During extracorporeal membrane oxygenation (ECMO), a delicate balance is required to titrate systemic anticoagulation to prevent thrombotic complications within the circuit and prevent bleeding in the patient. Despite focused efforts to achieve this balance, the frequency of both thrombotic and bleeding events remains high. Anticoagulation is complicated to manage in this population due to the complexities of the hemostatic system that are compounded by age-related developmental hemostatic changes, variable effects of the etiology of critical illness on hemostasis, and blood-circuit interaction. Lack of high-quality data to guide anticoagulation management in ECMO patients results in marked practice variability among centers. One aspect of anticoagulation therapy that is particularly challenging is the use of antithrombin (AT) supplementation for heparin resistance. This is especially controversial in the neonatal and pediatric population due to the baseline higher risk of bleeding in this cohort. The indication for AT supplementation is further compounded by the potential inaccuracy of the diagnosis of heparin resistance based on the standard laboratory parameters used to assess heparin effect. With concerns regarding the adverse impact of bleeding and thrombosis, clinicians and institutions are faced with making difficult, real-time decisions aimed at optimizing anticoagulation in this setting. In this clinically focused review, the authors discuss the complexities of anticoagulation monitoring and therapeutic intervention for patients on ECMO and examine the challenges surrounding AT supplementation given both the historical and current perspectives summarized in the literature on these topics.

Cardiac involvement has been reported in various mucopolysaccharidoses syndromes. Cardiac valve pathology is the most prominent cardiac manifestation of patients with these syndromes. To date, there have been no reports of early childhood onset of high-grade atrioventricular block in patients with Hunter syndrome. We present a case of a 3-year-old boy with Hunter syndrome who was found to have various degrees of atrioventricular block. This case highlights the importance of early routine cardiac screening for conduction abnormalities and close follow-up in patients with mucopolysaccharidoses syndromes.

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Cardiac myxoma is the most common cardiac tumor in patients of all ages; the majority are encountered as single left atrial tumors. Left ventricular myxomas are exceedingly rare, having been recorded in a small number of case reports involving children worldwide. We report a case of a left ventricular myxoma with left ventricular outflow tract obstruction in a previously healthy, asymptomatic adolescent black male. Transthoracic echocardiograms revealed a single, large (2.5 × 5-cm), lobulated, mobile mass within the left ventricular cavity that oscillated into the outflow tract, thereby causing moderate obstruction during systole. Advanced images delineated the location and tissue composition of the mass, characterizing it as a myxoma. Complete surgical excision of the mass was accomplished via aortotomy. Gross examination and histology confirmed the diagnosis of myxoma.

Introduction: Optimal management of neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) with confluent pulmonary arteries is unknown. We sought to compare outcomes for patients who underwent primary complete repair vs. initial surgical palliation followed by delayed repair. Methods: We conducted a retrospective study at 20 centers within CoRe-PCICS (Collaborative Research from the Pediatric Cardiac Intensive Care Society). Data were collected on infants undergoing initial surgical intervention at 0 - 60 days of age with TOF/PA from 2009 to 2018, excluding patients with MAPCAs or those undergoing ductal stenting (n=22). The primary outcome was days alive and out of hospital in first year of life (DAOH). Secondary outcomes were 1 year mortality and a composite major complication outcome (similar to that in prior STS-CHSD studies), defined as occurrence of ≥ 1 of the following: renal failure requiring dialysis, stroke/seizure, permanent pacemaker, ECMO, or diaphragm paralysis during a palliation and/or repair hospitalization, or unplanned reoperation in the first year. Multivariable modeling with generalized estimating equations were utilized to compare outcomes between groups. Results: Of 210 subjects, 79 underwent primary complete repair and 131 underwent surgical palliation. Patients who underwent palliation had greater use of preoperative mechanical ventilation at first procedure (26% vs. 8%, p = 0.002). Other baseline characteristics were similar between groups (p > 0.05 for all). There was no statistically significant difference in DAOH between the palliation and primary repair groups [median (25%,75% IQR): 319 (280,336) vs. 338 (314,348 days), adjusted p = 0.20]. Nine (7%) patients who underwent palliation died in the first year of life vs. 4 (6 %) who underwent primary repair (adjusted OR: 1.1, 95% CI: 0.3-4.5; p = 0.9). At least one major complication occurred in 35% of patients who underwent palliation vs. 18% of patients who underwent primary repair (adjusted OR: 2.5, 95% CI: 1.4-4.4, p = 0.001). Conclusions: For infants with TOF/PA with confluent pulmonary arteries, a strategy of surgical palliation or primary complete repair resulted in similar DAOH and early mortality, whereas the morbidity incidence favored primary repair.

Objectives: To reduce unnecessary antibiotic exposure in a pediatric cardiac intensive care unit (CICU). Design: Single-center, quality improvement initiative. Monthly antibiotic utilization rates were compared between 12-month baseline and 18-month intervention periods. Setting: A 25-bed pediatric CICU. Patients: Clinically stable patients undergoing infection diagnosis were included. Patients with immunodeficiency, mechanical circulatory support, open sternum, and recent culture-positive infection were excluded. Interventions: The key drivers for improvement were standardizing the infection diagnosis process, order-set creation, limitation of initial antibiotic prescription to 24 hours, discouraging indiscriminate vancomycin use, and improving bedside communication and situational awareness regarding the infection diagnosis protocol. Results: In total, 109 patients received the protocol; antibiotics were discontinued in 24 hours in 72 cases (66%). The most common reasons for continuing antibiotics beyond 24 hours were positive culture (n = 13) and provider preference (n = 13). A statistical process control analysis showed only a trend in monthly mean antibiotic utilization rate in the intervention period compared to the baseline period: 32.6% (SD, 6.1%) antibiotic utilization rate during the intervention period versus 36.6% (SD, 5.4%) during the baseline period (mean difference, 4%; 95% CI, −0.5% to −8.5%; P = .07). However, a special-cause variation represented a 26% reduction in mean monthly vancomycin use during the intervention period. In the patients who had antibiotics discontinued at 24 hours, delayed culture positivity was rare. Conclusions: Implementation of a protocol limiting empiric antibiotic courses to 24 hours in clinically stable, standard-risk, pediatric CICU patients with negative cultures is feasible. This practice appears safe and may reduce harm by decreasing unnecessary antibiotic exposure.

Recombinant angiotensin II is an emerging drug therapy for refractory hypotension. Its use is relevant to patients with disruption of the renin–angiotensin–aldosterone system denoted by elevated direct renin levels. We present a child that responded to recombinant angiotensin II in the setting of right ventricular hypertension and multi-organism septic shock.

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