Urinary Chloride Excretion Postcardiopulmonary Bypass in Pediatric Patients-A Pilot Study.

The aim of this study was to describe renal chloride metabolism following cardiopulmonary bypass (CPB) surgery in pediatric patients. A prospective observational trial in a tertiary pediatric intensive care unit (PICU) with 20 recruited patients younger than 2 years following CPB surgery was conducted. Urinary electrolytes, plasma urea, electrolytes, creatinine, and arterial blood gases were collected preoperatively, on admission to PICU and at standardized intervals thereafter. The urinary and plasma strong ion differences (SID) were calculated from these results at each time point. Fluid input and output and electrolyte and drug administration were also recorded. Median chloride administration was 67.7 mmol/kg over the first 24 hours. Urinary chloride (mmol/L; median interquartile range [IQR]) was 30 (19, 52) prior to surgery, 15 (15, 65) on admission, and remained below baseline until 24 hours. Plasma chloride (mmol/L; median [IQR]) was 105 (98, 107) prior to surgery and 101 (101, 106) on admission to PICU. It then increased from baseline, but remained within normal limits, for the remainder of the study. The urinary SID increased from 49.8 (19.1, 87.2) preoperatively to a maximum of 122.7 (92.5, 151.8) at 6 hours, and remained elevated until 48 hours. Plasma and urinary chloride concentrations were not associated with the development of acute kidney injury. Urinary chloride excretion is impaired after CPB. The urinary SID increase associated with the decrease in chloride excretion suggests impaired production and/or excretion of ammonium by the nephron following CPB, with gradual recovery postoperatively.

Outcomes After Stage I Norwood Palliation in a Recently Established Program Can Achieve Results Similar to Longer Established Services.

BACKGROUND: Infants with hypoplastic left heart syndrome (HLHS) or similar single ventricle cardiac lesions require a three-stage surgical approach, the first step being the Stage I Norwood procedure. The Queensland Children's Hospital (QCH) in Australia is a tertiary hospital providing the only cardiac surgical service to children in Queensland and northern New South Wales. OBJECTIVE: To review the centre's outcomes of Norwood procedures performed in the last 6 years. MATERIALS AND METHODS: We retrospectively evaluated all infants undergoing the stage I Norwood procedure between January 2015 and August 2021. Mortality, intensive care length of stay, events of cardiac arrest following surgery and duration of mechanical ventilation were calculated and analysed for subgroups depending on type of pulmonary shunt type (right-ventricle-to-pulmonary-artery shunt [RVPAS] vs the modified Blalock-Taussig shunt [MBTS]). RESULTS: Forty-nine (49) patients were included. Overall survival to stage two operation (Glenn) was 90%. Both shunts were used evenly with the RVPA conduit preferred for HLHS and the MBTS largely chosen for hypoplastic left heart variants. In univariable analysis there was no difference in cardiac arrest or mortality rate for the patient with a RVPAS compared to the patient with a MBTS. CONCLUSION: We show that a recently established Norwood program can achieve results that are comparable to those reported by longer established centres, and the international literature.

Universal implantation of temporary epicardial pacing wires after surgery for congenital heart disease: necessity or luxury?

OBJECTIVES: Routine implantation of temporary epicardial pacing wires after surgery for congenital heart disease (CHD) has recently been questioned. We evaluated the incidence of arrhythmias, arrhythmias causing haemodynamic compromise and the safety of a strategy of selective implantation of pacing wires in our unit. METHODS: All patients who underwent surgery for CHD using cardiopulmonary bypass between September 2015 and December 2016 were retrospectively enrolled in the study (n = 313). Patients were stratified into group A (universal implantation) and group B (selective implantation). Group B received pacing wires only when postoperative rhythm disturbances were anticipated based on the operating surgeon's judgement. The primary outcome was arrhythmia causing haemodynamic compromise. Outcomes were compared between unmatched and propensity matched groups. RESULTS: Forty-eight patients experienced an arrhythmia causing haemodynamic compromise (15.3%). Twenty-three patients (7.3%) experienced an arrhythmia causing haemodynamic compromise that required the use of pacing wires for therapeutic purposes (group A n = 13, group B n = 10, P = 0.34). There were no pacing wire related complications in either group. All patients in group A and 90% in group B had pacing wires when needed (P = 0.435). In group A, 89% of patients had pacing wires which were not used compared with 13% in group B (P < 0.001). Results were unchanged when repeated using propensity matching (81 pairs). CONCLUSIONS: The probability of developing a postoperative arrhythmia requiring therapeutic pacing can be predicted using the risk factors identified in our study. The routine implantation of pacing wires after surgery for CHD is not necessary. A measured reduction from universal implantation is safe.

