Stroke and presence of patent foramen ovale in sickle cell disease

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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Successful Patent Foramen Ovale Closure in a Patient with Pulmonary Arterial Hypertension of Sickle Cell Disease

10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a3631 ◽

2019 ◽

Author(s):

C. Cramer-Bour ◽

E.S. Klings

Keyword(s):

Pulmonary Arterial Hypertension ◽

Sickle Cell Disease ◽

Arterial Hypertension ◽

Sickle Cell ◽

Patent Foramen Ovale ◽

Foramen Ovale ◽

Cell Disease ◽

Pulmonary Arterial

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Unexplained Hypoxia and Shunting on Echocardiography in Patients with Sickle Cell Disease: A Retrospective Review

Blood ◽

10.1182/blood.v128.22.3673.3673 ◽

2016 ◽

Vol 128 (22) ◽

pp. 3673-3673

Author(s):

Payal C. Desai ◽

Nicole Kendel ◽

Melanie Heinlein ◽

Ying Huang ◽

Eric H. Kraut ◽

...

Keyword(s):

Pulmonary Embolism ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Patent Foramen Ovale ◽

Acute Chest Syndrome ◽

Foramen Ovale ◽

Cell Disease ◽

Intrapulmonary Shunting ◽

Pulmonary Shunting ◽

Jet Velocity

Abstract Introduction: During an episode of vaso-occlusive crisis, some patients with sickle cell disease may develop a transient hypoxemia. While frequently attributed to acute chest syndrome or pulmonary embolism, clinically a portion of patients develop hypoxemia without a clear etiology. On echocardiography, some patients were noted to have Patent Foramen Ovale (PFO) and others were noted to have intrapulmonary shunting. We sought to further characterize this clinical finding in sickle cell patients. Methods: We conducted a single institution retrospective chart review from 2008 through 2015 to evaluate the incidence of pulmonary shunting and PFOs in patients with SCD, as demonstrated on an echocardiography. We further characterize each of these episodes with clinical and laboratory findings at the time of the event. The presence of absence of shunting and type of shunting was further verified by a single cardiologist reviewing all episodes. Kruskal-Wallis test or Fisher's exact test was used to compare characteristics between patients with intracardiac or intrapulmonary shunting. Results: A total of 36 (18 female (F), 18 male (M)) of the 352 (10%) patients seen at the Ohio State Comprehensive Sickle Cell Center were noted to have shunting on their echocardiogram reported over the 7 year time period. Independent review by cardiology confirmed the presence of a shunt in 32 patients (9%, 95% CI: 6-13%) (15F, 17M). The median age at the time of reporting was 29 (range: 18-51 yrs). Shunting was observed in patients of all genotypes (94% SS/SB0; 6% SC/SBeta+). Fourteen (6 F, 8 M) of the thirty-two (44%, 95% CI: 26-62%) patients under study were noted to have cardiac shunting and eighteen (9 F, 9 M) (56%, 95% CI: 38-74%) were noted to have pulmonary shunting. In the patients with cardiac shunting, all 14/14 were confirmed to have a PFO by independent cardiac review. At the time of echo, 27/32 (87%) were hospitalized and 19/32 (59%) had clinical hypoxia on the day of the echocardiography. Patients with cardiac shunting tended to have higher proportion of hypoxia compared with patients with pulmonary shunting (71% vs. 50%). 19/32 (59%) had concurrent CT angiography and 1/32 (5%) patients was confirmed to have a pulmonary embolism. This patient demonstrated a concurrent PFO. 9 (28%) had a concurrent diagnosis of acute chest syndrome (4 with cardiac shunting and 5 with pulmonary shunting). The median value of TR jet velocity at baseline was 2.64 (range: 2.0-4.0) (n=18) and the median TR jet velocity at the time of event was 2.67 m/s (range: 2.0-3.8 m/s) (n=27). In the cardiac shunting group, the TR jet velocity was 2.85 m/s (range: 2.0 -3.27m/s) and in the pulmonary shunting group, the TR jet velocity was 2.6 m/s (range: 2.2-3.8 m/s). Conclusion: Patients with SCD presenting with increasing hypoxia may have intracardiac or intrapulmonary shunting. A patent foramen ovale that opens during vaso-ossclusive crisis may cause transient hypoxemia. This opening is thought to be due to increasing pulmonary pressures. While the long term implications of this finding are currently being studied, intracardiac shunting should be considered in the differential of patients with SCD presenting with increasing hypoxia of unclear etiology. Disclosures No relevant conflicts of interest to declare.

