Perioperative diagnosis and impact of acquired von Willebrand syndrome in infants with congenital heart disease.

Acquired von Willebrand syndrome (aVWS) has been reported in patients with congenital heart diseases associated with shear stress caused by significant blood flow gradients. Its etiology and impact on intraoperative bleeding during pediatric cardiac surgery have not been systematically studied. This single-center, prospective, observational study investigated appropriate diagnostic tools of aVWS compared with multimer analysis as diagnostic criterion standard and aimed to clarify the role of aVWS in intraoperative hemorrhage. A total of 65 newborns and infants aged 0 to 12 months scheduled for cardiac surgery at our tertiary referral center from March 2018 to July 2019 were included in the analysis. The glycoprotein Ib M assay (GPIbM)/von Willebrand factor antigen (VWF:Ag) ratio provided the best predictability of aVWS (area under the receiver operating characteristic curve [AUC], 0.81 [95% CI, 0.75-0.86]), followed by VWF collagen binding assay/VWF:Ag ratio (AUC, 0.70 [0.63-0.77]) and peak systolic echocardiographic gradients (AUC, 0.69 [0.62-0.76]). A cutoff value of 0.83 was proposed for the GPIbM/VWF:Ag ratio. Intraoperative high-molecular-weight multimer ratios were inversely correlated with cardiopulmonary bypass (CPB) time (r = -0.57) and aortic cross-clamp time (r = -0.54). Patients with intraoperative aVWS received significantly more fresh frozen plasma (P = .016) and fibrinogen concentrate (P = .011) than those without. The amounts of other administered blood components and chest closure times did not differ significantly. CPB appears to trigger aVWS in pediatric cardiac surgery. The GPIbM/VWF:Ag ratio is a reliable test that can be included in routine intraoperative laboratory workup. Our data provide the basis for further studies in larger patient cohorts to achieve definitive clarification of the effects of aVWS and its potential treatment on intraoperative bleeding.

Current Knowledge Regarding Long-Term Consequences of Pediatric Intensive Care: A Staff Survey in Intensive Care Units in German-Speaking Countries.

Background: The Post Intensive Care Syndrome (PICS) describes new impairments of physical, cognitive, social, or mental health after critical illness. In recent years, prevention and therapy concepts have been developed. However, it is unclear whether and to what extent these concepts are known and implemented in hospitals in German-speaking countries. Methods: We conducted an anonymous online survey in German-speaking pediatric intensive care units on the current state of knowledge about the long-term consequences of intensive care treatment as well as about already established prevention and therapy measures. The request to participate in the survey was sent to the heads of the PICUs of 98 hospitals. Results: We received 98 responses, 54% of the responses came from nurses, 43% from physicians and 3% from psychologist, all working in intensive care. As a main finding, our survey showed that for only 31% of the respondents PICS has an importance in their daily clinical practice. On average, respondents estimated that about 42% of children receiving intensive care were affected by long-term consequences after intensive care. The existence of a follow-up outpatient clinic was mentioned by 14% of the respondents. Frequent reported barriers to providing follow-up clinics were lack of time and staff. Most frequent mentioned core outcome parameters were normal developmental trajectory (59%) and good quality of life (52%). Conclusion: Overall, the concept of PICS seems to be underrepresented in German-speaking pediatric intensive care units. It is crucial to expand knowledge on long-term complications after pediatric critical care and to strive for further research through follow-up programs and therewith ultimately improve long-term outcomes.

Modified ABCDEF-Bundles for Critically Ill Pediatric Patients - What Could They Look Like?

