Search

Myxedema heart disease is an exceptionally rare condition with few reported cases. We present a rare case of a child who presented with severe congestive heart failure secondary to myxedema heart disease complicated by preexisting valvular heart disease. The patient was admitted to the pediatric intensive care unit and medical management. An echocardiogram showed severe biventricular dysfunction and severe valvular disease. She was started on levothyroxine and liothyronine. During the patient's hospitalization, the thyroxine level normalized but the thyroid-stimulating hormone continued to remain elevated. Likewise, her cardiac ventricular function improved, though it did not return to baseline. Our case highlights the importance of maintaining a broad differential diagnosis in a child who presents with congestive heart failure, to include thyroid disease, which necessitates a rare but specific management strategy.

Background: Among the known risk factors for children undergoing surgery for congenital heart defect (CHD), temperature and blood flow during cardiopulmonary bypass (CPB), are two risk factors, which may be altered to improve outcomes. Moderate - to - deep hypothermia (MDH), traditionally used, has been associated with short/long-term neurologic sequelae, so there is a move towards mild hypothermia (MH) with selective antegrade cerebral perfusion (SACP). Aims and Objectives: To assess feasibility of mild hypothermia with selective antegrade cerebral perfusion during cardiopulmonary bypass as a cardiopulmonary bypass strategy in neonates and infants undergoing surgery for congenital heart defect. Materials and Methods: We conducted a retrospective chart review of neonates and infants who underwent SACP on CPB during CHD surgery at our tertiary care children's hospital between January 2014 and February 2017. We categorized all the patients into 2 groups - MH + SACP or MDH + SACP. We gathered data on preoperative, intra-operative and post-operative parameters. We analyzed comparative data using student t-test and chi-squared test with a significant p-value < 0.05. Results: There were 6 in the MH + SACP group and 7 in the MDH + SACP group, who underwent SACP on CPB during study period at our children's hospital. All the neonates and infants in the MH + SACP group survived at the hospital discharge with favorable neurologic outcomes. There was no statistically significant difference between the two groups for end organ dysfunction, ventilator days, hospital days, ICU and hospital mortality and PCPC/POPC at hospital discharge. Conclusions: Mild hypothermia with selective antegrade cerebral perfusion during cardiopulmonary bypass is a feasible cardiopulmonary bypass strategy in neonates and infants undergoing surgery for congenital heart defect.

Background Purulent pericarditis is rare in the pediatric population. Three children with purulent pericarditis complicated by tamponade were seen at a children’s hospital from 2018-2019. A review of the literature was conducted to investigate the clinical significance and features of purulent pericarditis. Methods Cases of purulent pericarditis in children (age < 18 years) published in English from 2000 to 2020 were reviewed. Patients were included if there was presence of purulent pericardial fluid or if a bacterial pathogen was isolated from pericardial fluid. Results Three children with purulent pericarditis and tamponade with associated pneumonia were cared for at our institution. These infections were caused by methicillin-susceptible Staphylococcus aureus, Haemophilus influenzae, and Streptococcus pyogenes. Review of the literature identified 93 children with purulent pericarditis. The median age of the cohort is 4 years old. In 68.8% of children the etiology was identified from culture of pericardial fluid. The most common organism detected was S. aureus (38.7%) and a concurrent infection was seen in 49.4% -pneumonia (36.5%), osteomyelitis (17.2%), soft tissue (7.5%), and meningitis (2.1%). In North America specifically, methicillin-resistant S. aureus was most common 35% (7/20) and associated infection was seen in 80% (16/20). Clinical course was complicated by pericardial tamponade in 68.5% (37/54) of patients, 48.6% (18/37) of those children with tamponade also had pneumonia. Pericardiocentesis is the most frequent initial intervention, performed in 77.4% of cases. The mortality rate was 4.3%. Conclusion In pediatric purulent pericarditis, Gram-positive organisms account for 81.2% of all children with positive pericardial fluid culture, and 75% of infections in North America. A bacterial pathogen can be isolated from the pericardial fluid in a majority of patients with purulent pericarditis. There is a high rate of concurrent infection, most notably pneumonia, and there is a strikingly high percentage of tamponade in those cases. Disclosures All Authors: No reported disclosures