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Patent Foramen Ovale in Adult Patients with Sickle Cell Disease and Stroke

Blood ◽

10.1182/blood.v124.21.4084.4084 ◽

2014 ◽

Vol 124 (21) ◽

pp. 4084-4084

Author(s):

Sheila Razdan ◽

Anusha Reddy ◽

Danielle F. Resar ◽

John J. Strouse ◽

Rani Hasan ◽

...

Keyword(s):

Risk Factors ◽

Left Atrium ◽

Sickle Cell ◽

Patent Foramen Ovale ◽

Invasive Procedure ◽

Adult Patients ◽

Foramen Ovale ◽

Minimally Invasive Procedure ◽

Cell Disease ◽

Grade 3

Abstract Introduction: Stroke is a leading cause of death in adults with sickle cell disease (SCD), although little is known about the underlying pathophysiology or associated risk factors. A patent foramen ovale (PFO) or other intracardiac shunts are associated with ischemic stroke in children with SCD and young adults without SCD. PFOs are relatively common, with a prevalence of about 24% in the general population. Importantly, PFOs can be closed using a minimally invasive procedure, which could potentially prevent embolic strokes in individuals with SCD at risk for stroke. We therefore designed an epidemiological study to determine the prevalence of PFOs in adults with SCD and stroke. Methods: To identify SCD patients with stroke who had evidence for a PFO by echocardiagraph, we retrospectively reviewed charts from the Johns Hopkins Hospital. All adult patients (>18 years old) with the diagnosis of stroke followed in our Sickle Cell Program were included. PFO was diagnosed by conventional echocardiogram, color Doppler studies, and contrast studies with agitated saline (10 mls) through a peripheral IV or central venous catheter with and without a Valsalva maneuver (2 times each) to increase the sensitivity of detecting intracardiac shunting. The presence of bubbles in the left atrium, either spontaneously or after Valsalva, within five cardiac cycles was noted. PFOs or other intracardiac shunts were graded by the appearance of bubbles in the left atrium, including: Grade 1 (mild shunt with 1-10 bubbles), Grade 2 (moderate shunt with 10-25 bubbles and a distinct portion filling the left atrium), and Grade 3 (substantial shunt with >25 bubbles and complete filling of the left atrium). Brain MRI/MRAs were classified by stroke subtype and severity of cerebral vasculopathy. Age at the time of the study and stroke, gender, hemoglobinopathy, presence of a peripheral deep venous thrombosis (DVT), and evidence for other stroke risk factors (moyamoya or cerebral aneurysms) were ascertained by chart review. The study was approved by the IRB at our institution. Results: From this group of 65 adult patients with a sickling hemoglobinopathy (hemoglobin SS, SC, or S beta thalassemia) and stroke, we identified 15 patients who underwent an evaluation for a PFO or other intracardiac shunt (Table 1). The prevalence of PFO or other intracardiac shunt in these adults with SCD and a history of stroke was 40% (6/15). Of these 6 patients with SCD, PFO and stroke, 1 had hemoglobin SC and 5 had hemoglobin SS. Three of the PFOs were Grade 1 and 3 were Grade 3. Three patients with PFO and stroke also had a DVT at the time of the stroke; 3 patients with PFO and stroke also had Moyamoya. Conclusions: The high prevalence of PFOs in adults with SCD and stroke compared to the prevalence of PFOs in the general population suggest that PFOs could be a risk factor for stroke in this population. These findings also underscore the urgent need for further research to establish the role of PFOs in stroke in adults with SCD because PFOs can be closed with a minimally invasive procedure. Disclosures Naik: NHLBI: Research Funding.

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