Evaluation of a modified paediatric early warning score for children with congenital heart disease.

BACKGROUND: Paediatric early warning score systems are used for early detection of clinical deterioration of patients in paediatric wards. Several paediatric early warning scores have been developed, but most of them are not suitable for children with cyanotic CHD who are adapted to lower arterial oxygen saturation. AIM: The present study compared the original paediatric early warning system of the Royal College of Physicians of Ireland with a modification for children with cyanotic CHD. DESIGN: Retrospective single-centre study in a paediatric cardiology intermediate care unit at a German university hospital. RESULTS: The distribution of recorded values showed a significant shift towards higher score values in patients with cyanotic CHD (p < 0.001) using the original score, but not with the modification. An analysis of sensitivity and specificity for the factor "requirement of action" showed an area under the receiver operating characteristic for non-cyanotic patients of 0.908 (95% CI 0.862-0.954). For patients with cyanotic CHD, using the original score, the area under the receiver operating characteristic was reduced to 0.731 (95% CI 0.637-0.824, p = 0.001) compared to 0.862 (95% CI 0.809-0.915, p = 0.207), when the modified score was used. Using the critical threshold of scores ≥ 4 in patients with cyanotic CHD, sensitivity and specificity for the modified score was higher than for the original (sensitivity 78.8 versus 72.7%, specificity 78.2 versus 58.4%). CONCLUSION: The modified score is a uniform scoring system for identifying clinical deterioration, which can be used in children with and without cyanotic CHD.

Immediate postoperative topical lidocaine gel for the treatment of eye pain following corneal abrasion in descemet membrane endothelial keratoplasty (DMEK) under general anaesthesia: a pilot retrospective analysis.

BACKGROUND: Patients undergoing corneal abrasion as part of Descemet membrane endothelial keratoplasty (DMEK) under general anesthesia suffer from early burning pain postoperatively. This pain appears to be poorly treatable with systemic analgesics. This study aims to evaluate postoperative pain management using topical lidocaine gel after DMEK with iatrogenic corneal abrasion. METHODS: Retrospective analysis of 28 consecutive patients undergoing DMEK with corneal abrasion from October 19, 2021, to November 12, 2021, at a German university hospital. Patients during week 1 and 2 received peri-operative standard pain treatment (cohort S) and additional local lidocaine gel during week 3 and 4 immediately postoperatively (cohort L). RESULTS: 13 patients were included in cohort S and 15 patients in cohort L. At awakening all patients (100%) in cohort S reported burning pain, and six of 15 patients (40%) in cohort L reported burning pain. Burning pain scores were significantly lower in cohort L (p < 0.001 at awakening, p < 0.001 at 10 min, p < 0.001 at 20 min, p < 0.001 at 30 min, p = 0.007 at 40 min after awakening, and p < 0.001 at leaving recovery room). No significant differences between cohort S and cohort L were detected concerning surgical outcome during 1-month-follow-up (p = 0.901 for best corrected visual acuity). CONCLUSION: Patients undergoing DMEK with corneal abrasion suffer significant pain in the recovery room. A single dose of topic lidocaine gel reduces the early postoperative burning pain sufficiently and does not affect the surgical outcome.

Human metapneumovirus infection in the cardiac paediatric ICU before and during COVID-19 pandemic: a retrospective cohort analysis.