Idiopathic hyperammonemia is a rare complication with a high mortality rate that occurs in persons with hematologic malignancies or hematopoietic stem cell or solid organ transplant. Patients present with encephalopathy and hyperammonemia in the absence of liver disease or inborn errors of metabolism. Several etiologies have been proposed, including chemotherapeutic agents, medications, and a catabolic state with an elevated nitrogen load in the setting of acute illness. Recently, cases of hyperammonemia in adult lung transplant recipients have been attributed to infection from Ureaplasma parvum or U urealyticum Herein, we report a 12-year-old girl with acute myeloid leukemia and neutropenic fever who developed acute encephalopathy. Laboratory testing revealed severe hyperammonemia (blood ammonia level >1609 μmol/L) with normal liver function studies. U parvum was detected in blood, urine, and respiratory specimens by polymerase chain reaction testing. After antibiotic therapy directed against U parvum, blood ammonia levels normalized, the infection was eradicated, and the patient recovered. We propose that clinicians should test for invasive infection from Ureaplasma species in immunocompromised children with unexplained hyperammonemia.

Many known risk factors for adverse cardiovascular and neurological outcomes in children with congenital heart defects (CHD) are not modifiable, however, the temperature and blood flow during cardiopulmonary bypass (CPB), are two risk factors, which may be altered in an attempt to improve long-term neurological outcomes. Deep hypothermic circulatory arrest (DHCA), traditionally used for aortic arch repair, has been associated with short-term and long-term neurologic sequelae. Therefore, there is a rising interest in using moderate hypothermia with selective antegrade cerebral blood flow on CPB during aortic arch repair. Rewarming from moderate-to-deep hypothermia has been shown to be associated with neuronal injury, neuroinflammation and loss of cerebrovascular autoregulation. A significantly lesser degree of rewarming is required following mild (33-35°C) hypothermia as compared to moderate (28-32°C), deep (21–27°C) and profound (less than 20°C) hypothermia. Therefore, we believe that mild hypothermia is associated with a lower risk of rewarming-induced neurologic injury. We hypothesize that mild hypothermia with selective antegrade cerebral perfusion during CPB for neonatal aortic arch repair would be associated with improved neurologic outcome.

Procalcitonin appears to be an early and sensitive marker of bacterial infection in a variety of clinical settings. The use of levels of procalcitonin to predict infection in children undergoing cardiac surgery, however, may be complicated by the systemic inflammatory response that normally accompanies cardiopulmonary bypass. The aim of our study was to estimate peri-operative concentrations of procalcitonin in non-infected children undergoing cardiac surgery. Samples of serum for assay of procalcitonin were obtained in 53 patients at baseline, 24, 48, and 72 hours following cardiac surgery. Concentrations were assessed using an immunoluminetric technique. Median concentrations were lowest at baseline at less than 0.5 nanograms per millilitre, increased at 24 hours to 1.8 nanograms per millilitre, maximized at 48 hours at 2.1 nanograms per millilitre, and decreased at 72 hours to 1.3 nanograms per millilitre, but did not return to baseline levels. Ratios of concentrations between 24, 48 and 72 hours after surgery as compared to baseline were 6.15, with 95 percent confidence intervals between 4.60 and 8.23, 6.49, with 95 percent confidence intervals from 4.55 to 9.27, and 4.26, with 95 percent confidence intervals between 2.78 and 6.51, respectively, with a p value less than 0.001. In 8 patients, who had no evidence of infection, concentrations during the period from 24 to 72 hours were well above the median for the group. We conclude that concentrations of procalcitonin in the serum increase significantly in children following cardiac surgery, with a peak at 48 hours, and do not return to baseline within 72 hours of surgery. A proportion of patients, in the absence of infection, had exaggerated elevations post-operatively.

