Optimal right ventricle-to-pulmonary artery (RV-PA) conduit size for patients with truncus arteriosus is controversial. We aimed to determine the relationship between branch PA size and the need for conduit reoperation following repair of truncus arteriosus. We performed a single-center chart review of patients who underwent truncus arteriosus repair from January 2009 to December 2023. Branch PAs were measured in systole, at the narrowest point if focal stenosis was present. For branch PA diameter analyses, the smaller diameter PA was used. Univariate Cox proportional hazards regression analysis was performed to determine hazard ratios (HRs) with 95% confidence intervals (CIs) for echocardiographic measures and conduit reoperation. We included 33 patients. Median age at surgery was 23 days (range: 3--34). Thirty-two patients received a bovine jugular vein graft, one patient received an aortic homograft. Mean RV-PA conduit Z-score was 2.7 ± 0.5 and mean preoperative conduit-to-PA ratio 2.6 ± 0.6. Postoperative diameter of at least one branch PA was decreased in 31 patients (93.9%); mean change was -19% ± 17%. Mean postoperative conduit-to-PA ratio was 3.3 ± 0.9. Conduit reoperation occurred in 19 patients (58%); median time to reoperation was 1.6 years (range: 0.4-10.4). Conduit reoperation was not associated with conduit diameter or Z-score. Conduit reoperation was significantly associated with truncus type A2 or A3 PA anatomy (HR: 3.53; 95%CI: 1.14-10.94) and conduit-to-PA ratio ≥ 4 (HR: 4.94; 95%CI: 1.63-14.97). In a single-center cohort of children who underwent repair of truncus arteriosus, RV-PA conduit diameter was not associated with increased conduit longevity. Rather, larger postoperative RV-PA conduit to branch PA diameter ratio was significantly associated with greater hazard for conduit reoperation.
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Abstract Objective: We aimed to identify factors independently associated with the need for inotropic support for low cardiac output or haemodynamic instability after pulmonary artery banding surgery for CHD. Methods: We performed a retrospective chart review of all neonates and infants who underwent pulmonary banding between January 2016 and June 2019 at our institution. Bivariate and multivariable analyses were performed to identify factors independently associated with the use of post-operative inotropic support, defined as the initiation of inotropic infusion(s) for depressed myocardial function, hypotension, or compromised perfusion within 24 hours of pulmonary artery banding. Results: We reviewed 61 patients. Median age at surgery was 10 days (25%,75%:7,30). Cardiac anatomy was biventricular in 38 patients (62%), hypoplastic right ventricle in 14 patients (23%), and hypoplastic left ventricle in 9 patients (15%). Inotropic support was implemented in 30 patients (49%). Baseline characteristics of patients who received inotropic support, including ventricular anatomy and pre-operative ventricular function, were not statistically different from the rest of the cohort. Patients who received inotropic support, however, were exposed to larger cumulative doses of ketamine intraoperatively – median 4.0 mg/kg (25%,75%:2.8,5.9) versus 1.8 mg/kg (25%,75%:0.9,4.5), p < 0.001. In a multivariable model, cumulative ketamine dose greater than 2.5mg/kg was associated with post-operative inotropic support (odds ratio 5.5; 95% confidence interval: 1.7,17.8), independent of total surgery time. Conclusions: Inotropic support was administered in approximately half of patients who underwent pulmonary artery banding and more commonly occurred in patients who received higher cumulative doses of ketamine intraoperatively, independent of the duration of surgery.