INTRODUCTION: This study investigates the hygiene standards in the context of the COVID-19 pandemic and their impact on the perioperative incidence of human metapneumovirus as well as the typical symptom burden of human metapneumovirus-infected children with CHDs. MATERIALS AND METHODS: Between March 2018 and July 2021, all patients of a cardiac paediatric ICU of a German university hospital were included in this retrospective cohort analysis. RESULTS: A total of 589 patients with CHD were included in the analysis. Three hundred and fifty-two patients (148 females and 204 males) were admitted before the introduction of social distancing and face masks between March 2018 and 15 April 2020 (cohort A). Two hundred and thirty-seven patients (118 females and 119 males) were admitted after the introduction between April 16 and July 2021 (cohort B). In cohort A, human metapneumovirus was detected in 11 out of 352 patients (3.1%) during their stay at cardiac paediatric ICU. In cohort B, one patient out of 237 (0.4%) tested positive for human metapneumovirus. Patients who tested positive for human metapneumovirus stayed in cardiac paediatric ICU for a median of 17.5 days (range, 2-45 days). Patients without a detected human metapneumovirus infection stayed in the cardiac paediatric ICU for a median of 4 days (range, 0.5-114 days). Nine out of 12 (75%) human metapneumovirus-positive patients showed atelectasis. CONCLUSION: Perioperative human metapneumovirus infections prolong cardiac paediatric ICU stay in children with CHD. In affected patients, pulmonary impairment with typical symptoms appears. Under certain circumstances, a complication-rich perioperative infection with human metapneumovirus could be prevented in paediatric cardiac high-risk patients by prophylactic hygiene intervention.

[Anaesthesia and Sedation for Diagnostic Procedures in Children]. / Anästhesie und Analgosedierung für diagnostische Eingriffe bei Kindern.

Constant medical progress leads to an increasing range of indications and consequently increasing number of diagnostic procedures in (early) childhood. To prevent lasting traumatization of children (and parents) and to ensure proper examination conditions, adequate (analgo-)sedation or general anesthesia is usually required for the appropriate management of diagnostic procedures in childhood, whether painful or not. The safety of young patients is the first priority. Administrations, hospitals, and each individual anesthesiologist are responsible for establishing structures, experience, and knowledge in this area so that children of all ages receive optimum care. In this article, the authors provide an overview of basic principles (structures, requirements, recommendations), currently used drugs, and procedure-specific examples for providing procedural analgosedation/anesthesia in children.

Dexmedetomidine as a sole sedative for procedural sedation in preterm and neonate infants: A retrospective analysis.

BACKGROUND: Many different sedation concepts for magnetic resonance imaging have been described for prematurely and term-born infants, ranging from "no sedation" to general anesthesia. Dexmedetomidine is an alpha-2 receptor agonist that is frequently used to sedate older children, because the anesthesiologist can easily adjust sedation depth, the patient maintains spontaneous breathing, and awakens rapidly afterwards. AIMS: The present study evaluates whether dexmedetomidine could safely be used as the sole sedative for prematurely and term-born infants less than 60 weeks postconceptional age undergoing diagnostic procedures. METHODS: We performed a retrospective monocentric analysis of n = 39 prematurely and term-born infants (<60 weeks postconceptional age or a body weight <5 kg) who were sedated with dexmedetomidine for an MRI at a German university hospital from August 2016 to November 2018. RESULTS: Successful imaging was achieved in all cases. The median initial bolus of dexmedetomidine administered over 10 min was 1.39 µg kg-1 body weight (range 0.34-3.64 µg kg-1 ), followed with a continuous infusion at a median rate of 1.00 µg kg-1  h-1 (range 0.5-3.5 µg kg-1  h-1 ); however, 3 patients (7%) needed some additional sedation (ketamine or propofol). All patients, including 10 infants who had previously required respiratory support, underwent the procedure without any relevant desaturation or apnea. Bradycardia was observed in up to 15 out of 39 cases (38.5%), but only four (10.3% in total and 26.7% of bradycardia) required atropine. CONCLUSIONS: These results indicate that dexmedetomidine can be safely used for procedural sedation in the high-risk cohort of prematurely and term-born infants less than 60 weeks postconceptional age. Apnea during procedural sedation and subsequent stay in the recovery room is avoided, but bradycardia remains a relevant risk that may require treatment.

[SARS-CoV-2 positive child-What to do if inhalation induction of anesthesia is unavoidable?] / SARS-CoV-2-positives Kind ­ Was tun bei unvermeidbarer inhalativer Narkoseeinleitung?

Induction of anesthesia by inhalation is very common in children due to difficult venous conditions and especially in uncooperative children. During the study on complications in the pediatric anesthesia in Europe (APRICOT study) including almost 30,000 patients, 48% of the children were induced by inhalation.Under the conditions of the corona pandemic, however, induction of anesthesia by inhalation represents an increased risk of infection due to the potential release of aerosols. Rapid sequence induction is recommended for anesthesia induction and definitive airway management for adults and children in the current pandemic situation.The present case demonstrates that there can be situations in children in which induction of anesthesia by inhalation is unavoidable and shows a potential procedure for reducing the risk of infection for the anesthesia personnel.

