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Case Reports in Hematology
Volume 2013, Article ID 516705, 5 pages
http://dx.doi.org/10.1155/2013/516705
Case Report

Patent Foramen Ovale in Patients with Sickle Cell Disease and Stroke: Case Presentations and Review of the Literature

1Hematology Division, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
2Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
3Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
4Neurology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
5Cardiology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
6Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
7Institute for Cellular Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA

Received 10 April 2013; Accepted 26 May 2013

Academic Editors: G. Feher, M. Singh, and S. D. Wagner

Copyright © 2013 Sheila Razdan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Although individuals with sickle cell disease (SCD) are at increased risk for stroke, the underlying pathophysiology is incompletely understood. Intracardiac shunting via a patent foramen ovale (PFO) is associated with cryptogenic stroke in individuals without SCD. Recent evidence suggests that PFOs are associated with stroke in children with SCD, although the role of PFOs in adults with stroke and SCD is unknown. Here, we report 2 young adults with SCD, stroke, and PFOs. The first patient had hemoglobin SC and presented with a transient ischemic attack and a subsequent ischemic stroke. There was no evidence of cerebral vascular disease on imaging studies and the PFO was closed. The second patient had hemoglobin SS and two acute ischemic strokes. She had cerebral vascular disease with moyamoya in addition to a peripheral deep venous thrombosis (DVT). Chronic transfusion therapy was recommended, and the DVT was managed with warfarin. The PFO was not closed, and the patients' neurologic symptoms were stabilized. We review the literature on PFOs and stroke in SCD. Our cases and the literature review illustrate the dire need for further research to evaluate PFO as a potential risk factor for stroke in adults with SCD.