German Registry for Cardiac Operations and Interventions in Congenital Heart Disease: Annual Report 2022.

BACKGROUND: The German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease is a voluntary registry initiated by the German Society for Thoracic and Cardiovascular Surgery and the German Society for Pediatric Cardiology and Congenital Heart Defects. Since 2012, the registry collects data for the assessment of treatment and outcomes of surgical and interventional procedures in patients with congenital heart disease (CHD) of all age groups. METHODS: This real-world, prospective all-comers registry collects clinical and procedural characteristics, adverse events (AEs), mortality, and medium-term outcomes (up to 90 days) of patients undergoing surgical and interventional. A unique pseudonymous personal identifier (PID) allows longitudinal data acquisition in case of further invasive treatment in any participating German heart center. Prior to evaluation, all data sets are monitored for data completeness and integrity. Evaluation includes risk stratification of interventional and surgical procedures and classification of AEs. Each year's data are summarized in annual reports containing detailed information on the entire cohort, all subgroups, and 15 index procedures. In addition, each participating center receives an institutional benchmark report for comparison with the national results. This paper presents a comprehensive summary of the annual report 2021. RESULTS: In 2021, a total of 5,439 patients were included by 22 participating centers. In total, 3,721 surgical, 3,413 interventional, and 34 hybrid procedures were performed during 6,122 hospital stays. 2,220 cases (36.3%) could be allocated to the 15 index procedures. The mean unadjusted in-hospital mortality ranged from 0.4% among interventional and 2% among surgical cases up to 6.2 % in cases with multiple procedures. In-hospital mortality among index procedures accounted for 2.3% in total cavopulmonary connection, 20.3% in Norwood procedures, and 0.4% following interventional closure of patent ductus arteriosus. For the remaining seven surgical and five interventional index procedures, no in-hospital deaths were recorded. The 10-year longitudinal evaluation of 1,795 patients after tetralogy of Fallot repair revealed repeat interventional or surgical procedures in 21% of the patients. Over the same period, 31.1% of 2,037 patients, following initial treatment of native coarctation, required at least one additional hospital admission, 39.4% after initial interventional, and 21.3% after initial surgical therapy. CONCLUSION: The annual report 2021 of the German Registry for Cardiac Operations and Interventions in CHD shows continuously good results in accordance with previous data of the registry. Compared to international registries on CHD, it can be ascertained that in Germany invasive treatment of CHD is offered on a high medical level with excellent quality. The proven fact that patients with various malformations like tetralogy of Fallot and coarctation of the aorta require repeat procedures during follow-up confirms the urgent requirement for longitudinal assessment of all patients presenting with complex lesions.

The effect of intraoperative cerebral oxygen desaturations on postoperative cerebral oxygen metabolism in neonates and infants a pilot study.

