Extracorporeal membrane oxygenation in children with heart disease and del22q11 syndrome: a review of the Extracorporeal Life Support Organization Registry.

The study objective was to evaluate outcomes among children with del22q11 (DiGeorge) syndrome supported on ECMO for heart disease. The ELSO registry database was queried to include all children <18 years undergoing heart surgery for either common atrio-ventricular canal, tetralogy of Fallot, truncus arteriosus or transposition of the great vessels and interrupted aortic arch and requiring ECMO, from 1998-2011. The outcomes evaluated included mortality, ECMO duration and length of hospital stay in patients with del22q11 syndrome and with no del22q11 syndrome. Eighty-eight ECMO runs occurred in children with del22q11 syndrome while 2694 ECMO runs occurred in children without del22q11 syndrome. For patients with heart defects receiving ECMO, del22q11 syndrome did not confer a significant mortality risk or an increased risk of infectious complications before or while on ECMO support. Neither the duration of ECMO nor mechanical ventilation prior to ECMO deployment were prolonged in patients with del22q11 syndrome compared to the controls.

Is age at initiation of extracorporeal life support associated with mortality and intraventricular hemorrhage in neonates with respiratory failure?

OBJECTIVE: To describe differences in characteristics among neonates treated with extracorporeal life support (ECLS) in the first week of life for respiratory failure compared with later in the neonatal period and to assess risk factors for central nervous system (CNS) hemorrhage and mortality among the two groups. STUDY DESIGN: Review of the Extracorporeal Life Support Organization registry from 2001 to 2010 of neonates ⩽30 days comparing two age groups: those ⩽7 days (Group 1) to those >7 days (Group 2) at ECLS initiation. RESULT: Among 4888 neonates, Group 1 (n=4453) had significantly lower mortality (17 vs 39%, P<0.001) but greater CNS hemorrhage (11 vs 7%, P=0.02) than Group 2 (n=453). Mortality and CNS hemorrhage improved significantly with increasing gestational age only for Group 1 patients. CNS hemorrhage occurred more frequently in Group 1 patients receiving venoarterial (VA) than with venovenous ECLS (15 vs 7%, P<0.001). In Group 1, lower birth weight and pre-ECLS pH and VA mode were independently associated with mortality. In Group 2, higher mean airway pressure was independently associated with mortality. Complications of ECLS therapy, including CNS hemorrhage and renal replacement therapy were independently associated with mortality for both groups. CONCLUSION: Neonates cannulated for ECLS after the first week of life had greater mortality despite lower CNS hemorrhage than neonates receiving ECLS earlier. Premature infants cannulated after 1 week had fewer CNS hemorrhages than premature infants treated with extracorporeal membrane oxygenation starting within the first week of life.

Short-term outcome of neonates with congenital heart disease and diaphragmatic hernia treated with extracorporeal membrane oxygenation.

BACKGROUND: Major congenital heart disease (CHD) is seen in 10% to 15% of patients with congenital diaphragmatic hernia (CDH). Some neonates with CDH and major CHD are treated by surgery and some with extracorporeal membrane oxygenation (ECMO). Because of presumed poor survival, there is significant heterogeneity in management approaches for patients with CDH and associated CHD, and there is no published outcome data of patients who were placed on ECMO support. METHODS: To examine outcomes of children with CDH with CHD supported with ECMO, Extracorporeal Life Support Organization (ELSO) registry was queried from 1998 to May 2010. There were a total of 3,342 deployments for CDH and 316 (9.5%) neonates with associated CHD. The median values are as follows: age at presentation, 1 (0 to 52) days; gestational age, 38 (29 to 42) weeks; birth weight, 3 (1.35 to 4.7) kg; admission to time to ECMO, 16 (0 to 1,220) hours; duration of ECMO, 194 (3 to 823) hours; time off of ECMO to extubation, 366 (0 to 7,934) hours; and time off of ECMO to death was 114 (0 to 7,272) hours. RESULTS: Initially, ECMO support was venoarterial in 91% (283 of 316), 5 were converted from venovenous to venoarterial ECMO. Overall survival to hospital discharge for all patients with CDH and CHD was 47% (148 of 316). Survival to hospital discharge for hypoplastic left heart syndrome and single-ventricle physiology was 55% (33 of 60), 48% (43 of 89) for ventricular septal defect, and 40% (24 of 60) for coarctation of the aorta patients. CONCLUSIONS: Patients with CDH and CHD supported with ECMO have had better than predicted short-term outcomes. In this cohort, overall survival of patient's with CDH with or without CHD was similar. Patients with single-ventricle physiology had similar short-term outcome to those with 2 ventricle physiology.

Aspergillus infection and extracorporeal membrane oxygenation support.

