Neonatal Myxomas: Case Report and Literature Review.

Myxomas are rare tumors in neonates and tend to have a different presentation compared to adults. We present an infant with a left atrial myxoma presenting with episodic tachycardia who underwent successful surgical excision. In addition, we performed a review of the literature, identifying 17 cases of neonatal myxomas. Unlike adults, neonatal myxomas are more common in males and occur more often on the right side of the heart. Constitutional symptoms such as fever or embolism are rare among neonates. Most patients have favorable outcomes following surgical excision, refuting earlier claims that neonatal myxomas are associated with poor outcomes.

Elevated Levels of Urinary Biomarkers TIMP-2 and IGFBP-7 Predict Acute Kidney Injury in Neonates after Congenital Heart Surgery.

Objectives This article investigated the utility of urine biomarkers tissue inhibitor of metalloproteinase-2 (TIMP-2) and insulin-like growth factor binding protein-7 (IGFBP-7) in identifying acute kidney injury (AKI) in neonates after congenital heart surgery (CHS). TIMP-2 and IGFBP-7 are cell cycle arrest proteins detected in urine during periods of kidney stress/injury. Methods We conducted a single-center, prospective study between September 2017 and May 2019 with neonates undergoing CHS requiring cardiopulmonary bypass (CPB). Urine samples were analyzed using NephroCheck prior to surgery and 6, 12, 24, and 96 hours post-CPB. All patients were evaluated using the Acute Kidney Injury Network (AKIN) criteria. Wilcoxon rank sum tests were used to compare the medians of the [TIMP-2*IGFBP-7] values in the AKIN negative and positive groups at each time point. Receiver operating characteristic curves were used to measure how well the [TIMP-2*IGFBP-7] values predict AKIN status. Results Thirty-six patients were included. No patients met the AKIN criteria for AKI preoperatively. Postoperatively, 19 patients (53%) met the AKIN criteria for AKI diagnosis: 13 (36%) stage 1, 5 (14%) stage 2, and 1 (3%) stage 3. None required renal replacement therapy. At the 24-hour time points, patients who met the AKIN criteria for AKI had a statistically significantly higher [TIMP-2*IGFBP7] values than the patients without AKI (1.1 vs. 0.27 [ng/mL] 2 /1,000) at 24 hours (adj- p = 0.0019). Conclusion AKI is a serious complication associated with adverse outcomes in patients undergoing cardiac surgery. [TIMP-2*IGFBP-7] urinary level 24 hours after CPB is a good predictor of AKI in this population.

Ubiquitin C-Terminal Hydrolase 1 and Phosphorylated Axonal Neurofilament Heavy Chain in Infants Undergoing Cardiac Surgery: Preliminary Assessment as Potential Biomarkers of Brain Injury.

BACKGROUND: There are no reliable markers to assess brain injury in neonates following cardiac surgery. We examine ubiquitin C-terminal hydrolase 1 (UCHL1) and phosphorylated axonal neurofilament heavy chain (pNF-H), neuronal-specific biomarkers released following axonal and cortical injury, in neonates undergoing cardiac surgery involving cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). METHODS: Twenty-six patients younger than three months were prospectively enrolled (CPB only, n = 12 and DHCA, n = 14). Healthy newborns (n = 22) served as the control. Blood samples were collected preoperatively and postoperatively upon intensive care unit admission (hour 0) and subsequently at 12, 24, 36, and 48 hours. Serum was tested for UCHL1 and pNF-H using enzyme-linked immunosorbent assay. Concomitant arterial blood gas, lactate, and cerebral near-infrared spectroscopy (NIRS) monitoring were performed. RESULTS: Ubiquitin C-terminal hydrolase 1 showed a significant rise at 0 hours in the DHCA group compared to baseline (74.9 ± 13.7 pg/mL vs 33.9 ± 37.3 pg/mL, P < .0001). Levels returned to baseline at 12 hours. There was an early rise in UCHL1 at 0 hours in the CPB group, P = .09. Phosphorylated axonal neurofilament heavy chain was decreased at 0 hours in both the CPB and DHCA groups compared to baseline, P = .06. There was no difference between control and baseline levels of UCHL1 ( P = .9) or pNF-H ( P = .77). Decreased NIRS was observed in the DHCA group at 0 hours (57.3 ± 10.5) versus baseline (64.2 ± 12.3), but not significant ( P = .21). There was no correlation between biomarkers and NIRS at 0 hours. CONCLUSION: A rapid rise in UCHL1 levels was observed in the DHCA group, suggesting that it may be a marker for acute brain injury. Follow-up with neurodevelopmental studies is ongoing.

