Abstract P582: Accuracy of Transcranial Doppler in Detection of Intracranial Stenosis in Patients With Sickle Cell Disease

Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Nastajjia Krementz ◽  
Hannah Gardener ◽  
Luis F Torres ◽  
Moayd Alkhalifah ◽  
Iszet Campo-Bustillo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Predictive Value ◽  
Hemoglobin Level ◽  
Intracranial Stenosis ◽  
Cell Disease ◽  
Mean Flow ◽  
Vessel Stenosis ◽  
Flow Velocities

Background: Stroke is among the most devastating consequences of sickle cell disease (SCD). Most SCD strokes occur due to intracranial stenosis, that can be detected by increased flow velocities on Transcranial Doppler (TCD). However, increased velocities may also reflect anemia rather than arteriopathy and vessel stenosis. We aimed to assess the accuracy of TCD in detection of intracranial stenosis in SCD. Methods: Pediatric SCD patients with at least one TCD and MRA within one month apart were identified from a retrospectively collected database maintained at our institution from January 2000 to December 2016. Patient demographics, hemoglobin level, transfusion and hydroxyurea status were collected, along with mean flow velocities (mFV) and degree of stenosis from bilateral middle and anterior cerebral, and internal carotid arteries. A mFV of > 200 cm/s and vessel stenosis > 50% were considered abnormal. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were obtained to assess overall accuracy of TCD velocities in relation to vessel stenosis. Multivariate analysis was performed to identify independent factors associated with MRA stenosis. Results: A total of 164 patients were included in the database (median age 12 [IQR 8.9] years, 56% had ischemic strokes) and 64 of them had at least one TCD and MRA one month apart. Of these, 20% had ischemic strokes, 17% had MRA stenosis > 50% and 10% had TCD velocity > 200 cm/s. TCD mFV > 200 cm/s had a high specificity (95%) and NPV (87%) but low sensitivity (29%) and PPV (55%) when compared to MRA stenosis > 50%. As a continuous variable, TCD mFV 137.5 cm/s had the best balance between maximal specificity (77%) and sensitivity (72%). After adjustment for age, hemoglobin level, transfusion status, hydroxyurea, and vessel, for every increase in cm/sec on TCD, there was a 2% increase in the odds of > 50% stenosis on MRA (OR=1.02, 95% CI 1.01-1.03, p<0.001). Conclusion: Our study reports that TCD mFV is a positive predictor of MRA stenosis in SCD, independent of patient characteristics, including hemoglobin. A mFV > 200 cm/s is highly specific but less sensitive to detect stenosis > 50%. Lower mFV cut points may need to be considered for early detection of intracranial stenosis and risk of stroke.

scholarly journals Patients with sickle cell disease are frequently excluded from the benefits of transcranial doppler screening for the risk of stroke despite extensive and compelling evidence

2017 ◽  
Vol 75 (1) ◽  
pp. 15-19 ◽  
Author(s):  
Daniela Laranja Gomes Rodrigues ◽  
Samuel Ademola Adegoke ◽  
Rejane de Souza Macedo Campos ◽  
Josefina Aparecida Pellegrini Braga ◽  
Maria Stella Figueiredo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Stroke Prevention ◽  
Cell Disease ◽  
Mean Flow ◽  
Medical Centers ◽  
Left And Right ◽  
First Time ◽  
Missed Opportunity

ABSTRACT Transcranial doppler (TCD) is a strategic component of primary stroke prevention in children with sickle cell disease (SCD). This study was conducted to examine the TCD characteristics of children with SCD in nine different medical centers in Brazil. Methods: Transcranial doppler was performed in accordance with the Stroke Prevention Trial in Sickle Cell Anemia Protocol. Results: Of the 396 patients, 69.5% had homozygous SS hemoglobin. The TCD result was abnormal in 4.8%, conditional in 12.6%, inadequate in 4.3% and abnormally low in 1% of patients. The highest mean flow velocities were 121±23.83cm/s and 124±27.21cm/s in the left and right middle cerebral artery respectively. A total of 28.8% patients (mean age 9.19±5.92 years) were evaluated with TCD for the first time. Conclusions: The SCD patients were evaluated with TCD at an older age, representing an important missed opportunity for stroke prevention. Since TCD screening in patients with SCD is important to detect those at high risk for stroke, it is recommended that this screening should be made more readily available.

scholarly journals Transcranial Doppler Study Among Children with Sickle Cell Anaemia Vs Normal Children

2012 ◽  
Vol 32 (2) ◽  
pp. 146-149 ◽  
Author(s):  
BB Lakhkar ◽  
BN Lakhkar ◽  
P Vaswani
Keyword(s):  
Sickle Cell Disease ◽  
Flow Velocity ◽  
Sickle Cell ◽  
Cerebral Artery ◽  
Transcranial Doppler ◽  
Cell Disease ◽  
Mean Flow ◽  
Mean Velocity ◽  
Mean Flow Velocity ◽  
Age And Sex

