title: Patent foramen ovale in patients with sickle cell disease and stroke: case presentations and review of the literature.
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.
authors: Hematology Division, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA ; Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
authors: Sheila
authors: S
authors: Razdan
authors: John J
authors: JJ
authors: Strouse
authors: Rakhi
authors: R
authors: Naik
authors: Sophie
authors: S
authors: Lanzkron
authors: Victor
authors: V
authors: Urrutia
authors: Jon R
authors: JR
authors: Resar
authors: Linda M S
authors: LM
authors: Resar
references: Am J Med. 2003 Dec 15;115(9):721-8
references: 14693325
references: Am J Med. 1997 Sep;103(3):192-6
references: 9316551
references: N Engl J Med. 1972 Oct 26;287(17):846-9
references: 5071963
references: Hematol Oncol Clin North Am. 2004 Dec;18(6):1339-54, ix
references: 15511619
references: Br J Haematol. 2009 Aug;146(3):300-5
references: 19500105
references: Am J Hematol. 2009 Sep;84(9):548-52
references: 19623672
references: Am J Med. 2013 May;126(5):443-9
references: 23582935
references: J Sch Nurs. 2006 Dec;22(6):330-5
references: 17121443
references: Blood. 2002 May 1;99(9):3144-50
references: 11964276
references: Pediatr Neurol. 2009 Aug;41(2):124-6
references: 19589461
references: Curr Opin Cardiol. 2008 Nov;23(6):537-44
references: 18830067
references: Am J Obstet Gynecol. 2008 Aug;199(2):125.e1-5
references: 18533123
references: Blood. 1998 Jan 1;91(1):288-94
references: 9414296
references: Neurology. 2001 Apr 24;56(8):1109-11
references: 11320190
references: Neurology. 1998 Jun;50(6):1678-82
references: 9633710
references: Blood. 2012 Apr 19;119(16):3684-90
references: 22096242
references: Arch Neurol. 2001 Dec;58(12):2017-21
references: 11735775
references: J Am Coll Cardiol. 2006 Jan 17;47(2):440-5
references: 16412874
references: J Pediatr. 2006 Nov;149(5):710-2
references: 17095350
references: Neurology. 2000 Oct 24;55(8):1172-9
references: 11071496
references: J Am Coll Cardiol. 2007 Feb 20;49(7):797-802
references: 17306710
references: J Pediatr. 2002 Mar;140(3):348-54
references: 11953734
references: Expert Rev Hematol. 2011 Dec;4(6):597-606
references: 22077524
references: J Pediatr. 2001 Sep;139(3):391-7
references: 11562619
references: Blood. 2004 Jun 1;103(11):4023-7
references: 14764527
references: Catheter Cardiovasc Interv. 2007 Dec 1;70(7):999-1007
references: 18044736
references: Blood. 2011 Jan 20;117(3):772-9
references: 20940417
references: Am J Med. 2006 Oct;119(10):897.e7-11
references: 17000225
references: Radiology. 2003 Jul;228(1):216-25
references: 12775848
references: J Pediatr. 2010 Apr;156(4):645-50
references: 20022343
references: Neurology. 1998 Jul;51(1):169-76
references: 9674798