Impact of Lactate Clearance on Early Outcomes in Pediatric ECMO Patients.

Background and Objectives: Pediatric extracorporeal membrane oxygenation (ECMO) support is often the ultimate therapy for neonatal and pediatric patients with congenital heart defects after cardiac surgery. The impact of lactate clearance in pediatric patients during ECMO therapy on outcomes has been analyzed. Materials andMethods: We retrospectively analyzed data from 41 pediatric vaECMO patients between January 2006 and December 2016. Blood lactate and lactate clearance have been recorded prior to ECMO implantation and 3, 6, 9 and 12 h after ECMO start. Receiver operating characteristic (ROC) analysis was used to identify cut-off levels for lactate clearance. Results: Lactate levels prior to ECMO therapy (9.8 mmol/L vs. 13.5 mmol/L; p = 0.07) and peak lactate levels during ECMO support (10.4 mmol/L vs. 14.7 mmol/L; p = 0.07) were similar between survivors and nonsurvivors. Areas under the curve (AUC) of lactate clearance at 3, 9 h and 12 h after ECMO start were significantly predictive for mortality (p = 0.017, p = 0.049 and p = 0.006, respectively). Cut-off values of lactate clearance were 3.8%, 51% and 56%. Duration of ECMO support and respiratory ventilation was significantly longer in survivors than in nonsurvivors (p = 0.01 and p < 0.001, respectively). Conclusions: Dynamic recording of lactate clearance after ECMO start is a valuable tool to assess outcomes and effectiveness of ECMO application. Poor lactate clearance during ECMO therapy in pediatric patients is a significant marker for higher mortality.

RACHS-1 score as predictive factor for postoperative ventilation time in children with congenital heart disease.

BACKGROUND: Congenital heart disease is the most frequent malformation in newborns. The postoperative mortality of these patients can be assessed with the Risk Adjustment in Congenital Heart Surgery-1 (RACHS-1) score. This study evaluates whether the RACHS-1 score can also be used as a predictor for the length of postoperative ventilation and what is the influence of age. MATERIAL AND METHODS: In a retrospective study over the period from 2007 to 2013, all patient records were evaluated: 598 children with congenital heart disease and cardiac surgery were identified and 39 patients have been excluded because of additional comorbidities. For evaluation of mortality, 559 patients could be analysed, after exclusion of 39 deceased patients, 520 cases remained for analysis of postoperative ventilation. RESULTS: Overall mortality was 7% with a dependency on RACHS-1 categories. The median length of postoperative ventilation rose according to the RACHS-1 categories: RACHS-1 category 1: 9 hours (interquartile range (IQR) 7-13 hours), category 2: 30 hours (IQR 12-85 hours), category 4: 58 hours (IQR 13-135 hours), category 4: 71 hours (IQR 29-165 hours), and category 6: 189 hours (IQR 127-277 hours). Some of the RACHS-1 subgroups differed significantly from the categories, especially the repair of tetralogy of Fallot with a longer ventilation time and strong variability. Younger age was an independent factor for longer postoperative ventilation. CONCLUSION: RACHS-1 is a good predictor for the length of postoperative ventilation after cardiac surgery with the exception of some subgroups. Younger age is another independent factor for longer postoperative ventilation. These data provide better insight into ventilation times and allow better planning of operations in terms of available intensive care beds.

Incidence of peri-operative paediatric cardiac arrest and the influence of a specialised paediatric anaesthesia team: Retrospective cohort study.

