Umesh Dyamenahalli

Propranolol for infantile hemangiomas: Early experience at a tertiary vascular anomalies center

Propranolol has recently been introduced as a novel pharmacologic treatment for infantile hemangiomas. Systematic examination of this treatment in a tertiary care setting has not been described. This study explores the impact of propranolol on both proliferative and involuting hemangiomas at a tertiary vascular anomalies center.

Propranolol for Airway Hemangiomas: Case Report of Novel Treatment

Infantile hemangiomas arising in the trachea are rare. These lesions pose a management dilemma as several treatment options can provide safe management. Propranolol, a nonselective beta‐blocker, has recently been introduced as a novel modality for the treatment of proliferating hemangiomas. This report illustrates the successful management of tracheal hemangiomas using oral propranolol in a young patient with otherwise treatment‐resistant airway lesions. Despite various endoscopic therapeutic attempts, the patient remained stridulous with airway disease that persisted into the involution phase of the average hemangioma cycle. Within 6 weeks of beginning oral propranolol (2 mg/kg/day), her airway compromise was eliminated and she had complete resolution of endoscopically visible disease. No side effects from propranolol occurred. We propose that oral propranolol should be considered for use in airway hemangiomas. Laryngoscope, 2009

Bridge to Cardiac Transplant in Children: Berlin Heart versus Extracorporeal Membrane Oxygenation

BACKGROUND For small children requiring mechanical circulatory support as a bridge to transplantation (BTT), extracorporeal membrane oxygenation (ECMO) has been the only option until the recent introduction of the Berlin Heart EXCOR ventricular assist device (Berlin Heart AG, Berlin, Germany). We reviewed our recent experience with these two technologies with particular focus on early outcomes. METHODS Data for 55 consecutive children undergoing BTT between 2001 and 2008 were abstracted from an institutional database. The analysis excluded 13 patients because EXCOR was not used for acute postcardiotomy BTT. Patients were divided into ECMO (n = 21) and EXCOR groups (n = 21). Specific end points included survival to transplant, overall survival, and bridge to recovery. Incidences of adverse events and the duration of support were determined. RESULTS Groups were similar in weight, age, and etiologies of heart failure. Likewise, the incidences of stroke and multisystem organ failure were similar. Survival to transplant, recovery, or continued support was 57% in ECMO and 86% in EXCOR (p = 0.040). EXCOR patients had overall significantly better survival (p = 0.049). Two ECMO patients and 1 EXOR patient were bridged to recovery. The mean duration of support was 15 +/- 12 days in the ECMO group and 42 +/- 43 days in the EXCOR group (p < 0.001). CONCLUSIONS In children requiring BTT, EXCOR provided substantially longer support times than ECMO, without significant increase in the rates of stroke or multisystem organ failure. Survival to transplant and long-term survival was higher with EXCOR.

Outcomes after extracorporeal cardiopulmonary resuscitation (ECPR) following refractory pediatric cardiac arrest in the intensive care unit

AIM To describe our experience using extracorporeal cardiopulmonary resuscitation (ECPR) in resuscitating children with refractory cardiac arrest in the intensive care unit (ICU) and to describe hospital survival and neurologic outcomes after ECPR. METHODS A retrospective chart review of a consecutive case series of patients requiring ECPR from 2001 to 2006 at Arkansas Childrens Hospital. Data from medical records was abstracted and reviewed. Primary study outcomes were survival to hospital discharge and neurological outcome at hospital discharge. RESULTS During the 6-year study period, ECPR was deployed 34 times in 32 patients. 24 deployments (73%) resulted in survival to hospital discharge. Twenty-eight deployments (82%) were for underlying cardiac disease, 3 for neonatal non-cardiac (NICU) patients and 3 for paediatric non-cardiac (PICU) patients. On multivariate logistic regression analysis, only serum ALT (p-value=0.043; OR, 1.6; 95% confidence interval, 1.014-2.527) was significantly associated with risk of death prior to hospital discharge. Blood lactate at 24h post-ECPR showed a trend towards significance (p-value=0.059; OR, 1.27; 95% confidence interval, 0.991-1.627). The Hosmer-Lemeshow tests (p-value=0.178) suggested a good fit for the model. Neurological evaluation of the survivors revealed that there was no change in PCPC scores from a baseline of 1-2 in 18/24 (75%) survivors. CONCLUSIONS ECPR can be used successfully to resuscitate children following refractory cardiac arrest in the ICU, and grossly intact neurologic outcomes can be achieved in a majority of cases.

Incremental reduction in the incidence of stroke in children supported with the Berlin EXCOR ventricular assist device.

