Extracorporeal membrane oxygenation outcomes in children with Williams syndrome: a review of the ELSO registry
| Autor: | Ahmad Farooqi, Amarilis Martin, Katherine Cashen, Peter Rycus, John Dentel |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Williams Syndrome
medicine.medical_specialty Adolescent medicine.medical_treatment 030204 cardiovascular system & hematology 03 medical and health sciences Extracorporeal Membrane Oxygenation 0302 clinical medicine Internal medicine medicine Extracorporeal membrane oxygenation Humans Radiology Nuclear Medicine and imaging Hospital Mortality Registries Child Retrospective Studies Advanced and Specialized Nursing biology business.industry Infant Newborn Infant General Medicine medicine.disease Child Preschool Cardiology biology.protein Williams syndrome Cardiology and Cardiovascular Medicine business Safety Research Elastin Supravalvular aortic stenosis 030217 neurology & neurosurgery |
| Zdroj: | Perfusion. 37:359-366 |
| ISSN: | 1477-111X 0267-6591 |
| DOI: | 10.1177/0267659121999299 |
| Popis: | 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 |
| Databáze: | OpenAIRE |
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