INTRODUCTION: Cerebral oxygen desaturation during pediatric surgery has been associated with adverse perioperative outcomes. The aim of this pilot study was to analyze the frequency and severity of intraoperative cerebral oxygen desaturations and their impact on postoperative cerebral oxygen metabolism in neonates and infants undergoing pediatric surgery. METHODS: In a prospective pilot study, intra- and postoperative regional cerebral oxygen saturation and blood flow were measured noninvasively using a device combining laser Doppler flowmetry and white-light-spectrometry. Thirty-seven consecutive neonates and infants undergoing noncardiac surgery under general anesthesia for more than 30 min and necessity for invasive arterial blood pressure monitoring were included. Patients with pre-known congenital structural heart disease or cerebral disease were excluded. Continuously brain monitor recording was started in sedated patients before induction of anesthesia (preoperative baseline) and was completed 1 h postoperatively in the PICU in sedated, intubated, and mechanically ventilated states at the PICU (postoperative state). Baseline and postoperative state for cerebral fractional tissue oxygen extraction and approximated cerebral metabolic rate of oxygen were calculated. RESULTS: Seventeen (46%) of the 37 studied neonates and infants suffered from intraoperative periods of regional cerebral oxygen desaturation below 20% of the baseline (event group). Severity of cerebral desaturations was median 4.0%min/h [range 0.1-58.7; interquartile range [IQR] 0.99-21.29]. In the event group, the duration of surgery was significantly longer (median 135 min [range 11-260; IQR 113.5-167.0] vs median 46.5 min [range 11-180; IQR 30.5-159.3]; difference of -62.94; 95% confidence interval [CI] -105.17 to -20.71; p = .021). In the event group, cerebral fractional tissue oxygen extraction (median 0.41 [range 0.20-0.55; IQR 0.26-0.44] vs. median 0.27 [range 0.11-0.41; IQR 0.20-0.31]; difference of -0.11; 95% CI -0.17 to -0.05; p = .001) and approximated cerebral metabolic rate of oxygen (median 6.15 arbitrary unit [range 2.69-12.07; IQR 5.12-7.21] vs. median 4.14 arbitrary unit [range 1.78-7.86; IQR 3.82-6.31]; difference of -1.76; 95% CI -3.03 to -0.49; p = .009) were significantly higher and the cerebral regional oxygen saturation (median 58.99% [range 44.87-79.1; IQR 54.26-72.61] vs median 70.94% [range 57.9-86.13; IQR 67.07-76.59]; difference of 10.01; 95% CI 4.13-15.90; p = .002) significantly lower after surgery compared to the nonevent group. DISCUSSION: The increase of approximated cerebral metabolic rate of oxygen could indicate an elevated oxidative energy metabolism in the "stressed" brain, due to repair processes. The increased cerebral fractional tissue oxygen extraction fits with the decreased NIRS cerebral oxygenation. Our data suggest that an increase in cerebral oxygen metabolism was the cause. CONCLUSION: Cerebral oxygen desaturation during major surgery in neonates and infants is associated with early postoperative increased cerebral oxygen extraction and possibly increased cerebral oxygen metabolism.

Prognostic factors and predictive scores for 6-months mortality of hematopoietic stem cell transplantation recipients admitted to the pediatric intensive care unit.

Objective: Despite advances in hematopoietic stem cell transplantation (HSCT), a considerable number of pediatric HSCT patients develops post-transplant complications requiring admission to the pediatric intensive care unit (PICU). The objective of this study was to evaluate clinical findings, PICU supportive therapy and outcome as well as predictive factors for 6-months survival after discharge of HSCT patients from PICU. Study design: This retrospective single-center analysis investigated patient characteristics, microbiological findings, reasons for admission and death of 54 cases accounting for 94 admissions to the PICU of the University Children's Hospital Tuebingen from 2002 to 2017. We compared clinical characteristics between children with and without 6-months survival after discharge from PICU following HSCT. Finally, we assessed the potential prognostic value of the oncological Pediatric Risk of Mortality Score (O-PRISM), the Pediatric Sequential Organ Failure Assessment Score (pSOFA) and the pRIFLE Criteria for Acute Kidney Injury for 6-months survival using Generalized Estimating Equations (GEE) and Receiver Operating Characteristic curves. Results: Respiratory insufficiency, gastroenterological problems and sepsis were the most common reasons for PICU admission. Out of 54 patients, 38 (70%) died during or after their last PICU admission, 30% survived for at least six months. When considering only first PICU admissions, we could not determine prognostic factors for 6-months mortality. In contrast, under consideration of all PICU admissions in the GEE model, ventilation (p=0.03) and dialysis (p=0.007) were prognostic factors for 6-months mortality. Furthermore, pSOFA (p=0.04) and O-PRISM (p=0.02) were independent risk factors for 6-months mortality considering all PICU admissions. Conclusion: Admission of HSCT patients to PICU is still associated with poor outcome and 69% of patients died within 6 months. Need for respiratory support and dialysis are associated with poor outcome. Prediction of 6-months survival is difficult, especially during a first PICU admission. However, on subsequent PICU admissions pSOFA and O-PRISM scores might be useful to predict mortality. These scores should be prospectively evaluated in further studies to verify whether they can identify pediatric HSCT recipients profiting most from transferal to the PICU.

Whole-body perfusion improves intraoperative transfusions in neonatal aortic arch surgery.