Repair of congenital diaphragmatic hernia during extracorporeal life support: experience with six neonates.

BACKGROUND: The management of congenital diaphragmatic hernia (CDH) in neonates has evolved considerably over the last three decades. Initial stabilization followed by surgical repair is the current standard of care. A subset fails to achieve adequate oxygenation with medical management, including the use of high frequency oscillation and inhaled nitric oxide. The mortality in this group exceeds 80% without additional management strategies. Extracorporeal life support (ECLS) is a well-established modality for managing these neonates with CDH and has been shown to improve early survival in selected cases. METHODS: This is a retrospective analysis of six neonates with CDH who underwent repair during ECLS between September 2011 and November 2014. RESULTS: Of 24 admissions with CDH, there were six neonates (25%) who required ECLS. All the six had CDH repair during ECLS. There were no intra-operative bleeding complications. There were no clotting complications related to stopping heparin during CDH repair. There was one hospital death. Five neonates were weaned from ECLS and discharged home. CONCLUSIONS: Data from our small cohort of patients illustrate that early survival is possible in extremely compromised neonates who otherwise would have died without ECLS. Our experience demonstrates that CDH repair can safely be performed during ECLS. Use of ECLS, early repair during ECLS, lung protective ventilation strategies and aggressive management of pulmonary hypertension were associated with good early survival. ECLS should be considered as an integral part of therapeutic armamentarium for CDH in neonates.

Resuscitation of a neonate with medium chain acyl-coenzyme a dehydrogenase deficiency using extracorporeal life support.

We report a neonate with medium chain acyl-coenzyme A dehydrogenase deficiency (MCAD) who had a cardiac arrest due to ventricular tachycardia and fibrillation. Extracorporeal life support (ECLS) was deployed, from which the baby was subsequently separated and discharged from hospital. This case was a rare neonatal presentation of MCAD and an uncommon indication for ECLS. We discuss the presentations of patients with MCAD and the use of ECLS for patients with possible inborn errors of metabolism and other unknown primary diagnoses.

Embryonal rhabdomyosarcoma with metastases confined to the lungs: report from the CWS Study Group.

BACKGROUND: Embryonal rhabdomyosarcoma [RME] is the most common pediatric soft tissue sarcoma. Whereas the prognosis of localized rhabdomyosarcoma has improved, it remains poor for metastatic disease. METHODS: We analyzed RME-patients with isolated pulmonary metastases [PRME] treated in four consecutive CWS-trials. Treatment included multiagent chemotherapy and local treatment of the primary tumor. Therapy of lung metastases after induction chemotherapy depended on response and individual decisions. RESULTS: Twenty-nine patients <21 years had PRME. Their median age was six years, the median follow-up nine years. Twenty-eight children had their primary tumor located in an unfavorable site and 22 of the primaries were >5 cm. In addition to conventional chemotherapy, seven patients received high-dose treatment and eight patients oral metronomic chemotherapy. The lung metastases were in remission after induction chemotherapy in 22 individuals. 19 patients received no local treatment of metastases; 3 patients had pulmonary metastasectomy and lung radiation was administered to 9 individuals. In total, 24/29 patients achieved a complete remission [CR]. Actuarial 5-year event-free and overall survival for all patients was 37.9 ± 18% and 48.7 ± 18%, respectively; it was 45.8 ± 20% and 58.3 ± 20% for the 24 patients who achieved a CR. Local treatment of metastases had no impact on the failure pattern. Younger age, good response, achievement of CR and maintenance-treatment were favorable prognostic factors in univariate analysis. CONCLUSIONS: Children with PRME have a fair prognosis. Local treatment of metastases did not improve outcome in our sample. Metronomic treatment may be an attractive option for PREM-patients.

Does the time-point of relapse influence outcome in pediatric rhabdomyosarcomas?

