Andrew R. Yates

Hyperglycemia is a marker for poor outcome in the postoperative pediatric cardiac patient

Objective: Hyperglycemia in critical care populations has been shown to be a risk factor for increased morbidity and mortality. Minimal data exist in postoperative pediatric cardiac patients. The goal of this study was to determine whether hyperglycemia in the postoperative period was associated with increased morbidity or mortality. Design: Retrospective chart review. Setting: Tertiary care, free-standing pediatric medical center with a dedicated cardiac intensive care unit. Patients: We included 184 patients <1 yr of age who underwent cardiac surgery requiring cardiopulmonary bypass from October 2002 to August 2004. Patients with a weight <2 kg, a preoperative diagnosis of diabetes, preoperative extracorporeal membrane oxygenation support, solid organ transplant recipients, and preoperative renal or liver insufficiency were excluded. Interventions: None. Measurements and Main Results: Age was 4.3 ± 3.2 months and weight was 4.9 ± 1.7 kg at surgery. Duration of hyperglycemia was significantly longer in patients with renal insufficiency (p = .029), liver insufficiency (p = .006), infection (p < .002), central nervous system event (p = .038), extracorporeal membrane oxygenation use (p < .001), and death (p < .002). Duration of hyperglycemia was also significantly associated with increased intensive care (p < .001) and hospital (p < .001) stay and longer ventilator use (p < .001). Peak glucose levels were significantly different in patients with renal insufficiency (p < .001), infection (p = .002), central nervous system event (p = .01), and mortality (p < .001). Conclusions: Hyperglycemia in the postoperative period was associated with increased morbidity and mortality in postoperative pediatric cardiac patient. Strict glycemic control may improve outcomes in this patient population.

Dexmedetomidine: Applications for the Pediatric Patient With Congenital Heart Disease

This study aimed to provide a general description of the cardiovascular and hemodynamic effects of dexmedetomidine and an evidence-based review of the literature regarding its use in infants and children with congenital heart disease (CHD). A computerized bibliographic search of the literature on dexmedetomidine use in infants and children with CHD was performed. The cardiovascular effects of dexmedetomidine have been well studied in animal and adult human models. Adverse cardiovascular effects include occasional episodes of bradycardia, with rare reports of sinus pause or cardiac arrest. Both hypotension and hypertension also have been reported. The latter is related to peripheral α2B agonism leading to vasoconstriction. No adverse effects on the pulmonary vasculature have been noted even in patients with preexisting pulmonary hypertension. Although there are no direct effects on myocardial function, decreased cardiac output may result from changes in heart rate or increases in afterload. Although not currently Food and Drug Administration (FDA)-approved for the pediatric population, findings have shown dexmedetomidine to be effective in various clinical scenarios of patients with CHD including sedation during mechanical ventilation, prevention of procedure-related anxiety, prevention of emergence delirium and shivering after anesthesia, and treatment of withdrawal. Although dexmedetomidine may have limited utility for painful or invasive procedures, preliminary data suggest that the addition of ketamine to the regimen may offer benefits. When used during the perioperative period, additional benefits include blunting of the sympathetic stress response with a reduction of endogenous catecholamine release, a decrease in intraoperative anesthetic requirements, and a limitation of postoperative opioid requirements.

Pediatric Sub-specialist Controversies in the Treatment of Congenital Heart Disease in Trisomy 13 or 18

Trisomy 13 and 18 are associated with congenital heart disease. Cardiac palliation has been reported in the literature, but is not usually done in this population. Thus, a multi-disciplinary team may experience controversy in formulating a care plan that includes cardiac intervention. Our objective was to determine differences in recommendations for cardiac intervention in this population between physicians specializing in pediatric cardiac critical care, neonatology, and genetics. A web-based survey was performed between April 2007 and August 2008. This survey evaluated surgical and transcatheter cardiac palliations that had been performed for individuals with trisomy 13 or 18 at the respondent’s institution, the respondent’s recommendations for cardiac intervention in hypothetical symptomatic patients with trisomy 13 or trisomy 18 and the influence of parental preference on these recommendations. Eight hundred fifty-nine responses were obtained from a primarily academic practice setting (59%). Cardiologists were most likely to recommend intervention; low risk interventions were recommended by 32% of cardiologists, 7% of neonatologists and 20% of geneticists. Parental request to intervene resulted in a 3 fold increased in the likelihood of all specialist recommending intervention. Counseling of families frequently occurred by multiple sub specialists (50%) and there was frequently (71%) a difference in opinion. Individuals with trisomy 13 or 18 are receiving cardiac intervention at many institutions. Cardiologists were more likely than geneticists or neonatologists to recommend intervention on all heart lesions other than single ventricle palliation which no specialists recommended. Parental wishes that “everything be done” significantly influenced all specialists’ recommendations.

