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

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...

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Main Authors: Sheila Razdan, John J. Strouse, Rakhi Naik, Sophie Lanzkron, Victor Urrutia, Jon R. Resar, Linda M. S. Resar
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2013/516705
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author Sheila Razdan
John J. Strouse
Rakhi Naik
Sophie Lanzkron
Victor Urrutia
Jon R. Resar
Linda M. S. Resar
author_facet Sheila Razdan
John J. Strouse
Rakhi Naik
Sophie Lanzkron
Victor Urrutia
Jon R. Resar
Linda M. S. Resar
author_sort Sheila Razdan
collection DOAJ
description 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.
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publishDate 2013-01-01
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series Case Reports in Hematology
spelling doaj-art-0f959932842e4feea39166217b0a7c4f2025-02-03T01:07:33ZengWileyCase Reports in Hematology2090-65602090-65792013-01-01201310.1155/2013/516705516705Patent Foramen Ovale in Patients with Sickle Cell Disease and Stroke: Case Presentations and Review of the LiteratureSheila Razdan0John J. Strouse1Rakhi Naik2Sophie Lanzkron3Victor Urrutia4Jon R. Resar5Linda M. S. Resar6Hematology Division, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USAHematology Division, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USAHematology Division, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USAHematology Division, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USANeurology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USACardiology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USAHematology Division, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USAAlthough 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.http://dx.doi.org/10.1155/2013/516705
spellingShingle Sheila Razdan
John J. Strouse
Rakhi Naik
Sophie Lanzkron
Victor Urrutia
Jon R. Resar
Linda M. S. Resar
Patent Foramen Ovale in Patients with Sickle Cell Disease and Stroke: Case Presentations and Review of the Literature
Case Reports in Hematology
title Patent Foramen Ovale in Patients with Sickle Cell Disease and Stroke: Case Presentations and Review of the Literature
title_full Patent Foramen Ovale in Patients with Sickle Cell Disease and Stroke: Case Presentations and Review of the Literature
title_fullStr Patent Foramen Ovale in Patients with Sickle Cell Disease and Stroke: Case Presentations and Review of the Literature
title_full_unstemmed Patent Foramen Ovale in Patients with Sickle Cell Disease and Stroke: Case Presentations and Review of the Literature
title_short Patent Foramen Ovale in Patients with Sickle Cell Disease and Stroke: Case Presentations and Review of the Literature
title_sort patent foramen ovale in patients with sickle cell disease and stroke case presentations and review of the literature
url http://dx.doi.org/10.1155/2013/516705
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