BACKGROUND: Peri-operative critical events are still a major problem in paediatric anaesthesia care. Access to more experienced healthcare teams might reduce the adverse event rate and improve outcomes. OBJECTIVE: The current study analysed incidences of peri-operative paediatric cardiac arrest before and after implementation of a specialised paediatric anaesthesia team and training programme. DESIGN: Retrospective cohort study with before-and-after analysis. SETTING: Department of Anaesthesiology and Intensive Care Medicine, University Hospital of Cologne, Germany. PATIENTS: A total of 36 243 paediatric anaesthetics (0 to 18 years) were administered between 2008 and 2016. INTERVENTION: Implementation of a specialised paediatric anaesthesia team and training programme occurred in 2014 This included hands-on supervised training in all fields of paediatric anaesthesia, double staffing for critical paediatric cases and a 24/7 emergency team. A logistic regression analysis with risk factors (age, ASA physical status, emergency) was used to evaluate the impact of implementation of the specialised paediatric anaesthesia team. MAIN OUTCOME MEASURES: Incidences of peri-operative paediatric cardiac arrest and anaesthesia-attributable cardiac arrest before and after the intervention. RESULTS: Twelve of 25 paediatric cardiac arrests were classified as anaesthesia-attributable. The incidence of overall peri-operative paediatric cardiac arrest was 8.1/10 000 (95% CI 5.2 to 12.7) in the period 2008 to 2013 and decreased to 4.6/10 000 (95% CI 2.1 to 10.2) in 2014 to 2016. Likewise, the incidence of anaesthesia-attributable cardiac arrest was lower after 2013 [1.6/10 000 (95% CI 0.3 to 5.7) vs. 4.3/10 000 (95% CI 2.3 to 7.9)]. Using logistic regression, children anaesthetised after 2013 had nearly a 70% lower probability of anaesthesia-attributable cardiac arrest (odds ratio 0.306, 95% CI 0.067 to 1.397; P = 0.1263). For anaesthesia-attributable cardiac arrest, young age was the most contributory risk factor, whereas in overall paediatric cardiac arrest, ASA physical statuses 3 to 5 played a more important role. CONCLUSION: In this study on incidences of peri-operative paediatric cardiac arrest from a European tertiary care university hospital, implementation of a specialised paediatric anaesthesia team and training programme was associated with lower incidences of peri-operative paediatric cardiac arrest and a reduced probability of anaesthesia-attributable cardiac arrest.

A Cross-Sectional Survey of Near-Infrared Spectroscopy Use in Pediatric Cardiac ICUs in the United Kingdom, Ireland, Italy, and Germany.

OBJECTIVES: Despite the increasing use of near-infrared spectroscopy across pediatric cardiac ICUs, there is significant variability and equipoise with no universally accepted management algorithms. We aimed to explore the use of near-infrared spectroscopy in pediatric cardiac ICUs in the United Kingdom, Ireland, Italy, and Germany. DESIGN: A cross-sectional multicenter, multinational electronic survey of one consultant in each pediatric cardiac ICU. SETTING: Pediatric cardiac ICUs in the United Kingdom and Ireland (n = 13), Italy (n = 12), and Germany (n = 33). INTERVENTIONS: Questionnaire targeted to establish use, targets, protocols/thresholds for intervention, and perceived usefulness of near-infrared spectroscopy monitoring. RESULTS: Overall, 42 of 58 pediatric cardiac ICUs (72%) responded: United Kingdom and Ireland, 11 of 13 (84.6%); Italy, 12 of 12 (100%); and Germany, 19 of 33 (57%, included all major centers). Near-infrared spectroscopy usage varied with 35% (15/42) reporting that near-infrared spectroscopy was not used at all (7/42) or occasionally (8/42); near-infrared spectroscopy use was much less common in the United Kingdom (46%) when compared with 78% in Germany and all (100%) in Italy. Only four units had a near-infrared spectroscopy protocol, and 18 specifically used near-infrared spectroscopy in high-risk patients; 37 respondents believed that near-infrared spectroscopy added value to standard monitoring and 23 believed that it gave an earlier indication of deterioration, but only 19 would respond based on near-infrared spectroscopy data alone. Targets for absolute values and critical thresholds for intervention varied widely between units. The reasons cited for not or occasionally using near-infrared spectroscopy were expense (n = 6), limited evidence and uncertainty on how it guides management (n = 4), difficulty in interpretation, and unreliability of data (n = 3). Amongst the regular or occasional near-infrared spectroscopy users (n = 35), 28 (66%) agreed that a multicenter study is warranted to ascertain its use. CONCLUSIONS: Although most responding units used near-infrared spectroscopy for high-risk patients, the majority (31/35 [88%]) did not have any protocols or guidelines for intervention. Target thresholds and intervention algorithms are needed to support the use of near-infrared spectroscopy in pediatric cardiac ICUs; an international multicenter study is warranted.

