Abstract:
:Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction. The surviving patient had developmental delays. On the basis of this series, we suggest mitigating hypoxemia, and thus the risk of stroke, in patients who have sickle cell disease and cyanotic congenital heart disease. Potential therapies include chronic blood transfusions, hydroxyurea, earlier surgical correction to reduce the duration of hypoxemia, and heart or bone marrow transplantation.
journal_name
Tex Heart Inst Jjournal_title
Texas Heart Institute journalauthors
Iannucci GJ,Adisa OA,Oster ME,McConnell M,Mahle WTdoi
10.14503/THIJ-15-5610subject
Has Abstractpub_date
2016-12-01 00:00:00pages
509-513issue
6eissn
0730-2347issn
1526-6702journal_volume
43pub_type
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