Extracorporeal membrane oxygenation outcomes in children with Williams syndrome: a review of the ELSO registry

Perfusion ◽  
2021 ◽  
pp. 026765912199929
Author(s):  
Amarilis Martin ◽  
Peter T Rycus ◽  
Ahmad Farooqi ◽  
John Dentel ◽  
Katherine Cashen

Introduction: Williams syndrome (WS) results from a microdeletion that usually involves the elastin gene, leading to generalized arteriopathy. Cardiovascular anomalies are seen in 80% of WS patients, including supravalvular aortic stenosis (SVAS), pulmonary artery stenosis (PAS), and pulmonary stenosis (PS). Sudden death associated with procedural sedation and in the perioperative period in WS children have been reported. This study aims to describe extracorporeal membrane oxygenation (ECMO) use in WS children, identify risk factors for hospital mortality of WS patients, and compare outcomes between WS children and non-WS children with SVAS, PAS, and PS. Methods: Children 0–18 years-old in the Extracorporeal Life Support Organization (ELSO) Registry with a primary or secondary diagnosis of WS, SVAS, PAS, or PAS were included. Results: Included were 50 WS children and 1222 non-WS children with similar cardiac diagnoses. ECMO use increased over time in both groups (p = 0.93), with most cases occurring in the current era. WS children were younger (p = 0.004), weighed less (p = 0.048), had a pulmonary indication for ECMO (50% vs 10%, p < 0.001), and were placed more on high frequency ventilation (p < 0.001) than non-WS patients. Despite reporting a respiratory indication, most (84%) WS patients were placed on VA-ECMO. There were no significant differences between the two groups in terms of pre-ECMO cardiac arrest, ECMO duration, or reason for ECMO discontinuation. Both groups had a mortality rate of 48% (p = 1.00). No risk factors for WS mortality were identified.

Perfusion ◽  
2016 ◽  
Vol 32 (2) ◽  
pp. 151-156 ◽  
Author(s):  
Katherine Cashen ◽  
Roland L Chu ◽  
Justin Klein ◽  
Peter T Rycus ◽  
John M Costello

Introduction: Pediatric patients with hemophagocytic lymphohistiocytosis (HLH) may develop refractory respiratory or cardiac failure that warrants consideration for extracorporeal membrane oxygenation (ECMO) support. The purposes of this study were to describe the use and outcomes of ECMO in pediatric HLH patients, to identify risk factors for hospital mortality and to compare their ECMO use and outcomes to the ECMO population as a whole. Methods: Pediatric patients (⩽ 18 years) with a diagnosis of HLH in the Extracorporeal Life Support Organization (ELSO) Registry were included. Results: Between 1983 and 2014, data for 30 children with HLH were available in the ELSO registry and all were included in this study. All cases occurred in the last decade. Of the 30 HLH patients, 24 (80%) had a respiratory indication for ECMO and six (20%) had a cardiac indication (of which 4 were E-CPR and 2 cardiac failure). Of the 24 respiratory ECMO patients, 63% were placed on VA ECMO. Compared with all pediatric patients in the ELSO registry during the study period (n=17,007), HLH patients had worse hospital survival (non-HLH 59% vs HLH 30%, p=0.001). In pediatric HLH patients, no pre-ECMO risk factors for mortality were identified. The development of a hemorrhagic complication on ECMO was associated with decreased mortality (p=0.01). Comparing HLH patients with respiratory failure to patients with other immune compromised conditions, the overall survival rate is similar (HLH 38% vs. non-HLH immune compromised 31%, p=0.64). Conclusions: HLH is an uncommon indication for ECMO and these patients have increased mortality compared to the overall pediatric ECMO population. These data should be factored into decision-making when considering ECMO for pediatric HLH patients.


2017 ◽  
Vol 34 (3) ◽  
pp. 259-264 ◽  
Author(s):  
Christopher L. Jenks ◽  
Ayesha Zia ◽  
Ramgopal Venkataraman ◽  
Lakshmi Raman

