physicians, but radiologists often provide this crucial bedside test to children on specialist paediatric cardiac intensive care units. On a paediatric cardiac intensive care unit, complex congenital cardiac conditions are commonly encountered in both pre- and postoperative scenarios, often with the use of extracorporeal membrane oxygenation (ECMO), which both increases the risks of a number of neurologic complications and results in significant changes in vascular physiology. The aim of this pictorial essay is to discuss cranial ultrasound technique, demonstrate the changes in Doppler flow profiles resulting from veno-arterial extracorporeal membrane oxygenation and congenital cardiac conditions, and illustrate commonly encountered intracranial complications of extracorporeal membrane oxygenation support in congenital cardiac care.

L-carnitine preserves endothelial function in a lamb model of increased pulmonary blood flow.

BACKGROUND: In our model of a congenital heart defect (CHD) with increased pulmonary blood flow (PBF; shunt), we have recently shown a disruption in carnitine homeostasis, associated with mitochondrial dysfunction and decreased endothelial nitric oxide synthase (eNOS)/heat shock protein (Hsp)90 interactions that contribute to eNOS uncoupling, increased superoxide levels, and decreased bioavailable nitric oxide (NO). Therefore, we undertook this study to test the hypothesis that L-carnitine therapy would maintain mitochondrial function and NO signaling. METHODS: Thirteen fetal lambs underwent in utero placement of an aortopulmonary graft. Immediately after delivery, lambs received daily treatment with oral L-carnitine or its vehicle. RESULTS: L-Carnitine-treated lambs had decreased levels of acylcarnitine and a reduced acylcarnitine:free carnitine ratio as compared with vehicle-treated shunt lambs. These changes correlated with increased carnitine acetyl transferase (CrAT) protein and enzyme activity and decreased levels of nitrated CrAT. The lactate:pyruvate ratio was also decreased in L-carnitine-treated lambs. Hsp70 protein levels were significantly decreased, and this correlated with increases in eNOS/Hsp90 interactions, NOS activity, and NOx levels, and a significant decrease in eNOS-derived superoxide. Furthermore, acetylcholine significantly decreased left pulmonary vascular resistance only in L-carnitine-treated lambs. CONCLUSION: L-Carnitine therapy may improve the endothelial dysfunction noted in children with CHDs and has important clinical implications that warrant further investigation.

Altered lymphatics in an ovine model of congenital heart disease with increased pulmonary blood flow.

Abnormalities of the lymphatic circulation are well recognized in patients with congenital heart defects. However, it is not known how the associated abnormal blood flow patterns, such as increased pulmonary blood flow (PBF), might affect pulmonary lymphatic function and structure. Using well-established ovine models of acute and chronic increases in PBF, we cannulated the efferent lymphatic duct of the caudal mediastinal node and collected and analyzed lymph effluent from the lungs of lambs with acutely increased PBF (n = 6), chronically increased PBF (n = 6), and age-matched normal lambs (n = 8). When normalized to PBF, we found that lymph flow was unchanged following acute increases in PBF but decreased following chronic increases in PBF. The lymph:plasma protein ratio decreased with both acute and chronic increases in PBF. Lymph bioavailable nitric oxide increased following acute increases in PBF but decreased following chronic increases in PBF. In addition, we found perturbations in the transit kinetics of contrast material through the pleural lymphatics of lambs with chronic increases in PBF. Finally, there were structural changes in the pulmonary lymphatic system in lambs with chronic increases in PBF: lymphatics from these lambs were larger and more dilated, and there were alterations in the expression of vascular endothelial growth factor-C, lymphatic vessel endothelial hyaluronan receptor-1, and Angiopoietin-2, proteins known to be important for lymphatic growth, development, and remodeling. Taken together these data suggest that chronic increases in PBF lead to both functional and structural aberrations of lung lymphatics. These findings have important therapeutic implications that warrant further study.

Failure of the Milwaukee protocol in a child with rabies.

Rabies has the highest case-fatality rate of all infectious diseases, with 50,000 cases occurring annually worldwide. In 2004 an unvaccinated adolescent survived after novel therapy. We report the management of a child with rabies. Although the implementation of this same therapeutic protocol was successful, the child died after 1 month of hospitalization.