BACKGROUND: Childhood rhabdomyosarcoma (RMS), a soft tissue malignant tumor of skeletal muscle origin, accounts for approximately 3.5% of the cases of cancer among children 0-14 years and 2% of the cases among adolescents and young adults 15-19 years of age. PROCEDURE: We evaluated survival (SUR) after first relapse depending on the time to relapse (TTR) in RMSs of childhood and adolescence. Early, intermediate, and late relapsing patients were evaluated for prognostic risk factors. RESULTS: Two hundred thirty-four patients with RMS enrolled in the German sarcoma trial CWS-81, CWS-86, CWS-91, and CWS-96 met selection criteria. Of the 234 patients, 35%, 32%, and 33% relapsed within 6 (early), 6-12 (intermediate), and more than 12 (late) months respectively after the end of primary therapy. Four-year SUR was 12%, 21%, and 41% for early, intermediate, and late relapse respectively (P < 0.001). Four-year SUR after local relapse was 18% (early), 38% (intermediate), and 49% (late). Embryonal RMS showed four year SUR of 16%, 30%, and 46% (P < 0.001) whereas alveolar histology showed four year SUR of 8%, 6%, and 23% (P < 0.01) for early, intermediate, and late relapse respectively. CONCLUSION: TTR has significant influence on prognosis in relapsed RMS. It influences SUR independent of other features such as type of relapse, histology, tumor site, primary treatment time or irradiation in primary treatment.

Grossly-resected synovial sarcoma treated by the German and Italian Pediatric Soft Tissue Sarcoma Cooperative Groups: discussion on the role of adjuvant therapies.

Differently from adult oncologists that considered synovial sarcoma (SS) a tumor with uncertain chemosensitiveness, since two decades pediatric oncologists in Europe assumed that chemotherapy played an important role in SS treatment, so most pediatric patients were included in rhabdomyosarcoma protocols, receiving adjuvant chemotherapy regardless of risk factors. The German and Italian groups reviewed the data of grossly resected SS patients in order to define a risk-adapted treatment program for the next European protocol. A total of 150 patients < 21 years with localized SS who underwent initial gross resection between 1975 and 2002 were the object of this study. All but four cases received adjuvant chemotherapy. Post-operative radiotherapy was administered to 50% Group I and to 92% Group II patients. Five-year event-free survival (EFS) and overall survival (OS) were 77% and 89%, respectively. Survival rates were influenced by tumor size (EFS 92% and 56% for size < or = and > 5 cm, respectively) and local invasiveness, not by surgical margins. No metastatic relapses occurred in Group I < or = 5 cm patients, while the outcome was poor for T2B patients (EFS 41%) due to a high rate of metastatic relapse. Our study was unable to assess the role of adjuvant treatments in grossly-resected SS, but identified a subset of low-risk patients (IRS Group I, size < or = 5 cm), for which the omission of adjuvant chemotherapy could be suggested, and a subset of high-risk patients (T2B), who need treatment intensification.

Group II rhabdomyosarcoma and rhabdomyosarcomalike tumors: is radiotherapy necessary?

PURPOSE: In the prospective Cooperative Soft Tissue Sarcoma Study Group (CWS) 81, 86, 91, and 96 trials, radiotherapy was omitted in some patients with rhabdomyosarcoma and rhabdomyosarcoma-like tumors within Intergroup Rhabdomyosarcoma Study (IRS) group II. This analysis evaluates whether subgroups can be defined for which radiotherapy is not necessary. PATIENTS AND METHODS: Two hundred three patients who were registered between January 1981 and December 1998 were eligible for evaluation. Radiotherapy was given depending on tumor location, histology, and whether a secondary complete resection could be performed. The recommended radiation doses ranged from 32 to 54 Gy. RESULTS: One hundred ten patients did receive and 93 patients did not receive radiotherapy. The calculated local control after 5 years was 83% with and 65% without radiotherapy (P <.004). Event-free survival (EFS) at 5 years was 76% and 58%, respectively (P <.005). Overall survival (OS) at 5 years was 84% and 77% (P = not significant). The differences in local control were significant for the subgroups of irradiated patients with favorable histology, favorable site, and initial tumor size of less than 5 cm. A trend for improved local control with irradiation was observed for patients with unfavorable site, unfavorable histology, and large primary tumors. EFS was significantly improved for irradiated patients who had unfavorable histology, both favorable and unfavorable tumor sites, and small initial tumors. OS was significantly improved for patients with unfavorable histology through radiation. CONCLUSION: Local control and EFS in group II patients are improved with radiotherapy. No subgroup could be defined for which the omission of radiotherapy produced outcome equivalent to that of patients who were irradiated.