Objective: To evaluate risk factors for hemolysis in pediatric extracorporeal life support. Design: Retrospective, single-center study. Setting: Pediatric intensive care unit. Patients: Two hundred thirty-six children who received extracorporeal membrane oxygenation. Interventions: None. Measurements and Main Results: Risk factors for hemolysis were retrospectively analyzed from a single center in a total of 236 neonatal and pediatric patients who received extracorporeal membrane oxygenation support (ECMO). There was no difference in the incidence of hemolysis between centrifugal (127 patients) and roller head (109 patients) pump type or between venoarterial and venovenous ECMO. High hemoglobin (Hb) was found to be an independent risk factor for hemolysis in both pump types. The Hb level >12 g/dL was significant in the roller group and the Hb level >13 g/dL was significant in the centrifugal group for the development of hemolysis for the cumulative ECMO run. The presence of high Hb levels on any given day increased the risk of hemolysis for that day of the ECMO run regardless of ECMO pump type. Higher revolutions per minute (RPMs) and higher inlet pressures on any given day increased the risk for the development of hemolysis in the centrifugal pump. Lower inlet venous pressures and RPMs were not associated with hemolysis in the roller group. Conclusions: An Hb level greater than 13 g/dL was associated with an increased risk of hemolysis, and a high Hb on a given day was associated with a significantly higher risk of hemolysis on the same day. Higher RPMs and lower inlet venous pressures were associated with an increased risk of hemolysis in the centrifugal pump only.


2021 ◽  
pp. 089719002110212
Author(s):  
Kalynn A. Northam ◽  
Bobbie Nguyen ◽  
Sheh-Li Chen ◽  
Edward Sredzienski ◽  
Anthony Charles

Background: Anticoagulation monitoring practices vary during extracorporeal membrane oxygenation (ECMO). The Extracorporeal Life Support Organization describes that a multimodal approach is needed to overcome assay limitations and minimize complications. Objective: Compare activated clotting time (ACT) versus multimodal approach (activated partial thromboplastin time (aPTT)/anti-factor Xa) for unfractionated heparin (UFH) monitoring in adult ECMO patients. Methods: We conducted a single-center retrospective pre- (ACT) versus post-implementation (multimodal approach) study. The incidence of major bleeding and thrombosis, blood product and antithrombin III (ATIII) administration, and UFH infusion rates were compared. Results: Incidence of major bleeding (69.2% versus 62.2%, p = 0.345) and thrombosis (23% versus 14.9%, p = 0.369) was similar between groups. Median number of ATIII doses was reduced in the multimodal group (1.0 [IQR 0.0-2.0] versus 0.0 [0.0 -1.0], p = 0.007). The median UFH infusion rate was higher in the ACT group, but not significant (16.9 [IQR 9.6-22.4] versus 13 [IQR 9.6-15.4] units/kg/hr, p = 0.063). Fewer UFH infusion rate changes occurred prior to steady state in the multimodal group (0.9 [IQR 0.3 -1.7] versus 0.1 [IQR 0.0-0.2], p < 0.001). Conclusion: The incidence of major bleeding and thrombosis was similar between groups. Our multimodal monitoring protocol standardized UFH infusion administration and reduced ATIII administration.


2017 ◽  
Vol 07 (01) ◽  
pp. 007-013 ◽  
Author(s):  
Philip Bernard ◽  
Sean Skinner ◽  
Prasad Bhandary ◽  
Ana Ruzic ◽  
Matthew Bacon ◽  
...  

AbstractIn extracorporeal life support (ECLS), there are two main types of oxygenators in clinical use for neonates: polymethylpentene (PMP) hollow fiber and polypropylene (PP) hollow fiber. A retrospective study was performed on neonates (n = 44) who had undergone ECLS for noncardiac indications from 2009 to 2015. Between the two groups (PMP n = 21, PP n = 23), the PP oxygenators failed 91% of the time, whereas the PMP oxygenators failed 43% of the time (p < 0.05). Analysis suggests PMP oxygenators are less prone to failure than PP oxygenators, and they require fewer number of oxygenator changes during a neonatal ECLS.


2018 ◽  
Author(s):  
Julian Villar ◽  
Stephen Ruoss ◽  
Richard HA ◽  
Joe Hsu

Extracorporeal membrane oxygenation (ECMO), also known as extracorporeal life support, is the practice of using circulatory assist devices and a gas exchange system to maintain sufficient tissue oxygen delivery, supplementing pulmonary and/or cardiac function in patients whose native physiology is too severely altered to be successfully supported solely by conventional life support techniques (eg, mechanical ventilation and inotropic and vasopressor drugs). ECMO should be considered in patients who are at a high risk of death due to a potentially reversible etiology of cardiopulmonary collapse. Indications for ECMO can be broadly divided into profound respiratory failure and/or cardiogenic shock. The indications include acute respiratory distress syndrome, heart failure, postoperative cardiogenic shock, and as an adjunct to cardiopulmonary resuscitation in patients with cardiac arrest. ECMO is currently experiencing a renaissance, and familiarity with its concepts is important for all critical care practitioners. This review contains 8 figures, 8 tables and 34 references Key Words: complications, equipment, indications, management basics, outcomes


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