BACKGROUND Enoxaparin may be used to prevent central venous catheter-related thrombosis in patients with CHD. We aimed to determine whether current enoxaparin dosing regimens effectively achieve anti-factor Xa concentrations within prophylactic goal ranges in this patient population. METHODS We implemented a formal protocol aimed at reducing central venous catheter-related thrombosis in children with CHD in January, 2016. Standard empiric prophylactic enoxaparin dosing regimens were used - for example, 0.75 mg/kg/dose every 12 hours for patients <2 months of age and 0.5 mg/kg/dose every 12 hours for patients ⩾2 months of age - with anti-factor Xa goal range of 0.25-0.49 IU/ml. Patients <2 years of age who received enoxaparin and had at least one valid steady-state anti-factor Xa measurement between 25 January, 2016 and 31 August, 2016 were retrospectively reviewed. RESULTS During the study period, 47 patients had 186 anti-factor Xa concentrations measured, of which 20 (11%) were above and 112 (60%) were below the prophylactic goal range. Anti-factor Xa concentrations within the goal range were ultimately achieved in 31 patients. Median dose required to achieve anti-factor Xa concentrations within the prophylactic range was 0.89 mg/kg/dose (25, 75%: 0.75, 1.11) for patients <2 months (n=23 patients) and 0.79 mg/kg/dose (25, 75%: 0.62, 1.11) for patients ⩾2 months (n=8 patients). CONCLUSIONS Enoxaparin doses required to achieve prophylactic anti-factor Xa concentrations in young children with CHD were consistently higher than the currently recommended prophylactic dosing regimens. Further study is needed to determine whether dose titration to achieve prophylactic anti-factor Xa concentrations is effective in preventing central venous catheter-related thrombosis.
Division of Critical Care, Department of Pediatrics, Indiana University School of Medicine; Riley Hospital for Children, Indianapolis, IN The author has disclosed that he does not have any potential conflicts of interest.
Introduction: Due to concerns regarding high sodium loads in children with recovering cardiac function, use of hypotonic parenteral maintenance fluid therapy in children after cardiac surgery is still common at most centers. To our knowledge however, no data exists to date comparing different maintenance fluid strategies in this patient population. We aimed to compare the use of 0.45% saline to 0.2% saline in parenteral maintenance fluid therapy in children recovering from cardiac surgery. Methods: An IRB-approved retrospective study was conducted on children ≤ 12 years of age who underwent cardiac surgery with cardiopulmonary bypass at our institution from 8/15/2012–8/14/2013 and recovered in the ICU for at least 48 hours. Patients from 2/15/2013 - 8/14/2013, who received 0.45% saline as part of parenteral maintenance fluid therapy, were compared to patients from 8/15/2012 - 2/14/2013, who received 0.2% saline, using t-tests, Mann-Whitney U tests, or χ-square tests as appropriate. Sodium values were recorded for up to seven ICU days post-operatively. Data are provided as mean±standard deviation unless otherwise noted. Results: Sixty-nine patients receiving 0.45% saline and 74 receiving 0.2% saline were reviewed. Median ages of those receiving 0.45% and 0.2% were 6.3 months (range: 0.03–142) and 6.6 months (range: 0.17–120), respectively, P=0.72. Baseline sodium was 143.6 ± 0.3 meq/dL in patients receiving 0.45% and 144.5 ± 0.4 meq/dL in patients receiving 0.2%, P=0.10. In patients receiving 0.45% however, hyponatremia occurred less often (35% versus 52%, P=0.03), lowest sodium measured was higher (135.7 ± 0.5 meq/L versus 134.2 ± 0.4 meq/dL, P<0.016), and mean change in sodium from baseline was lower (7.9 ± 0.5 meq/L versus 10.3 ± 0.6 meq/dL, P=0.003). Of note, the number of post-operative ICU days on which sodium measurements were obtained was similar between groups, 4.5 ± 0.2 (0.45%) versus 4.4 ± 0.2 (0.2%), P=0.90. Unexpectedly, peak sodium was lower in those receiving 0.45%, 148.2 ± 0.4 meq/L versus 149.3 ± 0.4 meq/dL, P=0.04. Lastly, median ICU stay was not statistically different between groups, 4 days (intraquartile range: 3–14.5) in patients receiving 0.45% and 6 days (intraquartile range: 4–14) in patients receiving 0.2%, P=0.385. Conclusions: Use of 0.45% saline as compared to 0.2% saline as part of parenteral maintenance fluid therapy is associated with a lower incidence of hyponatremia but not a greater incidence of hypernatremia or prolonged ICU length of stay. Future research comparing isotonic parenteral maintenance fluid therapy to 0.45% saline is warranted in this patient population.
Primary myelofibrosis is rare in children. Many causes have been described for secondary myelofibrosis including vitamin D deficiency. Here, we describe a patient with myelofibrosis secondary to vitamin D deficiency, as diagnosed by laboratory evidence. The patient also developed resultant extramedullary hematopoiesis with secondary development of ascites as a result of myelofibrosis. These are findings rarely reported in association with vitamin D deficiency. Potential mechanisms are also discussed.
