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

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.

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