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Surgical palliation of univentricular heart disease in children with Down's syndrome: A systematic review

OBJECTIVES: No standard protocol is available for the management of children with Down's syndrome (DS) and a functional single ventricle. This review attempts to determine the outcomes of the single ventricular surgical palliation pathway in high-risk children with DS. METHODS: Several database...

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Detalles Bibliográficos
Autor principal: Khoshhal, Saad Q.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Taibah University 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6694996/
https://www.ncbi.nlm.nih.gov/pubmed/31435384
http://dx.doi.org/10.1016/j.jtumed.2018.10.006
Descripción
Sumario:OBJECTIVES: No standard protocol is available for the management of children with Down's syndrome (DS) and a functional single ventricle. This review attempts to determine the outcomes of the single ventricular surgical palliation pathway in high-risk children with DS. METHODS: Several databases were searched using the following MeSH terms: ‘Congenital heart disease’, ‘Atrioventricular septal defect’, ‘Balanced AVSD’, ‘Unbalanced AVSD’, ‘Down's syndrome’, ‘Univentricular repair’, ‘bidirectional Glenn procedure’, and ‘Fontan procedure’. A structured algorithm was used for the selection of studies for an in-depth analysis. RESULTS: There was no universal agreement on the best surgical approach for unbalanced atrioventricular septal defect in DS. The majority of paediatric cardiac surgeons did not recommend the complete Fontan procedure; conversely, the use of a Glenn shunt (superior cavopulmonary connection) was preferred. CONCLUSIONS: Careful assessment of the suitability for Fontan surgery, including the absence of elevated pulmonary vascular resistance, pulmonary arterial anatomy, and function of the dominant ventricle, is mandatory. A staged surgical procedure ending with complete Fontan repair provides acceptable medium-term results.