OBJECTIVES: Whole-body perfusion is the combination of lower body perfusion and antegrade cerebral perfusion. This perfusion technique is used in some centres when performing aortic arch reconstruction surgery in neonates and infants. Several studies have shown intra- and postoperative benefits of this technique. However, no studies have analysed the impact it may have on the transfusion of blood products and coagulation factors. METHODS: We retrospectively analysed 65 consecutive neonates and infants who underwent aortic arch reconstruction surgery from January 2014 to July 2020. Patients operated from 2014 to 2017 underwent surgery with antegrade cerebral perfusion; in patients who underwent surgery from 2017 to 2020 a whole-body perfusion strategy was used. Demographic, intra- and postoperative parameters were compared as well as intraoperative blood product and coagulation factor transfusions, chest-tube output in the first 24 h and postoperative bleeding complications. RESULTS: Both groups required intraoperative transfusion of red blood cells, fresh frozen plasma, and platelets, as well as substitution of coagulation factors. The amount of transfused volumes of red blood cells, fresh frozen plasma and platelets (P-values 0.01, <0.01 and <0.01) and intraoperative transfusions of fibrinogen and von Willebrand factor were significantly lower in the whole-body perfusion group (P-value 0.04 and <0.01). CONCLUSIONS: A whole-body perfusion strategy may lead to fewer intraoperative blood product and coagulation factor transfusions when compared to antegrade cerebral perfusion alone in neonates and infants undergoing complex aortic arch reconstruction surgery.

Successful on-ECLS Repair of CDH and Omphalocele in a Newborn.

Both congenital diaphragmatic hernias (CDHs) and omphaloceles show relevant overall mortality rates as individual findings. The combination of the two has been described only sparsely in the literature and almost always with a fatal course. Here, we describe a term neonate with a rare high-risk constellation of left-sided CDH and a large omphalocele who was successfully treated on extracorporeal life support (ECLS). Prenatally, the patient was diagnosed with a large omphalocele and a left CDH with a lung volume of ∼27% and an observed to expected lung-to-head ratio of 30%. Due to respiratory insufficiency, an ECLS device was implanted. As weaning from ECLS was not foreseeable, the female infant underwent successful surgery on ECLS on the ninth day of life. Perioperative high-frequency oscillatory ventilation and circulatory and coagulation management under point-of-care monitoring were the main anesthesiological challenges. Over the following 3 days, ECLS weaning was successful, and the patient was extubated after another 43 days. Surgical treatment on ECLS can expand the spectrum of therapy in high-risk constellations if potential risks are minimized and there is close interdisciplinary cooperation.

Left Atrioventricular Transvalvular Pressure Gradients Derived from Intraoperative and Postoperative Echocardiograms following Atrioventricular Septal Defect Repair.

BACKGROUND: Left atrioventricular valve (LAVV) stenosis following an atrioventricular septal defect (AVSD) repair is a rare but potentially life-threatening complication. While echocardiographic quantification of diastolic transvalvular pressure gradients is paramount in the evaluation of a newly corrected valve function, it is hypothesized that these measured gradients are overestimated immediately following a cardiopulmonary bypass (CPB) due to the altered hemodynamics when compared to postoperative valve assessments using awake transthoracic echocardiography (TTE) upon recovery after surgery. METHODS: Out of the 72 patients screened for inclusion at a tertiary center, 39 patients undergoing an AVSD repair with both intraoperative transesophageal echocardiograms (TEE, performed immediately after a CPB) and an awake TTE (performed prior to hospital discharge) were retrospectively selected. The mean (MPGs) and peak pressure gradients (PPGs) were quantified using a Doppler echocardiography and other measures of interest were recorded (e.g., a non-invasive surrogate of the cardiac output and index (CI), left ventricular ejection fraction, blood pressures and airway pressures). The variables were analyzed using the paired Student's t-tests and Spearman's correlation coefficients. RESULTS: The MPGs were significantly higher in the intraoperative measurements when compared to the awake TTE (3.0 ± 1.2 vs. 2.3 ± 1.1 mmHg; p < 0.01); however, the PPGs did not significantly differ (6.6 ± 2.7 vs. 5.7 ± 2.8 mmHg; p = 0.06). Although the assessed intraoperative heart rates (HRs) were also higher (132 ± 17 vs. 114 ± 21 bpm; p < 0.001), there was no correlation found between the MPG and the HR, or any other parameter of interest, at either time-point. In a further analysis, a moderate to strong correlation was observed in the linear relationship between the CI and the MPG (r = 0.60; p < 0.001). During the in-hospital follow-up period, no patients died or required an intervention due to LAVV stenosis. CONCLUSIONS: The Doppler-based quantification of diastolic transvalvular LAVV mean pressure gradients using intraoperative transesophageal echocardiography seems to be prone to overestimation due to altered hemodynamics immediately after an AVSD repair. Thus, the current hemodynamic state should be taken into consideration during the intraoperative interpretation of these gradients.

