2016
DOI: 10.1111/jocs.12721
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Staged Single Ventricle Palliation and Homozygous Sickle Cell Disease

Abstract: Hypoxemia is a well-known trigger for precipitating a sickling crisis in patients with sickle cell disease. Patients undergoing staged single ventricle palliation have hypoxemia during the initial stages of the Fontan pathway. The successful completion of staged single ventricle palliation in a child with a combination of homozygous sickle cell disease and a single ventricle physiology that tolerate prolonged hypoxemia during earlier stages of Fontan pathway is described.

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Cited by 1 publication
(4 citation statements)
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“…One case report recommends against Foley catheter insertion, due to the risk of infection . Intraoperative laboratory monitoring including hemoglobin levels and hemolysis parameters is routinely used by some . Lower limb venous access should be avoided because of the ulceration risk, according to one report…”
Section: Resultsmentioning
confidence: 99%
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“…One case report recommends against Foley catheter insertion, due to the risk of infection . Intraoperative laboratory monitoring including hemoglobin levels and hemolysis parameters is routinely used by some . Lower limb venous access should be avoided because of the ulceration risk, according to one report…”
Section: Resultsmentioning
confidence: 99%
“…Strategies include avoiding vascular stasis with proper positioning, and swiftly reversing hypotension . Vasoconstricting amines or other similar drugs, which are theoretically associated with a risk of peripheral vascular stasis, should if possible not be among the first line approaches to hypotension according to many . Hydration to avoid hyperviscosity and dehydration is considered essential .…”
Section: Resultsmentioning
confidence: 99%
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