Development of Consensus-Based Best Practice Guidelines for the Peri- and Post-operative Care of Pediatric Patients with Spinal Deformity and Programmable Implanted Devices.

STUDY DESIGN: Modified Delphi consensus study. OBJECTIVE: To develop consensus-based best practices for the care of pediatric patients who have implanted programmable devices (IPDs) and require spinal deformity surgery. SUMMARY OF BACKGROUND DATA: Implanted programmable devices (IPDs) are often present in patients with neuromuscular or syndromic scoliosis who require spine surgery. Guidelines for monitoring and interrogating these devices during the peri-operative period are not available. METHODS: A panel was assembled consisting of 25 experts (i.e., spinal deformity surgeons, neurosurgeons, neuro-electrophysiologists, cardiologists, and otolaryngologists). Initial postulates were based on literature review and results from a prior survey. Postulates addressed the following IPDs: vagal nerve stimulators (VNS), programmable ventriculo-peritoneal shunts (VPS), intrathecal baclofen pumps (ITBP), cardiac pacemakers and implantable cardioverter-defibrillators (ICD), deep brain stimulators (DBS), and cochlear implants. Cardiologist and otolaryngologists participants responded only to postulates on cardiac pacemakers or cochlear implants, respectively. Consensus was defined as ≥80% agreement, items that did not reach consensus were revised and included in subsequent rounds. A total of three survey rounds and one virtual meeting were conducted. RESULTS: Consensus was reached on 39 total postulates across six IPD types. Postulates addressed general spine surgery considerations, use of intraoperative monitoring and cautery, use of magnetically-controlled growing rods (MCGRs), and use of an external remote controller to lengthen MCGRs. Across IPD types, consensus for the final postulates ranged from 94.4-100%. Overall, experts agreed that MCGRs can be surgically inserted and lengthened in patients with a variety of IPDs and provided guidance for the use of intraoperative monitoring and cautery, which varied between IPD types. CONCLUSION: Spinal deformity correction surgery often benefits from the use of intraoperative monitoring, monopolar and bipolar cautery, and MCGRs. Final postulates from this study can inform the peri- and post-operative practices of spinal deformity surgeons who treat patients with both scoliosis and IPDs. LEVEL OF EVIDENCE: V- Expert opinion.

3D advanced imaging overlay with rapid registration in CHD to reduce radiation and assist cardiac catheterisation interventions.

Novel commercially available software has enabled registration of both CT and MRI images to rapidly fuse with X-ray fluoroscopic imaging. We describe our initial experience performing cardiac catheterisations with the guidance of 3D imaging overlay using the VesselNavigator system (Philips Healthcare, Best, NL). A total of 33 patients with CHD were included in our study. Demographic, advanced imaging, and catheterisation data were collected between 1 December, 2016 and 31 January, 2019. We report successful use of this technology in both diagnostic and interventional cases such as placing stents and percutaneous valves, performing angioplasties, occlusion of collaterals, and guidance for lymphatic interventions. In addition, radiation exposure was markedly decreased when comparing our 10-15-year-old coarctation of the aorta stent angioplasty cohort to cases without the use of overlay technology and the most recently published national radiation dose benchmarks. No complications were encountered due to the application of overlay technology. 3D CT or MRI overlay for CHD intervention with rapid registration is feasible and aids decisions regarding access and planned angiographic angles. Operators found intraprocedural overlay fusion registration using placed vessel guidewires to be more accurate than attempts using bony structures.

Congenital pulmonary vascular anomalies.

Congenital pulmonary vascular anomalies are typically found in infancy or early childhood however, some may remain silent and present in adult patients. Anomalies may be separated into anatomic categories based on involvement of the pulmonary arteries, pulmonary veins or both with or without involvement of the lung parenchyma. Association with congenital heart disease and other syndromes is very common. Computed tomography (CT) and magnetic resonance imaging (MRI) are both invaluable at assessment of these anomalies allowing for both diagnosis and detailed treatment planning. This article will focus primarily on the use of CT, as the high resolution evaluation of the lung parenchyma is also important in many of these conditions. In young patients especially, rapid heart rate and concerns of radiation exposure are important considerations when performing CT. This article will discuss scan techniques as well as clinical diagnostic considerations and basic endovascular treatment of congenital pulmonary vascular anomalies.

Serum Cystatin C as an Early Marker of Neutrophil Gelatinase-associated Lipocalin-positive Acute Kidney Injury Resulting from Cardiopulmonary Bypass in Infants with Congenital Heart Disease.