Intraoperative monitoring of cerebrovascular autoregulation in infants and toddlers receiving major elective surgery to determine the individually optimal blood pressure - a pilot study.

Introduction: Inducing general anesthesia (GA) in children can considerably affect blood pressure, and the rate of severe critical events owing to this remains high. Cerebrovascular autoregulation (CAR) protects the brain against blood-flow-related injury. Impaired CAR may contribute to the risk of cerebral hypoxic-ischemic or hyperemic injury. However, blood pressure limits of autoregulation (LAR) in infants and children are unclear. Materials and methods: In this pilot study CAR was monitored prospectively in 20 patients aged <4 years receiving elective surgery under GA. Cardiac- or neurosurgical procedures were excluded. The possibility of calculating the CAR index hemoglobin volume index (HVx), by correlating near-infrared-spectroscopy (NIRS)-derived relative cerebral tissue hemoglobin and invasive mean arterial blood pressure (MAP) was determined. Optimal MAP (MAPopt), LAR, and the proportion of time with a MAP outside LAR were determined. Results: The mean patient age was 14 ± 10 months. MAPopt could be determined in 19 of 20 patients, with an average of 62 ± 12 mmHg. The required time for a first MAPopt depended on the extent of spontaneous MAP fluctuations. The actual MAP was outside the LAR in 30% ± 24% of the measuring time. MAPopt significantly differed among patients with similar demographics. The CAR range averaged 19 ± 6 mmHg. Using weight-adjusted blood pressure recommendations or regional cerebral tissue saturation, only a fraction of the phases with inadequate MAP could be identified. Conclusion: Non-invasive CAR monitoring using NIRS-derived HVx in infants, toddlers, and children receiving elective surgery under GA was reliable and provided robust data in this pilot study. Using a CAR-driven approach, individual MAPopt could be determined intraoperatively. The intensity of blood pressure fluctuations influences the initial measuring time. MAPopt may differ considerably from recommendations in the literature, and the MAP range within LAR in children may be smaller than that in adults. The necessity of manual artifact elimination represents a limitation. Larger prospective and multicenter cohort studies are necessary to confirm the feasibility of CAR-driven MAP management in children receiving major surgery under GA and to enable an interventional trial design using MAPopt as a target.

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.

Abdominal and Peripheral Tissue Oxygen Supply during Selective Lower Body Perfusion for the Surgical Repair of Congenital Heart Disease: A Pilot Study.

Background: Lower body perfusion (LBP) may be a strategy for maintaining organ perfusion during congenital heart disease surgery. It is hypothesized that renal and lower limb oxygen supply during LBP is superior to off-pump surgery and comparable to that of a standard cardiopulmonary bypass (CPB). Methods: in this prospective single-center study, patients aged <1 year were recruited if they were scheduled for a correction of aortic arch anomalies using antegrade cerebral perfusion and LBP (group 1), a repair of coarctation during aortic cross-clamping (group 2), or surgery under whole-body CPB (group 3). Renal (prefix "r") and peripheral (prefix "p") oxygen saturation (SO2), hemoglobin amount (Hb), blood velocity (Velo), and blood flow (Flow) were measured noninvasively. Results: A total of 23 patients were included (group 1, n = 9; group 2, n = 5; group 3, n = 9). Compared to the baseline values, rSO2 and pSO2 decreased significantly in group 2 compared to groups 1 and 3. Conversely, rHB significantly increased in group 2 compared to groups 1 and 3, reflecting abdominal venous stasis. Compared to group 3, group 1 showed a significantly lower pFlow during CPB; however, rFlow, pFlow, and pVelo did not differ. Conclusion: according to these observations, LBP results in an improved renal oxygen supply compared to off-pump surgery and may prove to be a promising alternative to conventional CPB.