UNLABELLED The glial-derived protein S100B is a serum marker of cerebral ischemia and correlates with negative neurological outcome after cardiopulmonary bypass (CPB) in adults. We sought to characterize the S100B release pattern before and after CPB in neonates and infants with congenital heart disease and correlate it with surgical mortality. Serum was collected before surgery and at 24 postoperative h from 109 neonates and infants with congenital heart disease. All patients had presurgical transthoracic echocardiograms and CPB with or without hypothermic circulatory arrest. S100B concentrations were determined using a two-site immunoluminometric assay (Sangtec 100). Thirty-day surgical mortality was observed. All neonates had significantly increased S100B concentrations before surgery that decreased by 24 postoperative h. Preoperative S100B concentrations in 32 neonates with hypoplastic left heart syndrome correlated inversely with the forward flow and size of the ascending aorta and postoperative mortality (r(2) = -0.63; P = 0.03). Among infants, increased pulmonary blood flow was associated with higher S100B levels before surgery than cyanosis. There was no correlation with postoperative S100B and time on CPB, hypothermic circulatory arrest, or 30-day surgical mortality. In conclusion, preoperative S100B concentrations correlate inversely with the size of the ascending aorta in hypoplastic left heart syndrome and may serve as a marker for preexisting brain injury and mortality. IMPLICATIONS Neonates with hypoplastic left heart syndrome and no forward flow in the ascending aorta may have brain injury at birth before heart surgery.

Patients undergoing cardiac surgery experience an ab normal whole-body inflammatory response after the initiation of cardiopulmonary bypass (CPB). The inflam matory response to CPB may cause postoperative or gan dysfunction and morbidity, especially in the pedi atric population. The systemic inflammatory response may be initiated during CPB by blood contact with the foreign surface of the pump circuit, ischemia-reperfu sion injury, and endotoxemia. Although significant mortality is rare, most patients undergoing CPB expe rience some degree of organ dysfunction as a result of the inflammatory response.

Glycogen storage disorder type 1A (GSD 1A) is an inherited disorder of glycogen metabolism characterized by fasting hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia. These children have a higher risk of developing pancreatitis because of hypertriglyceridemia. Drug-induced pancreatitis accounts for a small proportion of cases of pancreatitis. The mechanism of drug-induced pancreatitis include hypersensitivity, direct toxic injury or indirectly by inducing hypertriglyceridemia. Propofol is often the drug of choice for induction of anesthesia in ambulatory surgical procedures. There are various reports in the literature describing pancreatitis induced by propofol. We present a 4-year-old girl with GSD 1A, who required tonsillectomy and adenoidectomy under general anesthesia. She developed acute pancreatitis in the postoperative period. Propofol was used as a general anesthetic and the postoperative incidence of pancreatitis is discussed.