Transcatheter Elimination of Left-to-Right Shunts in Infants with Bronchopulmonary Dysplasia Is Feasible and Safe

OBJECTIVE To test the hypothesis that transcatheter elimination of left-to-right (L-R) cardiac shunts in former premature infants with bronchopulmonary dysplasia (BPD) is feasible, safe, and is associated with an improvement in respiratory status. DESIGN Retrospective case review. PATIENTS Twelve patients with BPD who underwent an attempt at transcatheter closure of an L-R shunt lesion within the first year of life at a single center. Median weight was 5.4 kg and median age was 6 months. Fifteen L-R shunt lesions included patent ductus arteriosus (n = 1), atrial septal defect (ASD) (n = 9), and aortopulmonary collaterals (n = 5). OUTCOME MEASURES Echocardiographic and clinical markers were collected before and after intervention as well as procedural variables including successful elimination of the shunt and procedural complications. RESULTS The L-R shunts were successfully occluded in 11/12 (91.6%) patients without any significant procedural adverse event. The ASD closure group demonstrated a decrease in right heart size after the procedure. All patients required respiratory support prior to, and 1 month after, the procedure while only 5/10 (50%) required respiratory support at 12 months of age (P = .0129). There was no change in the median weight percentile over time. CONCLUSION Transcatheter occlusion of L-R shunts can be performed safely and effectively in children with BPD. Further studies may clarify the role of such therapy in improvement in respiratory physiology over time.

Cross-country transfer between two children's hospitals of a child using ambulatory extracorporeal membrane oxygenation for bridge to lung transplant.

With a limited number of pediatric lung transplant programs, the transfer of patients will be required for appropriate candidates. Pediatric patients have been successfully transferred using extracorporeal membrane oxygenation (ECMO) with no previous reports using ambulatory single‐site venovenous (VV) ECMO via a bicaval dual‐lumen (BCDL) catheter as a method for transport to a lung transplant center in order to bridge to lung transplantation. Therefore, we present the successful transfer of a 13‐yr‐old female on ambulatory VV ECMO between two free‐standing childrens hospitals and then bridged to bilateral lung transplantation.

Survival in children on extracorporeal membrane oxygenation at the time of lung transplantation.

Limited data exist on ECMO at the time of LTx in children. The UNOS database was queried from 2000 to 2013 for pediatric lung transplant recipients (<18 yr) to assess post‐transplant survival of patients on ECMO at the time of LTx. Of 587 pediatric recipients with 17 on ECMO, 585 were used for univariate and Kaplan–Meier function analysis, 535 for multivariate Cox models, and 24 for propensity score matching. Univariate Cox (HR = 1.777; 95% CI: 0.658, 4.803; p = 0.257) and Kaplan–Meier function (log‐rank test: chi‐square (df = 1): 1.32, p = 0.250) analyses did not identify a survival difference between ECMO and non‐ECMO, while multivariate Cox models (HR = 1.821; 95% CI: 0.654, 5.065; p = 0.251) did not demonstrate an increased risk for death. Propensity score matching analysis (HR = 1.500; 95% CI: 0.251, 8.977; p = 0.657) also failed to demonstrate a significantly increased hazard ratio. Using a contemporary cohort of pediatric lung transplant recipients, the use of ECMO at the time of lung transplantation did not negatively impact survival.

Improved outcomes with the comprehensive stage 2 procedure after an initial hybrid stage 1.

