Pulmonary blood flow in children with univentricular heart and unilateral diaphragmatic paralysis.

OBJECTIVES: Spontaneous breathing has an important effect on pulmonary arterial blood flow in patients with Glenn/Fontan circulation. Unilateral diaphragmatic paralysis (DP) is a frequent complication after heart surgery in congenital heart disease. The aim of this study was to investigate the influence of unilateral DP on blood flow distribution in the pulmonary arteries with Glenn/Fontan circulation. METHODS: Magnetic resonance phase-contrast imaging was used to evaluate stroke volume index (SVI) in the left and right pulmonary arteries in patients with Glenn/Fontan circulation with unilateral DP. Data for 18 patients with univentricular heart and unilateral DP were analysed, 8 in the Glenn stage and 10 in the Fontan stage. Ten patients had right-sided DP, and 8 had left-sided DP. A diaphragmatic plication was performed in 7 patients. The control group consisted of 36 patients with Glenn (n = 16)/Fontan (n = 20) circulation without DP. RESULTS: In both left- and right-sided DP, the SVI to the ipsilateral side was significantly lower than in controls [2.81 (1.45-4.50) ml/m2 left vs 11.97 (7.36-16.37) ml/m2 in controls, P < 0.0002; 8.2 (4.49-12.64) ml/m2 with right vs 12.64 (9.66-16.61) ml/m2 in controls; P = 0.0284]. The SVI to the contralateral side showed a slight but non-significant increase in the presence of unilateral DP. CONCLUSIONS: Unilateral DP in patients with Glenn/Fontan circulation has a negative impact on pulmonary arterial SVI on the side of the paralysis.

Implementation of 2D Barcode Medication Labels and Smart Pumps in Pediatric Acute Care: Lessons Learned.

BACKGROUND: In pediatric intensive care, prescription, administration, and interpretation of drug doses are weight dependent. The use of standardized concentrations simplifies the preparation of drugs and increases safety. For safe administration as well as easy interpretation of intravenous drug dosing regimens with standardized concentrations, the display of weight-related dose rates on the infusion device is of pivotal significance. OBJECTIVES: We report on challenges in the implementation of a new information technology-supported medication workflow. The workflow was introduced on eight beds in the pediatric heart surgery intensive care unit as well as in the pediatric anesthesia at the University of Bonn Medical Center. The proposed workflow utilizes medication labels generated from prescription data from the electronic health record. The generated labels include a two-dimensional barcode to transfer data to the infusion devices. METHODS: Clinical and technical processes were agilely developed. The reliability of the system under real-life conditions was monitored. User satisfaction and potential for improvement were assessed. In addition, a structured survey among the nursing staff was performed. The questionnaire addressed usability as well as the end-users' perception of the effects on patient safety. RESULTS: The workflow has been applied 44,111 times during the pilot phase. A total of 114 known failures in the technical infrastructure were observed. The survey showed good ratings for usability and safety (median "school grade" 2 or B for patient safety, intelligibility, patient identification, and handling). The medical management of the involved acute care facilities rated the process as clearly beneficial regarding patient safety, suggesting a rollout to all pediatric intensive care areas. CONCLUSION: A medical information technology-supported medication workflow can increase user satisfaction and patient safety as perceived by the clinical end-users in pediatric acute care. The successful implementation benefits from an interdisciplinary team, active investigation of possible associated risks, and technical redundancy.

Evaluation of the Roche point of care system for determination of NT-proBNP in urine samples.

INTRODUCTION: The diagnostic potential of urine NT-pro-BNP has been studied recently and shows great promise, especially for children. Bedside serum-NT-proBNP determination with point of care testing (POCT) is established in adults. This study investigated the analytical capabilities of NT-proBNP POCT measurements for urine-samples. Furthermore, the stability of this biomarker in urine samples at room-temperature was explored. METHODS: 104 urine samples were analysed, partly with double and triple determinations and compared to the established Elecsys system. To investigate the stability of NT-proBNP in urine, five samples were stored at room temperature and analysed 12-hourly over 96 h. RESULTS: 34 % of samples lay outside the POCT range (60 pg/ml-9000 pg/ml). The 69 values within the range were correlated with those measured by Elecsys (correlation coefficient of 0.92; p < 0.001). Linear regression yields an r2 of 0.84. The stability of urine NT-proBNP was shown with correlation coefficients between 0.97 and 1 (p < 0.001). CONCLUSION: Analysing NT-ProBNP in urine with cobas h232 POC system is feasible, but the detection range needs to be adjusted. As urine NT-proBNP proved stable for at least 96 h even postal shipping would be possible. Adjusting this system's measuring range could help introduce a new, non-invasive, pain-free parameter.

Clinical characteristics and outcome of biopsy-proven myocarditis in children - Results of the German prospective multicentre registry "MYKKE".

BACKGROUND: Heart failure (HF) due to myocarditis might not respond in the same way to standard therapy as HF due to other aetiologies. The aim of this study was to investigate the value of endomyocardial biopsies (EMB) for clinical decision-making and its relation to the outcome of paediatric patients with myocarditis. METHODS: Clinical and EMB data of children with myocarditis collected for the MYKKE-registry between 2013 and 2020 from 23 centres were analysed. EMB studies included histology, immunohistology, and molecular pathology. The occurrence of major adverse cardiac events (MACE) including mechanical circulatory support (MCS), heart transplantation, and/or death was defined as a combined endpoint. RESULTS: Myocarditis was diagnosed in 209/260 patients: 64% healing/chronic lymphocytic myocarditis, 23% acute lymphocytic myocarditis (AM), 14% healed myocarditis, no giant cell myocarditis. The median age was 12.8 (1.4-15.9) years. Time from symptom-onset to EMB was 11.0 (4.0-29.0) days. Children with AM and high amounts of mononuclear cell infiltrates were significantly younger with signs of HF compared to those with healing/chronic or healed myocarditis. Myocardial viral DNA/RNA detection had no significant effect on outcome. The worst event-free survival was seen in patients with healing/chronic myocarditis (24%), followed by acute (31%) and healed myocarditis (58%, p = 0.294). A weaning rate of 64% from MCS was found in AM. CONCLUSIONS: EMB provides important information on the type and stage of myocardial inflammation and supports further decision-making. Children with fulminant clinical presentation, high amounts of mononuclear cell infiltrates or healing/chronic inflammation and young age have the highest risk for MACE.