Introduction: Role of transcranial Doppler in prevention of stroke in sickle cell children has been well appreciated. Studies are being done to develop the protocol in children. Since we don’t find stroke very commonly in this part of the world, this study was done in order to see the prevalence of abnormal flow velocity in sickle children attending sickle cell clinic. The aims of this study were to measure mean flow velocity in different vessels in homozygous sickle cell patients using transcranial Doppler study, to compare the mean velocity in sickle children with age and sex matched controls and to correlate mean velocity with headache or stroke if any and also to correlate mean velocity with number of transfusions. Materials and Methods: The study was done in Paediatric wards. It was a prospective crosssectional comparative study. Twenty six children below 14yrs of age with homozygous sickle cell disease attending the Sickle cell clinic were selected as the cases. Forty cases of similar age and sex were recruited as normal control group. Transcranial Doppler was done in six different vessels in both the groups and mean flow velocity was measured. Mean flow velocity was correlated with symptoms and number of transfusions. Velocity was classified as normal (<170cm/sec), conditional (170-199cm/sec) or abnormal (>200cm/sec). Statistical analysis was done using SPSS 10 software. Results: In normal age and sex matched controls mean blood flow velocity was 50cm/sec where as in the cases of sickle cell disease was 180cm/sec. Maximum mean velocity was observed in middle and posterior cerebral artery. In two Sickle cell cases (8%) blood flow velocity was abnormal, these children had headache though received 5-10 transfusions/year. In only 4% sickle cell children flow velocity was normal and rest had conditional velocity. Among these children 39% received less than 5 and rest received 5-9 transfusions /yr and had no symptoms of stroke. Conclusions: Flow velocity measured by Transcranial Doppler is highest in middle cerebral artery and Posterior cerebral artery which appear to be the best arteries for this test in this region. Flow velocity was significantly high in children with sickle cell disease as compared to normal children. Prevalence of abnormal flow velocity in our children was 8% and children with abnormal mean flow velocity presented with headache. J. Nepal Paediatr. Soc. 32(2) 2012 146-149 doi: http://dx.doi.org/10.3126/jnps.v32i2.5681

First Report on Elevated Transcranial Doppler Ultrasound Velocities As First Stroke Risk and Their Association with Hemoglobin F Expression in Saudi Arabian Pediatric Sickle Cell Patients

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3665-3665
Author(s):  
Khalid Al Jamaan ◽  
Saleh Al Oraibi ◽  
Giuseppe Saglio ◽  
Mudassar Iqbal ◽  
Zafar Iqbal
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Stroke Risk ◽  
Protective Role ◽  
Cell Disease ◽  
Hemoglobin F ◽  
Female Ratio ◽  
Flow Velocities

Abstract Introduction: Sickle cell disease (SCD) is one of the most common inherited hemoglobinopathies in Middle East and specifically Saudi Arabia which leads to vaso-occlusive, hematological and infectious crises, including stroke as one of the common complications (1,2). Primary prevention of the first stroke is carried out by risk assessment using transcranial Doppler (TCD) ultrasound and providing blood transfusions to SCD patients with higher than 199 cm/sec TCD velocities (3). Furthermore, althoughhigher HbF expression is also associated with less severity of the SCD, no studies are available about association of HbF with TCD flow velocities and hence risk of stroke in SCD (4). This study has been carried out to identify SCD patients at high risk of stroke based on TCD results, and to determine the association between Hb-F levels and blood velocity using TCD results. Patients and Methods: Cross-sectional study included 56 pediatric SCD patients. TCD ultrasounds were performed at diagnosis for assessment of first stroke risk and then after every 6 months for follow-up studies. Patients with elevated TCD velocities given monthly packed RBCs transfusions to have HbS level around 40% (5,6). Data analysis was performed by "Statistical Package for the Social Sciences (SPSS, Version 17.0)". Results: In our patient population, male to female ratio was 1.33:1 (32 males, 24 females) and mean age was 7.5 ± 3.42 years (range: 2-14 years). Seven (12.5%) patients had TCD velocities higher than or equal to 200 cm/second, with mean age of 4.7±1.4 years and mean HbF 7.5±3.8, which were significantly different from patient group with normal flow velocities (mean age of 7.8±3.4 years; mean HbF 20±15.9). Patients with higher TCD velocities receiving blood transfusion got velocities in normal range within 6-12 months. Most importantly, normal TCD flow velocities were significantly associated with higher HbF expression and vice versa. As protective role of HbF in sickle cell patients is well established (11;13) and inducing the HbF expression in such patients using medications like hydroxyurea (20) or by genetically modifying the silencing of fetal γ-globin gene have been long standing goal in treatment of sickle cell disease (21), our findings about correlation of higher TCD flow velocities with lower HbF expression (and vice versa) and hence higher risk of first stroke in sickle cell patients provides a new direction for assessing the risk for vasculopathy in SCD patients. Discussion/Conclusions : Low prevalence of SCD patients with higher TCD velocities in our study population is in accordance with previous studies (5). Risk of first stroke was clinically manageable by 6-12 months of regular PRBCs transfusions, per established clinical practice (6). Moreover, normal TCD flow velocities and hence lower risk of stroke was significantly associated with the higher hemoglobin F expression and vice versa. As protective role of HbF in sickle cell patients is well established (4) and inducing the HbF expression in such patients have been long standing goal in treatment of sickle cell disease (7), our findings about correlation of higher TCD flow velocities with lower HbF expression (and vice versa) and hence higher risk of first stroke in sickle cell patients provides a new direction for assessing the risk for vasculopathy in SCD patients. References: 1. Kato GJ. Curr Opin Hematol. 2016 May;23(3):224-32. 2. Ahmed AE, et al. Health Qual Life Outcomes. 2015 Nov 16;13:183. 3. Lawrence C, Webb J. Curr Neurol Neurosci Rep. 2016 Mar;16(3):27. 4. Liu L, et al. Exp Biol Med (Maywood). 2016 Apr;241(7):706-18. 5. Adams R, McKie V, Hsu L, et al. N Engl J Med. 1998;339: 5-11. 6. Bernaudin F, et al. Blood. 2016 Apr7;127(14):1814-22. 7. Pule GD, et al. Clin Transl Med. 2016 Mar;5(1):15. doi: 10.1186/s40169-016-0092-7. Disclosures Saglio: Novartis: Consultancy, Honoraria; BMS: Consultancy, Honoraria; Ariad: Consultancy, Honoraria; Pfizer: Consultancy, Honoraria; Roche: Consultancy, Honoraria.