Use of Tolvaptan in a Patient With Palliated Congenital Heart Disease.

Heart failure is a common problem in the ever growing population of patients with palliated congenital heart disease. It is frequently complicated by hyponatremia that has been associated with increased morbidity and mortality. Tolvaptan is a vasopressin receptor antagonist that has been effective in improving hyponatremia and congestive symptoms in adults with chronic heart failure. We describe the short-term use of tolvaptan to treat hyponatremic hypervolemia in an adolescent patient with chronic heart failure in the setting of palliated congenital heart disease prior to definitive surgical intervention. In this case, the patient had improvement in hyponatremia and a decrease in body weight, without any adverse effects.

Procalcitonin as a biomarker of bacterial infection in pediatric patients after congenital heart surgery.

BACKGROUND: Bacterial infection (BI) after congenital heart surgery (CHS) is associated with increased morbidity and is difficult to differentiate from systemic inflammatory response syndrome caused by cardiopulmonary bypass (CPB). Procalcitonin (PCT) has emerged as a reliable biomarker of BI in various populations. AIM: To determine the optimal PCT threshold to identify BI among children suspected of having infection following CPB. SETTING AND DESIGN: Single-center retrospective observational study. MATERIALS AND METHODS: Medical records of all the patients admitted between January 2013 and April 2015 were reviewed. Patients in the age range of 0-21 years of age who underwent CHS requiring CPB in whom PCT was drawn between postoperative days 0-8 due to suspicion of infection were included. STATISTICAL ANALYSIS: The Wilcoxon rank-sum test was used for nonparametric variables. The diagnostic performance of PCT was evaluated using a receiver operating characteristic (ROC) curve. RESULTS: Ninety-eight patients were included. The median age was 2 months (25th and 75th interquartile of 0.1-7.5 months). Eleven patients were included in the BI group. The median PCT for the BI group (3.42 ng/mL, 25th and 75th interquartile of 2.34-5.67) was significantly higher than the median PCT for the noninfected group (0.8 ng/mL, 25th and 75th interquartile 0.38-3.39), P = 0.028. The PCT level that yielded the best compromise between the sensitivity (81.8%) and specificity (66.7%) was 2 ng/mL with an area under the ROC curve of 0.742. CONCLUSION: A PCT less than 2 ng/mL makes BI unlikely in children suspected of infection after CHS.

Use of the Aquadex™ system for ultrafiltration therapy in a hemodynamically unstable pediatric patient.

In this case report, we describe the use of the Aquadex™ system for ultrafiltration therapy in the pediatric cardiac intensive care setting in a patient with fluid overload and acute kidney injury after congenital heart surgery. The patient is an 11-year-old, 25 kg male with complex single ventricle anatomy who underwent a one and a half ventricle repair. The patient experienced multiple organ dysfunction syndrome including acute kidney injury in the early post-operative period secondary to low cardiac output syndrome and tachyarrhythmia. Ultrafiltration using the Aquadex™ system was utilized to treat fluid overload in the setting of acute kidney injury and hemodynamic instability. Negative fluid balance was safely achieved. It was subsequently possible to wean ventilatory and inotropic support. We conclude that the use of ultrafiltration therapy is feasible in hemodynamically unstable pediatric patients with significant fluid overload in the setting of acute kidney injury following congenital heart surgery.

Factors affecting the decision to defer endotracheal extubation after surgery for congenital heart disease: a prospective observational study.