Continuous infusion of clonidine in ventilated newborns and infants: a randomized controlled trial.

OBJECTIVES: To assess the influence of an infusion of clonidine 1 µg/kg/hr on fentanyl and midazolam requirement in ventilated newborns and infants. DESIGN: Prospective, double-blind, randomized controlled multicenter trial. Controlled trials.com/ISRCTN77772144. SETTING: Twenty-eight level 3 German PICUs/neonatal ICUs. PATIENTS: Ventilated newborns and infants: stratum I (1-28 d), stratum II, (29-120 d), and stratum III (121 d to 2 yr). INTERVENTIONS: Patients received clonidine 1 µg/kg/hr or placebo on day 4 after intubation. Fentanyl and midazolam were adjusted to achieve a defined level of analgesia and sedation according to Hartwig score. MEASUREMENTS AND MAIN RESULTS: Two hundred nineteen infants were randomized; 212 received study medication, 69.7% were ventilated in the postoperative care and 30.3% for other reasons. Primary endpoint: consumption of fentanyl and midazolam in the 72 hours following the onset of study medication (main observation period) in the overall study population. The confirmatory analysis of the overall population showed no difference in the consumption of fentanyl and midazolam. Explorative age-stratified analysis demonstrated that in stratum I (n = 112) the clonidine group had a significantly lower consumption of fentanyl (clonidine: 2.1 ± 1.8 µg/kg/hr, placebo: 3.2 ± 3.1 µg/kg/hr; p = 0.032) and midazolam (clonidine: 113.0 ± 100.1 µg/kg/hr, placebo: 180.2 ± 204.0 µg/kg/hr; p = 0.030). Strata II (n = 43) and III (n = 46) showed no statistical difference. Sedation and withdrawal-scores were significantly lower in the clonidine group of stratum I (p < 0.001). Frequency of severe adverse events did not differ between groups. CONCLUSIONS: Clonidine 1 µg/kg/hr in ventilated newborns reduced fentanyl and midazolam demand with deeper levels of analgesia and sedation without substantial side effects. This was not demonstrated in older infants, possibly due to lower clonidine serum levels.

Nalbuphine as analgesic in preschool children undergoing ophthalmic surgery and the occurrence of emergence delirium.

BACKGROUND/AIMS: Perioperative pain in children is often inadequately treated, and emergence agitation is common. The purpose of this analysis was to determine whether nalbuphine is suitable for perioperative eye pain and to analyse if it influences the occurrence of emergence delirium/agitation (EDA) in children undergoing ophthalmic surgery in general anaesthesia. METHODS: Retrospective cohort analysis of 50 children in preschool age undergoing general anaesthesia for ophthalmic surgery receiving nalbuphine as a postoperative analgesic in a German university hospital from June 2020 to February 2021.Scores and values for pain and EDA were routinely recorded after awakening and during the stay in the recovery room. Data were evaluated retrospectively from the medical records. RESULTS: A total of 50 children (17 girls and 33 boys) underwent general anaesthesia for ophthalmic surgery. The median age of the children included was 20.5 months (range, 1-68 months), the median body weight was 12.25 kg (range, 2.9-29 kg). All patients received ibuprofen (10 mg/kg1) during induction of anaesthesia and nalbuphine (0.1 mg/kg) at the end of surgery. All patients had an Paediatric-Anaesthesia-Emergence-Delirium-I-score (PAED-ED-I Score) of less than 6 and acceptable Face-Legs-Activity-Cry-Consolability-scores (FLACC less than 3) on waking and on leaving the recovery room. CONCLUSION: Nalbuphine shows a sufficient analgesic effect for pain therapy following ophthalmic surgery in preschool children. Nalbuphine seems to reduce the incidence of EDA in children undergoing ophthalmic surgery.