Routine Detachment of the Anterior and Septal Tricuspid Leaflets Simplifies VSD Closure and Improves the Outcomes.

Background and Objectives: The closure of perimembranous ventricular septal defects (VSDs) is one of the most common surgeries performed in infancy. The technique of detachment of the anterior and septal leaflets of the tricuspid valve (TV) with subsequent leaflet augmentation is frequently used for isolated as well as non-isolated VSD closure. In this study, we compared the incidence of tricuspid regurgitation (TR) in patients who underwent a VSD repair with and without detachment of the TV in the short- and long-term follow-up. Materials and Methods: A retrospective study that included 140 patients who underwent perimembranous VSD closure at our center from 2011-2016, where 102 of these patients underwent the procedure with detachment of the TV, was performed. The follow-up data were obtained from postoperative echocardiography performed in the follow-up visits. A total of 62 patients underwent follow-up at our center, where the follow-up time ranged from 1 to 9 years, with a mean of 71 ± 2.47 months. Results: Regarding patients who underwent a VSD repair with a detachment of the TV, 98.1% of the patients had none to mild TR, compared to 94.7% in patients without intraoperative TV detachment at the time of discharge. There were no reported cases of moderate to severe TR, atrioventricular blocks, aortic insufficiency, or deaths. A total of 98.1% of patients who underwent follow-up at our center with a TV detachment had none to mild TR compared to 94.7% in the group without TV detachment. Conclusion: TV detachment with leaflet augmentation for VSD closure is safe and effective and does not increase the incidence of TR in the short- and long-term follow-up.

Identifying the optimal blood pressure for cerebral autoregulation in infants after cardiac surgery by monitoring cerebrovascular reactivity-A pilot study.

BACKGROUND: Advances in the treatment of pediatric congenital heart disease have increased survival rates. Despite efforts to prevent neurological injury, many patients suffer from impaired neurodevelopmental outcomes. Compromised cerebral autoregulation can increase the risk of brain injury following pediatric cardiac surgery with cardiopulmonary bypass. Monitoring autoregulation and maintaining adequate cerebral blood flow can help prevent neurological injury. AIMS: Our objective was to evaluate autoregulation parameters and to define the optimal blood pressure as well as the lower and upper blood pressure limits of autoregulation. METHODS: Autoregulation was monitored prospectively in 36 infants after cardiopulmonary bypass surgery for congenital heart defects between January and December 2019. Autoregulation indices were calculated by correlating invasive arterial blood pressure, cortical oxygen saturation, and relative tissue hemoglobin levels with near-infrared spectroscopy parameters. RESULTS: The mean patient age was 4.1 ± 2.8 months, and the mean patient weight was 5.2 ± 1.8 kg. Optimal mean arterial pressure could be identified in 88.9% of patients via the hemoglobin volume index and in 91.7% of patients via the cerebral oxygenation index, and a lower limit of autoregulation could be found in 66.7% and 63.9% of patients, respectively. No significant changes in autoregulation indices at the beginning or end of the monitoring period were observed. In 76.5% ± 11.1% and 83.8% ± 9.9% of the 8 and 16 h monitoring times, respectively, the mean blood pressure was inside the range of intact autoregulation (below in 21.5% ± 25.4% and 11.3% ± 16.5% and above in 8.7% ± 10.4% and 6.0% ± 11.0%, respectively). The mean optimal blood pressure was 57.4 ± 8.7 mmHg and 58.2 ± 7.9 mmHg and the mean lower limit of autoregulation was 48.8 ± 8.3 mmHg and 45.5 ± 6.7 mmHg when generated via the hemoglobin volume index and cerebral oxygenation index, respectively. CONCLUSIONS: Postoperative noninvasive autoregulation monitoring after cardiac surgery in children can be reliably and safely performed using the hemoglobin volume index and cerebral oxygenation index and provides robust data. This monitoring can be used to identify individual hemodynamic targets to optimize autoregulation, which differs from those recommended in the literature. Further evaluation of this subject is needed.