Background and Significance: Advances in pediatric intensive care have led to markedly improved survival rates in critically ill children. Approximately 70% of those children survive with varying forms of complex chronic diseases or impairment/disabilities. Length of stay, length of mechanical ventilation and number of interventions per patient are increasing with rising complexity of underlying diseases, leading to increasing pain, agitation, withdrawal symptoms, delirium, immobility, and sleep disruption. The ICU-Liberation Collaborative of the Society of Critical Care Medicine has developed a number of preventative measures for prevention, early detection, or treatment of physical and psychiatric/psychological sequelae of oftentimes traumatic intensive care medicine. These so called ABCDEF-Bundles consist of elements for (A) assessment, prevention and management of pain, (B) spontaneous awakening and breathing trials (SAT/SBT), (C) choice of analgesia and sedation, (D) assessment, prevention and management of delirium, (E) early mobility and exercise and (F) family engagement and empowerment. For adult patients in critical care medicine, research shows significant effects of bundle-implementation on survival, mechanical ventilation, coma, delirium and post-ICU discharge disposition. Research regarding PICS in children and possible preventative or therapeutic intervention is insufficient as yet. This narrative review provides available information for modification and further research on the ABCDEF-Bundles for use in critically ill children. Material and Methods: A narrative review of existing literature was used. Results: One obvious distinction to adult patients is the wide range of different developmental stages of children and the even closer relationship between patient and family. Evidence for pediatric ABCDEF-Bundles is insufficient and input can only be collected from literature regarding different subsections and topics. Conclusion: In addition to efforts to improve analgesia, sedation and weaning protocols with the aim of prevention, early detection and effective treatment of withdrawal symptoms or delirium, efforts are focused on adjusting ABCDEF bundle for the entire pediatric age group and on strengthening families' decision-making power, understanding parents as a resource for their child and involving them early in the care of their children.

Implementation of a Delirium Bundle for Pediatric Intensive Care Patients.

BACKGROUND AND OBJECTIVE: Delirium represents the most common form of acute cerebral dysfunction in critical illness. The prevention, recognition, and treatment of delirium must become the focus of modern pediatric intensive care, as delirium can lead to increased morbidity and mortality. The aim of this study is to evaluate the impact of a delirium bundle consisting of mainly non-pharmacological measures. MATERIAL AND METHODS: This is a pre-/post-implementation study in an interdisciplinary pediatric intensive care unit of a tertiary care university hospital. In the pre-implementation period, pediatric intensive care delirium was monitored using the Sophia Observation withdrawal Symptoms and Pediatric Delirium scale. After introduction of a delirium bundle consisting of non-pharmacological prevention and treatment measures a period of 4 months was interposed to train the PICU staff and ensure that the delirium bundle was implemented consistently before evaluating the effects in the post-implementation period. Data collection included prevalence of delirium and withdrawal, length of PICU stay, duration of mechanical ventilation, and cumulative dose of sedatives and analgesics. RESULTS: A total of 792 critically ill children aged 0-18 years were included in this study. An overall delirium prevalence of 30% was recorded in the pre-implementation group and 26% in the post-implementation group (p = 0.13). A significant reduction in the prevalence of pediatric delirium from was achieved in the subgroup of patients under 5 years of age (27.9 vs. 35.8%, p = 0.04) and in patients after surgery for congenital heart disease (28.2 vs. 39.5%, p = 0.04). Young age, length of PICU stay, and iatrogenic withdrawal syndrome were found to be risk factors for developing delirium. CONCLUSIONS: Based on a validated delirium monitoring, our study gives new information regarding the prevalence of pediatric delirium and the characteristics of intensive care patients at risk for this significant complication. Especially young patients and patients after surgery for congenital heart disease seem to benefit from the implementation of non-pharmacological delirium bundles. Based on our findings, it is important to promote change in pediatric intensive care-toward a comprehensive approach to prevent delirium in critically ill children as best as possible.

Severe adverse events in children with tracheostomy and home mechanical ventilation - Comparison of pediatric home care and a specialized pediatric nursing care facility.

BACKGROUND: Advances in medical care and ventilator technologies increase the number of children with tracheostomy and home mechanical ventilation (HMV). Data on severe adverse events in home care and in specialized nursing care facilities are limited. PATIENTS AND METHODS: Retrospective analysis of incidence and type of severe adverse events in children with tracheostomy and HMV in home care compared to a specialized nursing care facility over a 7-year period. RESULTS: 163.9 patient-years in 70 children (home care: 110.7 patient-years, 24 patients; nursing care facility: 53.2 patient-years, 46 patients) were analyzed. In 34 (48.6%) patients tracheostomy was initiated at the age of <1 year. 35 severe adverse events were identified, incidence of severe adverse events per patient-year was 0.21 (median 0.0 (0.0-3.0)). We observed no difference in the rate of severe adverse events between home care and specialized nursing care facility (0.21 [y-1]; median 0.0 (0.0-3.0) versus 0.23 [y-1]; median 0.0 (0.0-1.6); p = 0.690), however, significantly more tracheostomy related incidents and infections occurred in the home care setting. Young age (<1 year) (Odds ratio 3.27; p = 0.045) and feeding difficulties (nasogastric tubes and percutaneous endoscopic gastrostomy) (Odds ratio 9.08; p = 0.016) significantly increased the risk of severe adverse events. Furthermore, the rate of severe adverse events was significantly higher in patients with a higher nursing score. CONCLUSION: Pediatric home mechanical ventilation via tracheostomy is rarely associated with emergencies or adverse events in home care as well as in a specialized nursing care facility setting.