OBJECTIVE To report our improving institutional experience with the hybrid alternative surgical strategy for the management of hypoplastic left heart syndrome, in which hybrid stage 1 is followed by a comprehensive stage 2 procedure (removal of patent ductus arteriosus stent and pulmonary artery [PA] bands, aorta and PA reconstruction, Damus-Kaye-Stansel, atrial septectomy, Glenn). METHODS In this Institutional Review Board-approved retrospective review of all patients undergoing a comprehensive stage 2 procedure between January 2002 and December 2014, data were compared between the pre-protocol group (n = 64; January 2002 to March 2010) and the post-protocol group (n = 55; March 2010 to December 2014). These 2 groups flank the implementation of a perioperative management protocol to prevent PA thrombosis. RESULTS Pre-protocol mortality was 19% (12 of 64), with the most common mode of death involving PA thrombosis in at least 7 patients, with an urgent indication for surgery and age as contributing factors. Care modifications instituted in March 2010 included avoidance of procedures on an emergent basis or in patients aged <3 months, use of a systemic PA shunt in cases of too-small superior vena cava and/or PA, completion angiogram with a low threshold for intraoperative stenting, and postoperative anticoagulation therapy for 6 weeks. There was a significant decrease in mortality (2 of 55; 4%; P = .01), PA thrombosis (0 of 55; 0%; P = .01), and use of extracorporeal membrane oxygenation (0/55 [0%] compared with 7 of 64 [11%]; P = .01) after protocol implementation. CONCLUSIONS Despite the technical challenges of the comprehensive stage 2 procedure, excellent outcomes are attainable. Experience coupled with an internal quality review drove the implementation of a successful perioperative management protocol.

Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome

OBJECTIVE Multiple surgical approaches to the initial palliation of patients with hypoplastic left heart syndrome (HLHS) have been advocated throughout the years. We sought to examine what procedure, if any, is recommended for HLHS management in regard to physician preference, anatomical variations, and concomitant medical issues. PATIENTS AND METHODS A Web-based survey of pediatric cardiologist subscribed to PediHeart was conducted. OUTCOME MEASURES The types of palliation recommended (Norwood palliation, Sano modification, hybrid palliation, primary cardiac transplantation, or hospice care) for patients with HLHS with anatomic or comorbid variants were queried. Counseling provided by the physicians to families was also documented as regards survival and outcomes. RESULTS Two hundred physicians (21% female) who averaged 12.3 years removed from training responded to the survey. US East Coast and Midwest respondents were more likely to recommend Norwood palliation (54% and 60%, respectively) and the US South and West respondents preferred Sano modification (73% and 82%, respectively). Norwood or Sano palliation was recommended over hospice care, hybrid palliation, or cardiac transplant for patients with an intact atrial septum (P < 0.05), moderate to severe tricuspid regurgitation (P < 0.05), or low birth weight defined as less than 2 kg (P < 0.05). Hospice was preferred in low-birth-weight infants over hybrid palliation or cardiac transplantation (P < 0.05). Hospice was recommended over any other palliation for premature infants (less than 30 weeks gestation), chromosomal abnormalities, or end-organ dysfunction (P < 0.05). CONCLUSIONS This survey demonstrates that different palliative options are primarily recommended by caretakers based upon institutional location and patient characteristics. Prospective comparative trials may force a rethinking of this approach over time.

Venovenous ECMO as a bridge to lung transplant and a protective strategy for subsequent primary graft dysfunction

Extracorporeal membrane oxygenation (ECMO) is an established therapy for primary graft dysfunction (PGD) in adults after lung transplant, while venovenous (VV) ECMO is an evolving therapy that can bridge patients to lung transplantation. This report describes a case of relatively quick improvement of grade 3 PGD, based on the PaO2/FIO2 (P/F) ratio, in a 17-year-old patient with cystic fibrosis who was bridged to lung transplantation with ambulatory VV ECMO and then received support with VV ECMO as a protective strategy during the initial phases of PGD after lung transplantation.

Active rehabilitation with venovenous extracorporeal membrane oxygenation as a bridge to lung transplantation in a pediatric patient

BackgroundActive physical rehabilitation while bridged to lung transplantation with venovenous (VV) extracorporeal membrane oxygenation (ECMO) is an evolving treatment option in adults with limited published experience in pediatric patients.MethodsThe administration of VV ECMO through the placement of a single-site bicaval dual-lumen (BCDL) catheter (Avalon Laboratories, Rancho Dominguez, CA, USA) permits respiratory support in a critically ill patient with avoidance of sedation and paralytics while allowing rehabilitation and oral nutrition.ResultsA 13-year-old girl with advanced interstitial lung disease underwent active rehabilitation while being bridged to lung transplantation with single-site VV ECMO.ConclusionsThe innovative use of single-site VV ECMO with a BCDL catheter is transforming the care of adult patients with advanced lung disease and acute respiratory failure as a method to extend the life of a lung transplantation candidate to maximize all opportunities for organ availability. Based on our experiences, clinicians caring for children should be aware of this potential option in pediatric patients requiring lung transplantation.