BACKGROUND Cerebrovascular events (CVEs) are common among children supported with the Berlin EXCOR (Berlin Heart GmbH, Berlin, Germany) ventricular assist device (VAD). Given the high incidence of CVEs associated with this device, we sought to describe our institutional experience in incrementally reducing CVEs in children supported with the Berlin EXCOR VAD. METHODS We collected pertinent data on 39 consecutive patients who underwent Berlin EXCOR VAD implantation at a single center. Frequency of CVEs was described in risk per implantation, per day, and in reference to the time of therapeutic anticoagulation. Risk factors were analyzed for association with CVEs. RESULTS Of the initial 39 Berlin EXCOR VAD implantations, 16 CVEs occurred in 12 patients. The incidence of CVEs decreased with institutional experience per patient (R(2) = 0.6909, p = 0.007) and per patient-day (R(2) = 0.8051, p = 0.002). CVEs occurred more frequently before therapeutic anticoagulation targets were achieved (4.1%/day) compared with after therapeutic anticoagulation targets were achieved (0.9%/day; p = 0.044). CONCLUSIONS Incidence of CVEs decreased with institutional experience. The risk of CVE is highest in the immediate postoperative period before therapeutic anticoagulation is achieved. Further studies are warranted in pediatric patients supported with the Berlin EXCOR VAD to confirm our findings in a larger cohort.

Ketamine as a neuroprotective and anti-inflammatory agent in children undergoing surgery on cardiopulmonary bypass: a pilot randomized, double-blind, placebo-controlled trial.

Objective: Infants are potentially more susceptible to cell death mediated via glutamate excitotoxicity attributed to cardiopulmonary bypass. We hypothesized that ketamine, via N-methyl D-aspartate receptor blockade and anti-inflammatory effects, would reduce central nervous system injury during cardiopulmonary bypass. Methods: We randomized 24 infants, without chromosomal abnormalities, to receive ketamine (2 mg/kg, n = 13) or placebo (saline, n = 11) before cardiopulmonary bypass for repair of ventricular septal defects. Plasma markers of inflammation and central nervous system injury were compared at the end of surgery, and 6, 24, and 48 hrs after surgery. Magnetic resonance imaging and spectroscopy before cardiopulmonary bypass and at the time of hospital discharge were performed in a subset of cases and controls (n = 5 in each group). Cerebral hemodynamics were monitored postoperatively using near-infrared spectroscopy, and neurodevelopmental outcomes were assessed using Bayley Scales of Infant Development-II before and 2–3 wks after surgery. Results: Statistically significant differences were noted in preoperative inspired oxygen levels, intraoperative cooling and postoperative temperature, respiratory rate, platelet count, and bicarbonate levels. The peak concentration of C-reactive protein was lower in cases compared to controls at 24 hrs (p = .048) and 48 hrs (p = .001). No significant differences were noted in the expression of various cytokines, chemokines, S100, and neuron-specific enolase between the cases and controls. Magnetic resonance imaging with spectroscopy studies showed that ketamine administration led to a significant decrease in choline and glutamate plus glutamine/creatine in frontal white matter. No statistically significant differences occurred between pre- and postoperative Bayley Scales of Infant Development-II scores. Conclusions: We did not find any evidence for neuroprotection or neurotoxicity in our pilot study. A large, adequately powered randomized control trial is needed to discern the central nervous system effect of ketamine on the developing brain. brain. Trial Registration: The trial is registered at www.ClinicalTrials.gov, NCT00556361.

Antithrombin III supplementation on extracorporeal membrane oxygenation: impact on heparin dose and circuit life.

Antithrombin III (ATIII) is used during extracorporeal membrane oxygenation (ECMO) based on physiologic rationale and studies during cardiopulmonary bypass. In February 2008, our institution began using ATIII as replacement for low ATIII activity (<70%) in patients supported with ECMO. We hypothesized that ATIII supplementation would reduce heparin infusion rates, increase unfractionated heparin anti-Xa levels, and prolong ECMO circuit life. Data from 40 consecutive patients (45 deployments) requiring ECMO support for >72 hours with venoarterial ECMO from January 1, 2007, through December 31, 2008, were collected. Antithrombin III concentrate was administered for ATIII activity <70% at the discretion of the attending physician. The primary outcome was whether the heparin infusion rate was reduced by 10% or more as a result of ATIII administration. No difference in heparin infusion rate (p = 0.245) as a result of ATIII administration was observed. Anti-Xa levels were lower before ATIII administration (p< 0.001) and were increased after ATIII administration (p < 0.001). There was an increased frequency of circuit failure in ATIII treatment group compared with nontreatment group (p = 0.018). Neither heparin responsiveness nor circuit life was enhanced by daily ATIII supplementation for activity <70%. Future studies are warranted to evaluate the effectiveness of antithrombin replacement.