Effects of hypertonic saline on intracranial pressure and cerebral autoregulation in pediatric traumatic brain injury.

OBJECTIVE: Hypertonic saline (HTS) is commonly used in children to lower intracranial pressure (ICP) after severe traumatic brain injury (sTBI). While ICP and cerebral perfusion pressure (CPP) correlate moderately to TBI outcome, indices of cerebrovascular autoregulation enhance the correlation of neuromonitoring data to neurological outcome. In this study, the authors sought to investigate the effect of HTS administration on ICP, CPP, and autoregulation in pediatric patients with sTBI. METHODS: Twenty-eight pediatric patients with sTBI who were intubated and sedated were included. Blood pressure and ICP were actively managed according to the autoregulation index PRx (pressure relativity index to determine and maintain an optimal CPP [CPPopt]). In cases in which ICP was continuously > 20 mm Hg despite all other measures to decrease it, an infusion of 3% HTS was administered. The monitoring data of the first 6 hours after HTS administration were analyzed. The Glasgow Outcome Scale (GOS) score at the 3-month follow-up was used as the primary outcome measure, and patients were dichotomized into favorable (GOS score 4 or 5) and unfavorable (GOS score 1-3) groups. RESULTS: The mean dose of HTS was 40 ml 3% NaCl. No significant difference in ICP and PRx was seen between groups at the HTS administration. ICP was lowered significantly in all children, with the effect lasting as long as 6 hours. The lowering of ICP was significantly greater and longer in children with a favorable outcome (p < 0.001); only this group showed significant improvement of autoregulatory capacity (p = 0.048). A newly established HTS response index clearly separated the outcome groups. CONCLUSIONS: HTS significantly lowered ICP in all children after sTBI. This effect was significantly greater and longer-lasting in children with a favorable outcome. Moreover, HTS administration restored disturbed autoregulation only in the favorable outcome group. This highlights the role of a "rescuable" autoregulation regarding outcome, which might be a possible indicator of injury severity. The effect of HTS on autoregulation and other possible mechanisms should be further investigated.

Ventriculo-arterial coupling in children with Still's murmur.

Still's murmur is the most common innocent heart murmur in children and considered flow related; however, so far the cause of the murmur has not yet been fully explained. Assessment of the hemodynamic ventriculo-arterial interaction and the proportional anatomical dimensions of the left ventricle and the aortic root were the objective for this study. This case-control study was conducted at the Division of Pediatric Cardiology, Vienna Medical University, including healthy children with and without Still's murmur. To assess ventriculo-arterial interaction, the model of ventriculo-arterial coupling (VAC) was applied. The model describes the interaction between the left ventricle (left ventricular contractility, ELV) and the arterial system (effective arterial elastance, EA) by the VAC ratio EA/ELV. The parameters EA and ELV can be derived from M-mode echocardiography thereby allowing a noninvasive pressure-volume analysis. Outcomes comprised VAC ratio and diameters of both the aortic root (AOD) and the left ventricle in end diastole (LVED) and end systole (LVES) as well as their relative proportions, ejection fraction (EF), stroke volume (SV), blood pressure (BP), and heart rate (HR). Forty-three healthy children with Still's murmur (mean age 5.2 years) and 42 healthy children without murmur (mean age 5.8 years) participated in this study. Children with Still's murmur had a significantly lower VAC ratio EA/ELV (0.5 ± 0.13 vs. 0.59 ± 0.15; P < 0.005), a significantly higher EF% (67.1 ± 5.8 vs. 63.3 ± 5.6; P < 0.005, P < 0.01), and a larger SV per kg bodyweight (1.84 ± 0.33 vs. 1.68 ± 0.38; P < 0.05) than controls. BP, HR, and diameters of AOD, LVED, and LVES as well as their relative anatomic proportions did not differ between children with Still's murmur and controls. Still's murmur seems to be generated by a subtle alteration in ventriculo-arterial coupling in healthy children. This result can be translated to parents, as they may be informed that their child's innocent murmur is caused by a more "lively interplay between the heart and the aorta."