Interventional closure of a bronchopleural fistula in a 2 year old child with detachable coils.

BACKGROUND: Bronchopleural fistula (BPF) is a severe complication following pneumonia or pulmonary surgery, resulting in persistent air leakage (PAL) and pneumothorax. Surgical options include resection, coverage of the fistula by video-assisted thoracoscopic surgery (VATS), or pleurodesis. Interventional bronchoscopy is preferred in complex cases and involves the use of sclerosants, sealants and occlusive valve devices. CASE PRESENTATION: A 2.5-year-old girl was admitted to our hospital with persistent fever, cough and dyspnoea. Clinical and radiological examination revealed right-sided pneumonia and pleural effusion. The child was started on antibiotics, and the effusion was drained by pleural drainage. Following removal of the chest tube, the child developed tension pneumothorax. Despite insertion of a new drain, the air leak persisted. Thoracoscopic debridement with placement of another new drain was performed after 4 weeks, without abolishment of the air leak. Bronchoscopy with bronchography revealed a BPF in right lung segment 3 (right upper-lobe anterior bronchus). We opted for an interventional approach that was performed under general anaesthesia during repeat bronchoscopy. Following bronchographic visualisation of the fistula, a 2.7 French microcatheter was placed in right lung segment 3 (upper lobe), allowing occlusion of the fistula by successive implantation of 4 detachable high-density packing volume coils, which were placed into the fistula. Subsequent bronchography revealed no evidence of residual leakage, and the chest tube was removed 2 days later. The chest X-ray findings normalized, and follow-up over 4 years was uneventful. CONCLUSIONS: Bronchoscopic superselective occlusion of BPF using detachable high-density packing large-volume coils was a successful minimally invasive therapeutic intervention performed with minimal trauma in this child and has not been reported thus far. In our small patient, the short interventional time, localized intervention and minimal damage in the lung seemed superior to the corresponding outcomes of surgical lobectomy or pleurodesis in a young growing lung, enabling normal development of the surrounding tissue. Follow-up over 4 years did not show any side effects and was uneventful, with normal lung-function test results to date.

Impact of Whole-Body Perfusion in Postoperative Outcomes After Aortic Arch Reconstruction Surgery in Neonates and Infants.

INTRODUCTION: The aim of this study was to determine whether whole-body perfusion (WBP) consisting of a combined antegrade cerebral perfusion (ACP) and lower body perfusion (LBP) improves the outcome after aortic arch reconstruction surgery in neonates compared with ACP. METHODS: Sixty-five consecutive patients under one year of age who underwent aortic arch reconstruction as the main procedure or as part of a more complex surgery from 2014-2020 in our center were included. The patients were separated into two groups, according to the perfusion strategy, either ACP (34 patients) as the control group or WBP (31 patients) as the intervention group. LBP was achieved through an arterial sheath in the femoral artery. Outcome parameters were postoperative renal, gastrointestinal, and neurological complications and 30-day mortality. RESULTS: The patients in the WBP group showed lower intraoperative lactate levels and close to normal early postoperative renal and hepatic enzymes and LDH at PICU admission compared with the patients in the ACP group. The number of patients suffering from postoperative neurological complications and multiorgan failure was lower in the WBP group. CONCLUSION: In our experience, the combined use of ACP and LBP through the femoral artery showed an improvement, regarding postoperative neurologic complications in neonates and infants undergoing aortic arch surgery.

Spectrum of Interventional Procedures During Hybrid Central Line Placement in Pediatric Intestinal Rehabilitation Patients With End-Stage Vascular Access.

