Publication Date
12-1-2016
Journal
The Texas Heart Journal
DOI
10.14503/THIJ-15-5610
PMID
28100970
Publication Date(s)
December 2016
Language
English
PMCID
PMC5179156
PubMedCentral® Posted Date
12-1-2016
PubMedCentral® Full Text Version
Post-Print
Published Open-Access
yes
Keywords
Anemia, sickle cell/blood/complications/physiopathology/therapy; heart defects, congenital/complications/surgery; hemoglobin, sickle/analysis; hemoglobin SC disease/complications; hemoglobin SS disease/complications; infant, newborn; retrospective studies; stroke/etiology/prevention & control
Copyright
This work is licensed under a Creative Commons Attribution-NonCommercial-No Derivative Works 4.0 International License.
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.