Role of real-time reverse transcription polymerase chain reaction for detection of respiratory viruses in critically ill children with respiratory disease: Is it time for a change in algorithm?

OBJECTIVES: To identify the respiratory viral pathogens associated with acute lower respiratory tract infection in critically ill pediatric patients by using real-time reverse transcription-polymerase chain reaction, and compare results with those of direct fluorescence antibody assay testing. DESIGN: Observational cohort study. SETTING: Pediatric intensive care unit at a tertiary care academic hospital. PATIENTS: Pediatric patients admitted to the pediatric intensive care unit with severe respiratory symptoms consistent with viral lower respiratory tract infection. INTERVENTIONS: None. MEASUREMENTS: Respiratory samples of pediatric patients admitted to the pediatric intensive care unit with severe respiratory symptoms between January 2008 and July 2009 were tested with direct fluorescence antibody assay and real-time reverse transcription-polymerase chain reaction. MAIN RESULTS: At least one viral agent was detected in 70.5% of specimens by real-time reverse transcription-polymerase chain reaction and in 16.5% by direct fluorescence antibody assay (p < .001). Real-time reverse transcription-polymerase chain reaction increased the total viral yield five-fold compared to direct fluorescence antibody assay. Rhinovirus was the most commonly identified virus (41.6%). For viruses included in the direct fluorescence antibody assay panel, direct fluorescence antibody assay had a sensitivity of 0.42 (95% confidence interval 0.25-0.61) and a specificity of 1 (95% confidence interval 0.86-1.00) compared with real-time reverse transcription-polymerase chain reaction. Coinfections were not uncommon, in particular with rhinovirus, and these patients tended to have higher mortality. CONCLUSIONS: Direct fluorescence antibody assay testing is a suboptimal method for the detection of respiratory viruses in critically ill children with lower respiratory tract infection. Given the importance of a prompt and accurate viral diagnosis for this group of patients, we suggest that real-time reverse transcription-polymerase chain reaction becomes part of the routine diagnostic algorithm in critically ill children when a viral etiology is suspected, even if conventional tests yield a negative result.

Caring for critically ill adults in paediatric intensive care units in England during the COVID-19 pandemic: planning, implementation and lessons for the future.

OBJECTIVE: To describe the experience of paediatric intensive care units (PICUs) in England that repurposed their units, equipment and staff to care for critically ill adults during the first wave of the COVID-19 pandemic. DESIGN: Descriptive study. SETTING: Seven PICUs in England. MAIN OUTCOME MEASURES: (1) Modelling using historical Paediatric Intensive Care Audit Network data; (2) space, staff, equipment, clinical care, communication and governance considerations during repurposing of PICUs; (3) characteristics, interventions and outcomes of adults cared for in repurposed PICUs. RESULTS: Seven English PICUs, accounting for 137 beds, repurposed their space, staff and equipment to admit critically ill adults. Neighbouring PICUs increased their bed capacity to maintain overall bed numbers for children, which was informed by historical data modelling (median 280-307 PICU beds were required in England from March to June). A total of 145 adult patients (median age 50-62 years) were cared for in repurposed PICUs (1553 bed-days). The vast majority of patients had COVID-19 (109/145, 75%); the majority required invasive ventilation (91/109, 85%). Nearly, a third of patients (42/145, 29%) underwent a tracheostomy. Renal replacement therapy was provided in 20/145 (14%) patients. Twenty adults died in PICU (14%). CONCLUSION: In a rapid and unprecedented effort during the first wave of the COVID-19 pandemic, seven PICUs in England were repurposed to care for adult patients. The success of this effort was underpinned by extensive local preparation, close collaboration with adult intensivists and careful national planning to safeguard paediatric critical care capacity.

Stage I palliation for hypoplastic left heart syndrome: Is a right ventricle to pulmonary artery conduit associated with improved outcomes? Critical appraisal of Cua et al: Early postoperative outcomes in a series of infants with hypoplastic left heart syndrome undergoing stage I palliation operation with either modified Blalock-Taussig shunt or right ventricle to pulmonary artery conduit (Pediatr Crit Care Med 2006; 7:238-244).