Background: Loss of available central vein access sites for parenteral nutrition delivery represents one of the main indications for intestinal transplantation in children with intestinal failure. Placement of central venous catheters can be challenging in advanced loss of patent venous pathways. We recently described the hybrid technique (interventional plus surgical approach) of central line placement in children. The aim of this study was to describe and analyze the interventions used during the hybrid procedures regarding feasibility, safety and outcome. Methods: We retrospectively analyzed the course of all children in our intestinal rehabilitation program undergoing hybrid central line placement. We evaluated patients' conditions, interventional techniques and surgical peculiarities as well as outcome. Results: 203 children were treated in our intestinal rehabilitation program between 2010 and 2021. Due to loss of venous access, hybrid technique was performed in 53 children during 76 interventions. In 40 cases the same vessel was reused via Seldinger technique. Among the 30 ultrasound-guided new vessel punctures, 12 were performed by puncture of collateral vessels. Extended interventions due to thoracic central venous obstruction and/or thrombosis requiring additional access via a femoral vein for rehabilitation of the vascular system was performed during 29 procedures including catheter extraction (1), angioplasties (18), stent placement (1), revascularization (5) and thrombectomy (4). Placement of a central line was not possible in 6 children which eventually underwent extended thoracic/vascular surgery: in three children the previously placed catheter could not be removed, in one child, placement of a thrombectomy-catheter was not possible because of inferior vena cava occlusion, and in two children, revascularization failed. Intestinal transplantation was considered in one patient because of impending loss of vascular access. Two self-limiting minor extravasations and one intervention-associated pericardial effusion occurred. Conclusions: Hybrid interventions for central venous catheter placement and vascular rehabilitation enable a high success rate in children with intestinal failure and end-stage vascular access, circumventing the need for intestinal transplantation or advanced surgery. The relevant procedures are complex and require a foresighted and individualized approach with a wide range of interventional techniques. If performed with expertise, this combined interventional/surgical approach is feasible and safe.

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.

Post-operative kinetics of C-reactive protein to distinguish between bacterial infection and systemic inflammation in infants after cardiopulmonary bypass surgery: the early and the late period.

OBJECTIVES: Differentiation between post-operative inflammation and bacterial infection remains an important issue in infants following congenital heart surgery. We primarily assessed kinetics and predictive value of C-reactive protein for bacterial infection in the early (days 0-4) and late (days 5-28) period after cardiopulmonary bypass surgery. Secondary objectives were frequency, type, and timing of post-operative infection related to the risk adjustment for congenital heart surgery score. METHODS: This 3-year single-centre retrospective cohort study in a paediatric cardiac ICU analysed 191 infants accounting for 235 episodes of CPBP surgery. Primary outcome was kinetics of CRP in the first 28 days after CPBP surgery in infected and non-infected patients. RESULTS: We observed 22 infectious episodes in the early and 34 in the late post-operative period. CRP kinetics in the early post-operative period did not accurately differentiate between infected and non-infected patients. In the late post-operative period, infected infants displayed significantly higher CRP values with a median of 7.91 (1.64-22.02) and 6.92 mg/dl (1.92-19.65) on days 2 and 3 compared to 4.02 (1.99-15.9) and 3.72 mg/dl (1.08-9.72) in the non-infection group. Combining CRP on days 2 and 3 after suspicion of infection revealed a cut-off of 9.47 mg/L with an acceptable predictive accuracy of 76%. CONCLUSIONS: In neonates and infants, CRP kinetics is not useful to predict infection in the first 72 hours after CPBP surgery due to the inflammatory response. However, in the late post-operative period, CRP is a valuable adjunctive diagnostic test in conjunction with clinical presentation and microbiological diagnostics.

Sedation practices during high dose rate brachytherapy for children with urogenital and perianal rhabdomyosarcoma.