BACKGROUND: The clinical characteristics of patients with Aspergillus isolation while supported on extracorporeal membrane oxygenator (ECMO) remain unclear. OBJECTIVES: We present a case report of angioinvasive Aspergillus infection on an infant supported on ECMO and also investigate outcomes among patients with Aspergillus infection reported to the Extracorporeal Life Support Organization (ELSO) registry. DESIGN: Case report and retrospective analysis of ELSO registry data set from 1985 to 2009. SETTING: One hundred and seventy ECMO centers contributing data to the ELSO registry. PATIENTS: Single case report and patients 0 to 90 years of age with Aspergillus infection requiring ECMO support as reported to the ELSO registry. METHODS: Besides presenting details of our institutional case, we compared clinical characteristics and outcomes between pediatric and adult patients with Aspergillus isolation. Risk factors for in-hospital mortality were investigated. Kaplan-Meier estimates for freedom from death on ECMO for pediatric and adult patients were investigated. MEASUREMENTS AND MAIN RESULTS: (a) we report a case with Aspergillus supported on ECMO, (b) the ELSO registry yielded 46 patients with 59% (n = 27) in the pediatric data set (≤20 years of age) and 41% (n = 19) in the adult data set (>20 years of age) with Aspergillus infection requiring ECMO support. Overall survival to hospital discharge was 30% (14/46) with 22% (6/27) in children as compared to 42% (8/18) in adults (P = .19). Table 1 shows a comparison of clinical characteristics between children and adults. The comparison between adults and pediatric groups differed significantly in age (P = .0001), more use of venoarterial ECMO in children (P = .028). The median age of pediatric group was 1.95 years (range 0-17.3 years) versus 30.2 years (range 22-60 years) among adults. CONCLUSIONS: Aspergillus infection/colonization is associated with a 70% overall mortality among patients supported on ECMO. The ELSO registry data confirms that Aspergillus infection among ECMO supported patients occurs often in hosts who do not have known immunodeficiencies. The case stresses the need for a high level of suspicion for Aspergillus infection in nonimproving lung disease in patients on ECMO support.

The changing demographics of neonatal extracorporeal membrane oxygenation patients reported to the Extracorporeal Life Support Organization (ELSO) Registry.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is an important treatment tool in the management of near-term and term neonates with severe hypoxemic respiratory failure. To better understand how health care for patients treated with ECMO has changed, we studied the demographic and treatment data reported to the Extracorporeal Life Support Organization (ELSO) registry from January 1, 1988, through January 1, 1998. METHODS: We used data stored in the ELSO registry and evaluated the changes in demographics, use of alternate therapies before ECMO, severity of illness, duration of ECMO therapy, and mortality over a 10-year period. All data on neonates reported between January 1, 1988, and January 1, 1998 were used. Verification checks were performed on all fields to eliminate nonsense outliers. We separated the neonates into 2 groups-those with and those without a congenital diaphragmatic hernia (CDH). All analyses were performed on the total group and each subgroup separately. Changes in continuous data were analyzed by year using analysis of variance. Year differences in categorical data were evaluated with chi(2) analysis. We also used the linear trend test and the Cochran-Armitage trend test to evaluate time-related changes. RESULTS: We reviewed 12 175 neonates. Over the decade, there were no changes in mean gestational age, gender, age at which ECMO was started, pH, or PaCO(2) just before ECMO. The proportion of neonates with CDH increased from 18% to 26%, while the proportion with respiratory distress syndrome decreased from 15% to 4%. Other diagnostic categories remained constant. The use of surfactant, high-frequency ventilation, and inhaled nitric oxide increased from 0% in 1988 to 36%, 46%, and 24%, respectively, in 1997. The mean peak pressure being used just before ECMO decreased (47 +/- 10 in 1988 to 39 +/- 12 in 1997), and the mean PaO(2)/FIO(2) ratio increased (38 +/- 23 in 1988 to 48 +/- 36 in 1997). The primary mode of ECMO remains venoarterial; however, the use of venovenous ECMO increased from 1% to 32% over the decade. Duration of ECMO treatment increased overall, and this trend was seen for patients with and without CDH (124 +/- 67 to 141 +/- 104 hours for the non-CDH group, 161 +/- 99 to 238 +/- 141 hours for the CDH group). The number of centers reporting neonatal data to the ELSO registry increased from 52 in 1988 to a peak of 100 in 1993. In 1997, 96 centers reported data to ELSO. The average number of neonatal patients reported from each site decreased from a peak of 18 in 1991 to 9 in 1997. Mortality increased from 18% to 22%; however, when corrected for the relative increase in neonates with CDH, this trend disappeared. Diagnoses-specific mortality rates remained constant. The occurrence of intracranial hemorrhage and/or infarct also stayed constant at 16%. CONCLUSIONS: The population of neonates treated with ECMO in 1997 was very different from patients treated in the 1980s and early 1990s. They were exposed to an ever-expanding group of new therapies, appeared to be healthier based on indices of gas exchange, and were cared for at centers that reported fewer cases per year.

Extracorporeal life support 1997.

Data from the annual international Extracorporeal Life Support Organization (ELSO) Registry Report for 1997 are presented. Over 17,000 patients treated with extracorporeal life support as submitted to the ELSO Registry are reported. Seventy-five percent (over 13,000) patients were cases of neonatal respiratory failure, with an 80% overall survival. The number of neonatal respiratory cases reported to the Registry has been decreasing for 5 years, whereas the number of pediatric respiratory, cardiac, and adult respiratory cases has been increasing. Cumulative survival in these categories is 53%, 42%, and 47% respectively. Survival in pediatric and adult respiratory failure cases supported with extracorporeal life support continues to improve. A description of the re-engineering of the ELSO registry, to include redesigning of the database structure and elements, electronic submission and validation of data, and Internet based submission and retrieval of data, is also provided.