OBJECTIVE: To review the findings and discuss the implications of studies comparing Norwood procedure with either modified Blalock-Taussig shunt (NW-BT) or right ventricle to pulmonary artery conduit (NW-RVPA) in stage I palliation of hypoplastic left heart syndrome (HLHS). DESIGN: A critical appraisal of "Early postoperative outcomes in a series of infants with hypoplastic left heart syndrome undergoing stage I palliation operation with either modified Blalock-Taussig shunt or right ventricle to pulmonary artery conduit" by Cua et al. (Pediatr Crit Care Med 2006; 7:238-244) with literature review. FINDINGS: Several retrospective studies using historical controls have reported improved results with NW-RVPA compared with NW-BT for stage I palliation of HLHS. The study by Cua et al. is one of a few recent articles with concurrent series that have found no difference in early postoperative mortality or morbidity, although in comparison with the NW-BT group, the NW-RVPA group had significantly shorter duration of mechanical ventilation, decreased length of intensive care unit stay and hospital stay, and shorter time to sternal closure and to establishment of enteral feeds. CONCLUSIONS: NW-RVPA has theoretical advantages over NW-BT and is associated with excellent outcome results. However, available data are insufficient to prove benefit in terms of mortality or morbidity. A multicenter randomized trial is currently being conducted, which should provide more conclusive information.

A reverse evidence of rotavirus vaccines impact.

In 2010, and due to a quality problem identified in the vaccine manufacture, the rotavirus (RV) vaccination was withheld in Spain during 5 months. Our study aimed to evaluate the impact that this sudden cease had on rotavirus acute gastroenteritis (RAGE) hospitalizations. An increase in RAGE hospitalization was observed in parallel to the drop in vaccine coverage. Here, we report the first reverse evidence of rotavirus vaccine impact.

Preserving mitochondrial function prevents the proteasomal degradation of GTP cyclohydrolase I.

The development of pulmonary hypertension is a common accompaniment of congenital heart disease (CHD) with increased pulmonary blood flow. Our recent evidence suggests that asymmetric dimethylarginine (ADMA)-induced mitochondrial dysfunction causes endothelial nitric oxide synthase (eNOS) uncoupling secondary to a proteasome-dependent degradation of GTP cyclohydrolase I (GCH1) that results in a decrease in the NOS cofactor tetrahydrobiopterin (BH(4)). Decreases in NO signaling are thought to be an early hallmark of endothelial dysfunction. As l-carnitine plays an important role in maintaining mitochondrial function, in this study we examined the protective mechanisms and the therapeutic potential of l-carnitine on NO signaling in pulmonary arterial endothelial cells and in a lamb model of CHD and increased pulmonary blood flow (Shunt). Acetyl-l-carnitine attenuated the ADMA-mediated proteasomal degradation of GCH1. This preservation was associated with a decrease in the association of GCH1 with Hsp70 and the C-terminus of Hsp70-interacting protein (CHIP) and a decrease in its ubiquitination. This in turn prevented the decrease in BH(4) levels induced by ADMA and preserved NO signaling. Treatment of Shunt lambs with l-carnitine also reduced GCH1/CHIP interactions, attenuated the ubiquitination and degradation of GCH1, and increased BH(4) levels compared to vehicle-treated Shunt lambs. The increases in BH(4) were associated with decreased NOS uncoupling and enhanced NO generation. Thus, we conclude that L-carnitine may have a therapeutic potential in the treatment of pulmonary hypertension in children with CHD with increased pulmonary blood flow.

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Afectació pericàrdica i infecció per Mycoplasma pneumoniae. Podem establir una relació causal? / Afectación pericárdica e infección por mycoplasma pneumoniae. ¿Podemos establecer una relación causal? / Pericarditis and mycoplasma pneumoniae infection, can we stablish a causal (causative) relationship?