BACKGROUND: A novel concept for an organ-preserving treatment of pediatric urogenital and perianal rhabdomyosarcoma includes high dose rate brachytherapy following surgical tumor resection. For the duration of the brachytherapy of 6 days plus 2-day recovery break the patients are not allowed to move and are kept under deep sedation, which can lead to difficult weaning from mechanical ventilation, withdrawal, delirium, and prolonged hospital stay. The aim of this study was to evaluate a protocol which includes a switch from fentanyl to ketamine 3 days prior to extubation to help ensure a rapid extubation and transfer from PICU. METHODS: Patients who underwent surgical tumor resection of rhabdomyosarcoma and subsequent brachytherapy were treated according to a standardized protocol. We evaluated doses of fentanyl, midazolam and clonidine, time of extubation, length of PICU stay and occurrence of withdrawal symptoms and delirium. We compared fentanyl dose at time of extubation, duration of weaning from mechanical ventilation and time to discharge from PICU with patients after isolated severe traumatic brain injury. RESULTS: Twentytwo patients (age 39.9 ± 29.8 months) were treated in our PICU to undergo brachytherapy. Extubation was performed 21.6 ± 13.5 h after the last brachytherapy session with an average fentanyl dose of 1.5 ± 0.5 µg/kg/h and patients were discharged from PICU 58.4 ± 30.3 h after extubation, which all is significantly lower compared to the control group (extubation after 88.0 ± 42.2 h, p < 0.001; fentanyl dose at the time of extubation 2.5 ± 0.6 µg/kg/h, p < 0.001; PICU discharge after 130.1 ± 148.4 h, p < 0.009). Withdrawal symptoms were observed in 9 patients and delirium in 13 patients. CONCLUSION: A standardized analgesia and sedation protocol including an opioid break, scoring systems to detect withdrawal symptoms and delirium, and tapering plans contributes to successful early extubation and discharge from PICU after long-term deep sedation.

A Comprehensive Functional Analysis in Patients after Atrial Switch Surgery.

BACKGROUND: Long-term course after atrial switch operation is determined by increasing right ventricular (RV) insufficiency. The aim of our study was to investigate subtle functional parameters by invasive measurements with conductance technique and noninvasive examinations with cardiac magnetic resonance imaging (CMR). METHODS: We used invasive (pressure-volume loops under baseline conditions and dobutamine) and noninvasive techniques (CMR with feature tracking [FT] method) to evaluate RV function. All patients had cardiopulmonary exercise testing (CPET). RESULTS: From 2011 to 2013, 16 patients aged 28.2 ± 7.3 (22-50) years after atrial switch surgery (87.5% Senning and 12.5% Mustard) were enrolled in this prospective study. All patients were in New York Heart Association (NYHA) class I to II and presented mean peak oxygen consumption of 30.1 ± 5.7 (22.7-45.5) mL/kg/min. CMR-derived end-diastolic volume was 110 ± 22 (78-156) mL/m2 and RV ejection fraction 41 ± 8% (25-52%). CMR-FT revealed lower global systolic longitudinal, radial, and circumferential strain for the systemic RV compared with the subpulmonary left ventricle. End-systolic elastance (Ees) was overall reduced (compared with data from the literature) and showed significant increase under dobutamine (0.80 ± 0.44 to 1.89 ± 0.72 mm Hg/mL, p ≤ 0.001), whereas end-diastolic elastance (Eed) was not significantly influenced (0.11 ± 0.70 to 0.13 ± 0.15 mm Hg/mL, p = 0.454). We found no relevant relationship between load-independent conductance indices and strain or CPET parameters. Conductance analysis revealed significant mechanical dyssynchrony, higher during diastole (mean 30 ± 4% baseline, 24 ± 6% dobutamine) than during systole (mean 17 ± 6% baseline, 19 ± 7% dobutamine). CONCLUSIONS: Functional assessment of a deteriorating systemic RV remains demanding. Conductance indices as well as the CMR-derived strain parameters showed overall reduced values, but a significant relationship was not present (including CPET). Our conductance analysis revealed intraventricular and predominantly diastolic RV dyssynchrony.

Persistent common arterial trunk with hexaleaflet truncal valve and intact ventricular septum.

We report a case of a 3-week-old infant who presented a heart murmur and low oxygen saturation. An echocardiography was performed and presented a common arterial trunk type B4 with an interrupted aortic arch and intact ventricular septum. We describe the surgical management and short-term follow-up.