Intrahospital transport of children on extracorporeal membrane oxygenation: Indications, process, interventions, and effectiveness

Objective: To evaluate indications, process, interventions, and effectiveness of patients undergoing intrahospital transport. Critically ill patients supported with extracorporeal membrane oxygenation are transported within the hospital to the radiology suite, cardiac catheterization suite, operating room, and from one intensive care unit to another. No studies to date have systematically evaluated intrahospital transport for patients on extracorporeal membrane oxygenation. Design: Retrospective cohort analysis. Setting: Cardiac intensive care unit in a tertiary care childrens hospital. Patients: All patients on extracorporeal membrane oxygenation who required intrahospital transport between January 1996 and March 2007 were included and analyzed. Measurements and Main Results: A total of 57 intrahospital transports for cardiac catheterization and head computed tomography scans were analyzed. In 14 (70%) of 20 of patients with cardiac catheterization, a management change occurred as a result of the diagnostic cardiac catheterization. In ten (59%) of 17 patients, bedside echocardiography was of limited value in defining the critical problem. In the interventional group, the majority of transports were for atrial septostomy. In the head computed tomography group, significant pathology was identified, which led to management change. No major complications occurred during these intrahospital transports. Conclusions: Although transporting patients on extracorporeal membrane oxygenation is labor intensive and requires extensive logistic support, it can be carried out safely in experienced hands and it can result in important therapeutic and diagnostic yields. To our knowledge, this is the first study designed to evaluate safety and efficacy of intrahospital transport for patients receiving extracorporeal membrane oxygenation support.

Extracorporeal membrane oxygenation support for intractable primary arrhythmias and complete congenital heart block in newborns and infants: Short-term and medium-term outcomes

Objectives: To describe the experience with extracorporeal membrane oxygenation support for intractable primary arrhythmias in newborns and infants. Design: Retrospective study. Setting: A tertiary care pediatric hospital. Patients: Patients younger than 1 yr supported with extracorporeal membrane oxygenation for primary cardiac arrhythmias were identified from the institutional extracorporeal membrane oxygenation registry. Interventions: Extracorporeal membrane oxygenation support. Measurements and Main Results: Clinical characteristics and outcomes were investigated for patients with primary cardiac arrhythmia supported with extracorporeal membrane oxygenation. Outcomes investigated were time from initiation of extracorporeal membrane oxygenation support to arrhythmia control, duration of extracorporeal membrane oxygenation support, and results of interventions performed while supported with extracorporeal membrane oxygenation. We summarized the independent categorical and continuous variables using frequencies, percentages, and medians and ranges, respectively. Extracorporeal membrane oxygenation support was used in nine patients for rescue therapy for primary tachyarrhythmia and bradycardia. The primary arrhythmias were: focal atrial tachycardia (n = 2); reentrant supraventricular tachycardia (n = 3); junctional ectopic tachycardia (n = 2); and congenital complete atrioventricular block (n = 2) patients. Seven patients presented with severe hemodynamic compromise, with six patients requiring extracorporeal cardiopulmonary resuscitation. All patients required extracorporeal membrane oxygenation within 24 hrs of initial presentation. Balloon atrial septostomy was performed in three patients and ablation was performed in two patients. Sinus rhythm was achieved in all reentrant supraventricular tachycardia and rate control was established in both patients with focal atrial tachycardia and in one patient with junctional ectopic tachycardia while using extracorporeal membrane oxygenation support. All patients survived to hospital discharge, and median follow-up for the cohort was 5 yrs. There was one late death; all survivors had good overall and neurologic outcomes. Conclusions: The requirement of extracorporeal membrane oxygenation support in newborns and infants with intractable arrhythmia is rare. Extracorporeal membrane oxygenation support does potentially carry morbidity; however, to prevent arrhythmia-related mortality, extracorporeal membrane oxygenation support and/or extracorporeal cardiopulmonary resuscitation should be considered in the management of hemodynamically unstable primary arrhythmias as an emergent lifesaving procedure.

Preoperative lymphopenia is a predictor of postoperative adverse outcomes in children with congenital heart disease

OBJECTIVE Lymphopenia is a predictor of adverse clinical outcomes in adults with various systemic diseases. We hypothesized that preoperative absolute lymphopenia (absolute lymphocyte count of less than 3000 cells/microL) is associated with adverse postoperative outcomes in children with congenital heart disease undergoing corrective or palliative surgery on cardiopulmonary bypass during the first 2 years of life. METHODS A retrospective single center cohort study was performed. Categorical variables were analyzed with the chi(2) test. Preoperative variables were analyzed with logistic and linear regression analysis to determine whether they were associated with adverse outcomes. RESULTS Analysis was performed on 280 patients, of whom 124 were female and 156 were male. Seventy-one patients were neonates (< or =30 days) at the time of the operation. Ninety patients had an absolute lymphocyte count of less than 3000 cells//microL before the operation. Regression models showed that RACHS-1 categories 5 and 6, age, and preoperative lymphopenia were significantly associated with postoperative mortality (P < .0006). Within RACHS-1 groups, lymphopenia remained a significant predictor of mortality for patients in RACHS categories 3 and 4. Lymphopenia and age were associated with longer length of stay and length of mechanical ventilation within RACHS categories 1 to 4 (P < .05). Preoperative lymphopenia was the only predictor of use of postoperative nitric oxide (P < .05). CONCLUSIONS Preoperative lymphopenia is a predictor of adverse postoperative outcomes in children with congenital heart disease who undergo a corrective or palliative procedure with cardiopulmonary bypass during the first 2 years of life.