OBJECTIVE: Acute kidney injury (AKI) is a common complication resulting from cardiopulmonary bypass in infants. Urinary neutrophil gelatinase-associated lipocalin (NGAL) is a sensitive and specific marker of such injury. In this study, we compared the performance of serum cystatin C (Cys C) and serum creatinine (Cr) as early markers of renal dysfunction in infants undergoing cardiac surgery under bypass. STUDY DESIGN, SETTING, AND PATIENTS: The study was designed as a prospective observational study. The study was conducted in the cardiac intensive care unit (ICU) of a tertiary, academic children's hospital in the United States. Infants (age <1 year) undergoing cardiac surgery under cardiopulmonary bypass were included in the study. OUTCOME MEASURE: Acute kidney injury was defined based on postoperative urinary NGAL. RESULTS: A total of 17 infants were included in the study, and five of them developed AKI. Serum Cys C and Cr levels were measured postoperatively on days 1, 2, and 3, and compared with baseline levels. On postoperative day 2, infants with AKI showed significant change from baseline in serum Cys C levels compared with non-AKI infants (28% vs. -9%, P = .03). The two groups did not show significant differences with respect to rise in serum Cr on any of the 3 postoperative days. Serum Cr on days 1 and 2 showed nonspecific increases in both AKI and non-AKI groups. The area under the receiver operating characteristic curve for day 2 Cys C was 0.87 (95% CI 0.67-1.00) in recognizing NGAL-positive AKI. CONCLUSIONS: Postoperative serum Cys C appears to be a more specific and sensitive biomarker for NGAL-positive AKI resulting from cardiopulmonary bypass surgery in infants undergoing cardiac surgery.

Management and outcomes of heterotaxy syndrome associated with pulmonary atresia or pulmonary stenosis.

BACKGROUND: Historic outcomes of patients with heterotaxy and pulmonary atresia or pulmonary stenosis (PA/PS) have been poor and in the current era are incompletely described. We reviewed our management of these patients and associated risk factors for death. METHODS: We retrospectively reviewed the records of all patients with heterotaxy and PA/PS treated in our institution from January 1, 2002, to August 31, 2012. Death data were also confirmed with the Social Security Death Index. The log-rank test was done to assess six risk factors for death. RESULTS: We identified 42 patients with heterotaxy and PA/PS. Median age at the first operation was 6.5 days, and median follow-up was 3.5 years. Death data were complete for all patients. Overall mortality was 19% (8 of 42). The 30-day, 1-year, and 5-year mortality estimation was 4.76%, 12.3%, and 19.1% respectively, as determined by the Kaplan-Meier method. The log-rank test showed total anomalous pulmonary venous return (TAPVR) (p<0.05) and obstructed TAPVR requiring an operation at less than 30 days (p=0.001) were significant risk factors for death. CONCLUSIONS: In the current era, surgical treatment of heterotaxy and PA/PS can result in good outcomes. Associated TAPVR and obstructed TAPVR requiring neonatal correction were noted to be risk factors for death.

Incidence of acute kidney injury following cardiac catheterization prior to cardiopulmonary bypass in children.

OBJECTIVES: To determine whether contrast administration was a risk factor for development of acute kidney injury (AKI) in cyanotic congenital heart disease (CHD) patients undergoing cardiopulmonary bypass (CPB). BACKGROUND: AKI following CPB or contrast administration is well described. In previous studies, administration of contrast prior to CPB has been shown to increase the risk of AKI. Chronic cyanosis leads to glomerular damage and dysfunction, thus potentially placing this population at increased risk of developing AKI following contrast administration prior to CPB. METHODS: One hundred twenty-two patients with cyanotic CHD undergoing preoperative cardiac catheterization (PCC) and subsequent CPB at Children's Medical Center of Dallas from January 1, 2007 until November 30, 2010 were identified, looking specifically at bi-directional Glenn (BDG) anastomoses and Fontan procedures. One hundred thirteen patients undergoing PCC ≤ 48 hr prior to and > 5 days prior to CPB were included. Occurrence of AKI following CPB was the primary outcome variable. RESULTS: Logistic regression analysis revealed pre-catheterization serum creatinine was a risk factor for post-CPB AKI (P < 0.001) in both Fontan and BDG patients. All other variables were not significantly associated with the development of AKI in either BDG or Fontan patients. Length of stay (hospital or ICU) was not different among the groups regardless of the occurrence of AKI. CONCLUSIONS: In this study of cyanotic CHD patients, contrast administration within 48 hr prior to CPB was not an additional risk factor for the development of AKI.