BACKGROUND: Fast-tracking and early endotracheal extubation have been described in patients undergoing surgery for congenital heart disease (CHD); however, criteria for patient selection have not been validated in a prospective manner. Our goal in this study was to prospectively identify factors associated with the decision to defer endotracheal extubation in the operating room (OR). METHODS: We performed a prospective observational study of 275 patients (median age 18 months) at the Mount Sinai Medical Center (MSMC), New York, New York, and 49 patients (median age 25 months) at the University of Tokyo Hospital (UTH), Tokyo, Japan, undergoing surgery for CHD requiring cardiopulmonary bypass. These patients were all eligible for fast-tracking, including extubation in the OR immediately after surgery, according to the respective inclusion/exclusion criteria applied at the 2 sites. RESULTS: Eighty-nine percent of patients at the MSMC, and 65% of patients at the UTH were extubated in the OR. At the MSMC, all patients without aortic cross-clamp, and patients with simple procedures (Risk Adjustment for Congenital Heart Surgery [RACHS] score 1) were extubated in the OR. Among the remaining MSMC patients, regression analysis showed that procedure complexity was still an independent predictor for not proceeding with planned extubation in the OR. Extubation was more likely to be deferred in the RACHS score 3 surgical risk patients compared with the RACHS score 2 group (P = 0.005, odds ratio 3.8 [CI: 1.5, 9.7]). Additionally, trisomy 21 (P = 0.0003, odds ratio 9.9 [CI: 2.9, 34.5]) and age (P = 0.0015) were significant independent predictors for deferring OR extubation. We tested our findings on the patients from the UTH by developing risk categories from the MSMC data that ranked eligible patients according to the chance of OR extubation. The risk categories proved to predict endotracheal extubation in the 49 patients who had undergone surgery at the UTH relative to their overall extubation rate, despite differences in anesthetic regimen and inclusion/exclusion criteria. CONCLUSIONS: Preoperatively known factors alone can predict the relative chances of deferring extubation after surgery for CHD. The early extubation strategies applied in the 2 centers were successful in the majority of cases.

Multisite near-infrared spectroscopy predicts elevated blood lactate level in children after cardiac surgery.

OBJECTIVES: To determine if a relationship exists between regional oxyhemoglobin saturation (rSO(2)) measured at various body locations by near-infrared spectroscopy (NIRS) and blood lactate level in children after cardiac surgery. DESIGN: A prospective, observational study. SETTING: A pediatric cardiac intensive care unit in a university hospital. PARTICIPANTS: Twenty-three children undergoing repair of congenital heart disease. Patients with single-ventricle physiology and/or residual intracardiac shunts were excluded. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Cerebral, splanchnic, renal, and muscle rSO(2) values were recorded every 30 seconds via NIRS for 24 hours postoperatively. Blood lactate levels measured minimally at 0, 2, 4, 6 and 24 hours postoperatively were correlated with rSO(2) values derived by averaging all values recorded during the 60 minutes preceding the blood draw. Twenty-three patients were enrolled with 163 lactate measurements and more than 39,000 rSO(2) observations analyzed. Cerebral rSO(2) had the strongest inverse correlation with lactate level followed by splanchnic, renal, and muscle rSO(2) (r = -0.74, p < 0.0001, r = -0.61, p < 0.0001, r = -0.57, p < 0.0001, and r = -0.48, p < 0.0001, respectively). The correlation improved by averaging the cerebral and renal rSO(2) values (r = -0.82, p < 0.0001). Furthermore, an averaged cerebral and renal rSO(2) value or=3.0 mmol/L with a sensitivity of 95% and a specificity of 83% (p = 0.0001). CONCLUSIONS: Averaged cerebral and renal rSO(2) less than 65% as measured by NIRS predicts hyperlactatemia (>3 mmol/L) in acyanotic children after congenital heart surgery. Hence, this noninvasive, continuous monitoring tool may facilitate the identification of global hypoperfusion caused by low cardiac output syndrome in this population.