Objective: The primary study objective was to examine the impact of diagnosis on the inflammatory response in neonates with congenital heart disease undergoing cardiac surgery. The secondary objective was to study the impact of the inflammatory response on postoperative outcome in these neonates. Design: Observational study. Setting: Tertiary care children’s hospital heart center. Patients: Neonates with hypoplastic left-heart syndrome (HLHS) undergoing stage I repair and patients with transposition of the great arteries (TGA) undergoing arterial switch operation. Measurements and Main Results: There were 24 neonates with HLHS and 21 neonates with TGA. Serum samples to measure interleukin (IL)-6 and -10 were obtained before and after CPB at 1, 3, 6, and 24 hours postoperatively. Time to extubation, intensive care unit (ICU) length of stay, and peritoneal fluid drainage were compared between the groups. Serum IL-6 and IL-10 concentrations increased after CPB when compared to the preoperative concentration. Preoperative concentrations of IL-6 were significantly elevated in the HLHS group (HLHS: 32 [21.1, 69.6] pg/mL v TGA: 7.2 [3.6, 22.5] pg/mL [median, 25th, and 75th percentile], p = 0.003) and remained elevated immediately after CPB, and at 3 and 6 hours postoperatively. The IL-10 to IL-6 ratio was lower in the HLHS group preoperatively and immediately after CPB. ICU length of stay was significantly longer in the HLHS group (TGA 4 [3-6] days v HLHS 6 [5-8] days, p = 0.031). Mortality in the HLHS group (4/24) was associated with significantly higher IL-6 postoperatively (IL-6 immediately postoperatively: HLHS survivors 59.9 [34.3, 65.7] pg/mL v nonsurvivors 98.7 [94.4, 104.5] pg/mL, p Conclusions: All neonates with TGA or HLHS have a significant inflammatory response after CPB. Neonates with HLHS have evidence of an activated inflammatory response before CPB, which remains significant in the postoperative period. Accelerated interleukin expression and an abnormal cytokine balance correlate with longer time to extubation, longer ICU length of stay, and increased peritoneal fluid volume.

Background . Cardiopulmonary bypass (CPB) in neonates induces a cytokine-mediated capillary leak syndrome that can cause organ dysfunction. Removing harmful cytokines after CPB may attenuate this response. This study measured the concentrations of serum and peritoneal fluid (PF) cytokines after CPB to determine if harmful cytokines can be removed with peritoneal catheters. Methods . Neonates (n = 18) had cardiac surgery using CPB with circulatory arrest. Peritoneal catheters were placed at the end of surgery to drain excess fluid. Serum samples were obtained before and after CPB, and PF after CPB. Cytokines were measured by enzyme-linked immunosorbent assay. Results . Tumor necrosis factor-α and interleukin-1β (IL-1β) were not detected in any serum or PF sample. Serum concentrations of IL-6, IL-8, and IL-10 increased significantly after CPB. PF concentrations of IL-6 and IL-8 exceeded serum concentrations, whereas IL-10 concentrations were higher in the serum. There was a significant negative correlation between serum and PF concentrations of IL-6 after CPB ( r = −0.63; p Conclusions . PF has very high concentrations of the proinflammatory cytokines, IL-6 and IL-8, after CPB but not the antiinflammatory cytokine IL-10. The PF may be a depot for the harmful inflammatory cytokines after CPB, and removing the PF could lower serum concentrations.

Objective To define the indications for tracheotomy in patients requiring prolonged intubation (>1 week) in the pediatric intensive care unit (PICU). Design Retrospective chart review and follow-up telephone survey. Setting A tertiary care center PICU. Outcome Measure Tracheotomy or extubation. Patients All patients older than 30 days in the PICU intubated for longer than 1 week between 1997 and 1999. Results During the study, 63 total admissions required intubation for longer than 1 week. A tracheotomy was necessary in 14% of admissions (n = 9). The mean length of intubation before the tracheotomy was 424 hours, whereas the mean length of intubation without the need for tracheotomy was 386 hours. Length of intubation, age, and number of intubations did not increase the probability of having a tracheotomy. Of those requiring a tracheotomy, 2 had tracheomalacia, 1 had subglottic edema, 1 had plastic bronchitis, 1 had Down syndrome with apnea resulting in right heart failure, 3 required long-term ventilation after cardiopulmonary collapse, and 1 had mitochondrial cytopathy. Of these 9 children, 7 were successfully decannulated, 1 patient died of underlying disease, and 1 patient remained cannulated secondary to the mitochondrial cytopathy. Twenty families of the patients who did not undergo a tracheotomy were reached by telephone after discharge. Most of the families reported that their children were free of stridor and hoarseness after extubation. Conclusions Children tolerate prolonged intubation without laryngeal complications. The consideration for tracheotomy in the PICU setting must be highly individualized for each child.