Introducció. L’afectació pericàrdica per M. pneumoniae (MP) és una entitat poc freqüent en pediatria, però amb conseqüències que poden ser greus si no s’instaura un tractament adequat i precoç. Arran de dos casos viscuts al nostre centre es revisa aquesta entitat i se n’emfatitza el diagnòstic. Casos clínics. Cas clínic 1: nena de 7 anys d’edat amb clínica prèvia de vies altes, que presenta dispnea per la qual cosa se li fa una radiografia de tòrax, que mostra cardiomegàlia, i una ecocardiografia compatible amb taponament cardíac que requereix pericardiocentesi evacuadora. Va rebre tractament amb ibuprofè i azitromicina orals. Les proves serològiques fetes van ser compatibles amb infecció recent per M. pneumoniae. Cas clínic 2: noi de 15 anys intervingut als dos mesos de vida de coartació d’aorta, que presenta clínica compatible amb pericarditis i radiografia que mostra cardiomegàlia i vessament pleural esquerre. Se li fa un ecocardiograma compatible amb vessament pericàrdic per la qual cosa rep tractament amb aspirina via oral i azitromicina, per serologies MP compatibles amb infecció recent per aquest germen. L’evolució posterior va ser favorable en tots dos casos. Comentari. S’ha de considerar la possibilitat d’infecció per MP i el seu tractament empíric en casos d’afectació cardíaca aguda, sobretot si va acompanyada o precedida de símptomes respiratoris, miàlgies, febre, anèmia normocí- tica o eosinofília, però tenint molt en compte una valoració diagnòstica acurada. Un diagnòstic precoç i un tractament instaurat a les primeres fases de la malaltia determinaran una evolució clínica favorable lliure de seqüeles (AU)

Introducción. La afectación pericárdica por M. pneumoniae (MP) es una entidad poco frecuente en pediatría pero con consecuencias que pueden ser graves si no se instaura un tratamiento adecuado y precoz. A partir de dos casos vividos en nuestro centro se revisa esta entidad enfatizando en su diagnóstico. Casos clínicos. Caso clínico 1: niña de 7 años de edad con clínica previa de vías altas, que presenta disnea motivo por el que se realiza radiografía de tórax que muestra cardiomegalia y ecocardiografía compatible con taponamiento cardíaco que requiere pericardiocentesis evacuadora. Recibió tratamiento con ibuprofeno y azitromicina orales. Las pruebas serológicas realizadas fueron compatibles con infección reciente por M. pneumoniae. Caso clínico 2: chico de 15 años intervenido a los dos mesos de vida de coartación de aorta, que presenta clínica compatible com pericarditis y radiografia que muestra cardiomegalia y derrame pleural izquierdo. Se realiza ecocardiograma que muestra derrame pericárdico recibiendo tratamiento con aspirina vía oral y azitromicina por serologías a MP compatibles con infección reciente por este germen. La evolución posterior fue favorable en ambos casos. Comentario. Se debe considerar la posibilidad de infección por MP y su tratamiento empírico en caso de afectación cardíaca aguda, sobre todo si se acompaña o va precedida de síntomas respiratorios, mialgias, fiebre, anemia normocítica o eosinofilia, pero siendo cautelosos en su valoración diagnóstica. Un diagnóstico precoz y un tratamiento instaurado en las primeras fases de la enfermedad determinaran una evolución clínica favorable libre de secuelas (AU)

Introduction. Mycoplasma pneumoniae-associated pericarditis is rarely described in the pediatric population, although it can result in severe consequences if an early diagnosis is not performed and the appropriate terament administered. We describe two cases of pericardial effusion associated with M. pneumoniae infection and emphasize the key diagnostic elements. Case Reports. Case 1: A chest X-ray performed on a 7 yo girl with dyspnea and prior history of flu-like symptoms revealed cardiomegaly. An echocardiogram showed cardiac tamponade. A pericardiocentesis was performed, with good clinical response, and treatment with azithromycin and ibuprofen was started. Serological tests for Mycoplasma pneumoniae indicated acute infection. Case 2: A 15 yo boy diagnosed and treated for aortic coarctation at 2 months of age presented with symptoms of pericarditis. A chest X-ray revealed cardiomegaly and a left pleural effusion. An echocardiogram was performed showing pericardial effusion. Treatment with acetylsalicylic acid was started, and azithromycin was added after serological confirmation of Mycoplasma pneumoniae infection. The patient recovered uneventfully. Comment. The diagnosis of Mycoplasma pneumoniaeassociated pericarditis must be considered, and empirical treatment given, when a patient presents with acute pericarditis, particularly if fever, flu-like symptoms, normocytic anemia or eosinophilia, are present. However, we must be cautious when interpreting diagnostic tests. Early diagnosis and treatment will result in a good clinical outcome (AU)