Changes in Transcranial Doppler Flow Velocities in Children with Sickle Cell Disease: The Impact of Hydroxyurea Therapy

2018 ◽  
Vol 27 (2) ◽  
pp. 425-431 ◽  
Author(s):  
Samuel Ademola Adegoke ◽  
Rejane de Souza Macedo-Campos ◽  
Josefina Aparecida Pellegrini Braga ◽  
Maria Stella Figueiredo ◽  
Gisele Sampaio Silva
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Cell Disease ◽  
Doppler Flow ◽  
The Impact ◽  
Hydroxyurea Therapy ◽  
Flow Velocities

Abstract 65: Anemia-related Heart Failure in Sub-saharan African Sickle Cell Disease Patients

2017 ◽  
Vol 121 (suppl_1) ◽  
Author(s):  
Adebayo C Atanda ◽  
Yahya Aliyu ◽  
Oluwafunmilayo Atanda ◽  
Aliyu Babadoko ◽  
Aisha Suleiman ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Hemoglobin Level ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Jet Velocity ◽  
Sub Saharan

Introduction: Anemia has been implicated in heart failure. Existing literatures, involving predominantly African-Americans, suggests that Sickle Cell Disease (SCD) maybe linked to various cardiovascular complications including pulmonary hypertension and left venticular dysfunction. Peculiarly, our study involves exclusively Sub-Saharan population. Method: We conducted a cross sectional observational study of 208 hydroxyurea-naive consecutive SCD patients aged 10-52 years at steady state and 94 healthy non-matched controls who were studied in an out patient clinic in Sub-Saharan Africa. SCD patients were required to have electrophoretic or liquid chromatography documentation of major sickling phenotypes. Control group was required to have non-sickling phenotypes. Cardiac measurements were performed with TransThoracic Echo according to American Society of Echocardiography guidelines. Hemoglobin level was also obtained. Results: Hemoglobin level in SCD group (8.5+/- 1.5) was significant (P<0.001) compared to control (13.8+/- 1.7). Although SCD group had significantly higher values of left ventricular (LV) size, there was no qualitative evidence of LV dysfunction. SCD group had higher values of Ejection Fraction but not statistically significant. There was no evidence of LV wall stiffening to impair proper filling in SCD group, with the ratio of early to late ventricular filling velocities, E/A ratio elevated (1.7+/-0.4 compared to 1.6+/- 0.4; P=0.010). Right ventricular systolic pressure was determined using the formula of 4x Tricuspid Reugurgitant jet (TRV) square as an indirect measurement of Pulmonary arterial systolic pressure. SCD patients had significantly higher mean±SD values for tricuspid regurgitant jet velocity than did the controls (2.1±0.6 vs. 1.8±0.5; p= 0.001). Within the SCD group, there was no clear pattern of worsening diastolic function with increased TRV. Furthermore, E/A had a significant positive relationship with jet velocity in bivariate analysis (R=0.20; P=0.013). Conclusions: We were unable to demonstrate existence of anemia-associated left ventricular dysfunction in Sub-Saharan African with SCD. Further studies is required to highlight the reason behind this finding.

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