Performance of urinary NT-proBNP in ambulatory settings.

BACKGROUND: NT-proBNP (N-terminal prohormone of brain natriuretic peptide) has been established as a useful biomarker in plasma for children with congenital heart disease (CHD). Plasma values were shown to correlate well with urinary values. We designed a study to investigate the general utility of urinary NT-proBNP in children with and without CHD in an ambulatory setting. MATERIAL AND METHODS: 202 children (mean age 93 months (1-225 months)) were included in the analysis. We investigated the performance of urinary NT-proBNP values determined from spot urine as a diagnostic tool for different forms of congenital heart disease. RESULTS: Urinary NT-proBNP is a good diagnostic tool for children with congenital heart disease (ROC area under the curve 0.807). Combining these values with the Ross-classification further improves the diagnostic power (ROC area under the curve 0.831) Analysis also showed significant differences between Lg10 urinary NT-proBNP values of healthy controls and those of children after corrective surgery. Furthermore, children who have completed the stages of Fontan palliation showed higher values than age matched controls. CONCLUSIONS: Urinary NT-proBNP can be used in an ambulatory setting to discriminate between relevant and nonrelevant CHD and might be valuable as a follow up parameter for children after biventricular repair or univentricular palliation. Age dependant urinary NT-proBNP normal values for children could be an easy-to-use tool for general practitioners as well as specialised clinics.

Perioperative urinary NT-ProBNP values and their usefulness as diagnostic and prognostic markers in children with congenital heart disease.

INTRODUCTION: NT-proBNP and especially the changes in values are important markers in patients with congenital heart disease (CHD). NT-proBNP values determined from a urine sample correlate well with the plasma values of NT-proBNP. This study investigated the perioperative development of plasma and urinary values, examining their diagnostic and prognostic value. METHODS: 83 children undergoing cardiac surgery for a myriad of CHDs were included. Urine and plasma samples were collected at different points in time. Urinary values were corrected for urine creatinine concentration and transformed into Lg10-values. RESULTS: The correlation between urine and plasma is weaker postoperatively (r = 0.70-0.80) in comparison to preoperatively (r = 0.87). Neonates had higher urinary values than older children. A ROC-analysis for the differentiation between complex and simple CHD showed an area under the curve of 0.854 for zlog-NT-proBNP plasma values and 0.826 for creatinine corrected urine values. A decline of NT-proBNP plasma values from the day before surgery to the time after intubation correlated with the duration of postoperative non-invasive ventilation (r = 0.9, sig. < 0.001). CONCLUSION: Urinary NT-proBNP shows potential in discriminating between complex and simple CHD. This study is the first to show a prognostic role of NT-proBNP in establishing spontaneous respiration postoperatively in children with CHD.

"Body modification: piercing and tattooing in congenital heart disease patients", decoration or disaster?-a narrative review.

OBJECTIVE: Tattoos and piercings are types of body art, which are gaining popularity over the last decades. An increasing number of adolescents and adults with congenital heart disease (CHD) have piercings or tattoos. This review will provide prudent information on the subject for affected patients and health care professionals caring for them. BACKGROUND: Amongst others, local infections are a common complication in up to 20% of all piercings and isolated cases of systemic infections like endocarditis have been reported. Individuals with congenital heart disease are especially susceptible to endocarditis and prone to suffer severe health consequences from it. In terms of tattooing endocarditis is less common but the localization must be well considered as it might interfere with cardiovascular magnetic resonance imaging (CMR), which constitutes an important part of follow up investigations in these patients. METHODS: This article is written as a commentary narrative review and will provide an update on the current literature and available data on common forms of body modification and the potential risks for patients with CHD. CONCLUSIONS: In order to best advise patients and their families, health care professionals must be aware of potential risks accompanying the implementation of body art. Neither the European nor the American guidelines for endocarditis prophylaxis address piercings and tattoos. To our knowledge, there are no clear recommendations concerning piercings and tattoos for adolescents and adults with CHD.

Urinary values of NT-proBNP in children with congenital heart disease - Is it feasible?

Plasma NT-proBNP (N-terminal prohormone of brain natriuretic peptide) is an established clinical biomarker for children with congenital heart disease. In adult studies the relation between plasma and urinary NT-proBNP has been investigated with a good correlation. Considering the age dependence of NT-proBNP in healthy children and the age dependence of kidney function, an investigation of the correlation between NT-proBNP plasma and urinary values in children of different ages is necessary. We analyzed plasma and urine samples of 33 children (mean age 7 months) with congenital heart disease before surgery. Plasma and urinary creatinine were also measured to evaluate the influence of kidney function. A Pearson correlation between Lg10-plasma and Lg10-urine values of NT-proBNP corrected for urine creatinie showed a correlation coefficient of r = 0,902 (P < 0,000) without discriminating for age. This study demonstrates that urinary NT-proBNP values correlate well with plasma NT-proBNP values in infants and toddlers and that single random urine sample corrected to urine-creatinine can be used as an alternative to plasma samples. The use of urinary biomarkers could help reduce the need of stressful blood sampling in infants and children.