Use of propofol in pediatric intensive care units: a national survey in Germany.

OBJECTIVE: Propofol is not licensed for sedation in pediatric intensive care medicine mainly due to the risk of propofol infusion syndrome. Nevertheless, it is applied by many pediatric intensive care units. The aim of this national survey was to asses the current use of propofol in pediatric intensive care units in Germany. DESIGN: We performed a nationwide survey. The questionnaire assessed the intensive care unit type, patient numbers, dosing, duration, age and time limits, indications, side effects, and institutional protocols for propofol usage. SETTING: Pediatric intensive care units in Germany. SUBJECTS: Questionnaire about routine use of propofol sent to 214 pediatric departments. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: One hundred ninety-four questionnaires (90.7%) were returned, ten had to be censored. The final analysis comprised 184 questionnaires (134 pediatric/neonatal intensive care units, 28 pediatric intensive care units, 22 neonatal intensive care units). Seventy-nine percent of intensive care units (n = 145 of 184) used propofol in children under the age of 16 yrs. Of these, 98% were for bolus application (n = 142 of 145), 78% for infusion ≥3 hrs (n = 113 of 145), and 33% for infusion >3 hrs (n = 48 of 145). A lower age limit was applied by 52% (n = 75 of 145) and a dose limit by 51% (n = 74 of 145). The median dose limit was 4 mg/kg/hr; 48% (n = 70 of 145) used 3 mg/kg/hr or less. A time limit was applied by 98% (n = 46 of 47), 70% (n = 33 of 47) used it for ≤24 hrs, and 30% (n = 15 of 47) for >24 hrs. MAIN INDICATIONS FOR PROPOFOL APPLICATION WERE: difficult sedation (44%), postoperative ventilation (43%), and difficult extubation (30%). Seven cases of propofol infusion syndrome were reported by seven centers. CONCLUSIONS: This study shows that propofol is used off-license by many pediatric intensive care units in Ge. The majority of users has adopted tightly controlled regimens for propofol sedation, and limits the dose to ≤3-4 mg/kg/hr and the maximum application time to 24-48 hrs.

Remifentanil and propofol for weaning of mechanically ventilated pediatric intensive care patients.

Mechanically ventilated pediatric intensive care patients usually receive an analgesic and sedative to keep them comfortable and safe. However, common drugs like fentanyl and midazolam have a long context sensitive half time, resulting in prolonged sedation and an unpredictable extubation time. Children often awake slowly and struggle against the respirator, although their respiratory drive and their airway reflexes are not yet sufficient for extubation. In this pilot study, we replaced fentanyl and midazolam at the final phase of the weaning process with remifentanil and propofol. Twenty-three children aged 3 months-10 years were enrolled. Remifentanil and propofol revealed throughout excellent or good weaning conditions with rapid transition from hypnosis to the development of regular spontaneous breathing, airway protective reflexes, and an appropriate level of alertness. Extubation time following discontinuation of the remifentanil and propofol infusion was only 24 ± 20 min (5-80 min). We conclude that the combination of remifentanil and propofol is a promising option to improve the weaning conditions of pediatric intensive care patients. Randomized controlled trials are needed to compare remifentanil and propofol with conventional weaning protocols.

Dilated cardiomyopathy in children with ventricular preexcitation: the location of the accessory pathway is predictive of this association.

BACKGROUND: Ventricular preexcitation may be associated with dilated cardiomyopathy, even in the absence of recurrent and incessant tachycardia. METHODS: This report describes the clinical and electrophysiologic characteristics of 10 consecutive children (6 males), with median age of 8 years (range, 1-17 years), who presented with dilated cardiomyopathy and overt ventricular preexcitation on the 12-lead electrocardiogram. Incessant tachycardia as the cause of dilated cardiomyopathy could be excluded. Coronary angiography, right ventricular endomyocardial biopsy (4/10 patients), and metabolic and microbiologic screening were nondiagnostic. RESULTS: The electrocardiograms suggested right-sided pathways in all patients. A right-sided accessory pathway was demonstrated in 8 patients during invasive electrophysiologic study (superoparaseptal, n = 5; septal, n = 2; fasciculoventricular, n = 1). All pathways were successfully ablated (radiofrequency ablation in 7, cryoablation in 1). Two patients had spontaneous loss of ventricular preexcitation during follow-up. Left ventricular (LV) function completely recovered after a loss of preexcitation in all patients. CONCLUSIONS: Right-sided accessory pathways with overt ventricular preexcitation and LV dyssynchrony may cause dilated cardiomyopathy. An association between such pathways and dilated cardiomyopathy is suggested by the rapid normalization of ventricular function and reverse LV remodeling after a loss of ventricular preexcitation.

Dyssynchronous ventricular activation in asymptomatic wolff-Parkinson-white syndrome: a risk factor for development of dilated cardiomyopathy.

A subset of children and adults with Wolff-Parkinson-White (WPW) syndrome develop dilated cardiomyopathy (DCM). Although DCM may occur in symptomatic WPW patients with sustained tachyarrhythmias, emerging evidence suggests that significant left ventricular dysfunction may arise in WPW in the absence of incessant tachyarrhythmias. An invariable electrophysiological feature in this non-tachyarrhythmia type of DCM is the presence of a right-sided septal or paraseptal accessory pathway. It is thought that premature ventricular activation over these accessory pathways induces septal wall motion abnormalities and ventricular dyssynchrony. LV dyssynchrony induces cellular and structural ventricular remodelling, which may have detrimental effects on cardiac performance. This review summarizes recent evidence for development of DCM in asymptomatic patients with WPW, discusses its pathogenesis, clinical presentation, management and treatment. The prognosis of accessory pathway-induced DCM is excellent. LV dysfunction reverses following catheter ablation of the accessory pathway, suggesting an association between DCM and ventricular preexcitation. Accessory pathway-induced DCM should be suspected in all patients presenting with heart failure and overt ventricular preexcitation, in whom no cause for their DCM can be found.

Severe Hyponatraemia Associated with the Use of Arginine-Vasopressin in Two Neonates with Complex Congenital Heart Disease.

We report two cases of neonates with complex congenital heart disease and volume, catecholamine, and corticosteroid refractory shock treated with arginine-vasopressin. Arginine-vasopressin was able to reverse critical hypotension, but both patients developed severe hyponatremia, which recovered after discontinuation of arginine-vasopressin. Close control and prompt substitution of serum sodium is required in neonates with advanced heart failure on high-dose vasopressin therapy.

Stenting the Fontan pathway in paediatric patients with obstructed extracardiac conduits.

OBJECTIVES: An unobstructed extracardiac conduit (ECC) is essential for optimal Fontan haemodynamics. We aimed to evaluate the feasibility and results of percutaneous transcatheter stenting of the ECC in paediatric patients with a significant Fontan pathway obstruction. METHODS: Our institutional database was searched to identify all Fontan patients who had a stent placed in their ECC. Medical records, cardiac catheterisation data and echocardiographic investigations were reviewed. Vessel diameters were normalised to account for differences in body surface area. RESULTS: Nineteen Fontan patients (age 6.5±3.2 years; male 78.9%) with a significant stenosis of their Dacron ECC graft were identified. Seven patients presented with protein-losing enteropathy (36.8%). An ECC obstruction was suspected on echocardiography in only 6/19 patients (31.6%). The mean minimum diameter of the ECC was 8.3±2.4 mm. A stenosis of >45% was seen in the majority of patients (n=12, 63.1%). Significant correlations between the severity of the ECC obstruction and Fontan pathway vessel diameters were found (all p<0.05). Stenting was successful in all children. The ECC diameter increased significantly after stenting (p<0.0001). An acute clinical benefit of ECC stenting was observed in 18/19 (94.7%) patients. ECC patency was good during a mean follow-up of 1.8±0.9 years. CONCLUSIONS: The feasibility and acute results of percutaneous transcatheter ECC stenting are promising and may provide a good alternative to postpone surgery to a later age. The mechanisms contributing to the development of ECC stenoses are likely multifactorial.