DOPPLER ULTRASOUND RELIABLY IDENTIFIES SICKLE CELL DISEASE ADULT PATIENTS WITH INTRACRANIAL VASCULOPATHY
(Abstract release date: 05/19/16)
EHA Library. Bartolucci P. 06/11/16; 135219; S463
Prof. Pablo Bartolucci
Contributions
Contributions
Abstract
Abstract: S463
Type: Oral Presentation
Presentation during EHA21: On Saturday, June 11, 2016 from 11:45 - 12:00
Location: Hall C14
Background
Stroke is one of the leading causes of death in both children and adults with sickle-cell disease (SCD) most often related to a specific large-vessel vasculopathy. Prevention of stroke is based on blood exchange transfusion if stenosant vasculopathy is confirmed. However magnetic resonance angiography or angio scanner are not easily available in many AFrican countries instead of Doppler ultrasound which is the main diagnosis strategy in SCD children. Doppler ultrasound has never been demontrated to be effecient in SCD adults.
Aims
The objective of this study was to assess whether Doppler ultrasound is sensitive and specific to identify SCD adult patients with vasculopathy, compared with magnetic resonance angiography.
Methods
80 adult SCD patients likely to have brain attempt were referred to our center for vasculopathy screening. 77 patients could benefit from our protocol including both a 3T brain MR imaging with a 3D time-of-flight MR angiography and a Doppler-Ultrasound examination performed on the same day. 3 patients presented contraindication to MRI.On MR imaging, patients were categorized according to the grade of the highest stenosis and to the presence of Moya-Moya. On EchoDoppler-Utrasound examination, time-averaged mean of the maximum velocity (TAMMx) was measured and a TAMMx ratio was established using the highest TAMMx value recorded on one axis, divided by the TAMMx value of the ipsilateral extracranial internal carotid artery. Presence of Moya-Moya was also recorded.
Results
Among the 38 patients with an intracranial vasculopathy (stenosis >=50% on MR imaging, presence of an occlusion or Moya-Moya), 33 had a severe vasculopathy according to EchoDoppler criteria defined as a TAMMx ratio >=3 (n=5) or presenting an occlusion (n=11) or a Moya-Moya (n=17). Sensitivity and specificity of EchoDoppler to identify patients with >=50% vasculopathy on MRA is respectively of 87%, 90%. Positive and negative predictive values are respectively of 86% and 87%.
Conclusion
Doppler ultrasound may be used to identify SCD adult patients with vasculopathy.
Session topic: Red blood cells and iron - Clinical
Keyword(s): Sickle cell disease, Stroke
Type: Oral Presentation
Presentation during EHA21: On Saturday, June 11, 2016 from 11:45 - 12:00
Location: Hall C14
Background
Stroke is one of the leading causes of death in both children and adults with sickle-cell disease (SCD) most often related to a specific large-vessel vasculopathy. Prevention of stroke is based on blood exchange transfusion if stenosant vasculopathy is confirmed. However magnetic resonance angiography or angio scanner are not easily available in many AFrican countries instead of Doppler ultrasound which is the main diagnosis strategy in SCD children. Doppler ultrasound has never been demontrated to be effecient in SCD adults.
Aims
The objective of this study was to assess whether Doppler ultrasound is sensitive and specific to identify SCD adult patients with vasculopathy, compared with magnetic resonance angiography.
Methods
80 adult SCD patients likely to have brain attempt were referred to our center for vasculopathy screening. 77 patients could benefit from our protocol including both a 3T brain MR imaging with a 3D time-of-flight MR angiography and a Doppler-Ultrasound examination performed on the same day. 3 patients presented contraindication to MRI.On MR imaging, patients were categorized according to the grade of the highest stenosis and to the presence of Moya-Moya. On EchoDoppler-Utrasound examination, time-averaged mean of the maximum velocity (TAMMx) was measured and a TAMMx ratio was established using the highest TAMMx value recorded on one axis, divided by the TAMMx value of the ipsilateral extracranial internal carotid artery. Presence of Moya-Moya was also recorded.
Results
Among the 38 patients with an intracranial vasculopathy (stenosis >=50% on MR imaging, presence of an occlusion or Moya-Moya), 33 had a severe vasculopathy according to EchoDoppler criteria defined as a TAMMx ratio >=3 (n=5) or presenting an occlusion (n=11) or a Moya-Moya (n=17). Sensitivity and specificity of EchoDoppler to identify patients with >=50% vasculopathy on MRA is respectively of 87%, 90%. Positive and negative predictive values are respectively of 86% and 87%.
Conclusion
Doppler ultrasound may be used to identify SCD adult patients with vasculopathy.
Session topic: Red blood cells and iron - Clinical
Keyword(s): Sickle cell disease, Stroke
Abstract: S463
Type: Oral Presentation
Presentation during EHA21: On Saturday, June 11, 2016 from 11:45 - 12:00
Location: Hall C14
Background
Stroke is one of the leading causes of death in both children and adults with sickle-cell disease (SCD) most often related to a specific large-vessel vasculopathy. Prevention of stroke is based on blood exchange transfusion if stenosant vasculopathy is confirmed. However magnetic resonance angiography or angio scanner are not easily available in many AFrican countries instead of Doppler ultrasound which is the main diagnosis strategy in SCD children. Doppler ultrasound has never been demontrated to be effecient in SCD adults.
Aims
The objective of this study was to assess whether Doppler ultrasound is sensitive and specific to identify SCD adult patients with vasculopathy, compared with magnetic resonance angiography.
Methods
80 adult SCD patients likely to have brain attempt were referred to our center for vasculopathy screening. 77 patients could benefit from our protocol including both a 3T brain MR imaging with a 3D time-of-flight MR angiography and a Doppler-Ultrasound examination performed on the same day. 3 patients presented contraindication to MRI.On MR imaging, patients were categorized according to the grade of the highest stenosis and to the presence of Moya-Moya. On EchoDoppler-Utrasound examination, time-averaged mean of the maximum velocity (TAMMx) was measured and a TAMMx ratio was established using the highest TAMMx value recorded on one axis, divided by the TAMMx value of the ipsilateral extracranial internal carotid artery. Presence of Moya-Moya was also recorded.
Results
Among the 38 patients with an intracranial vasculopathy (stenosis >=50% on MR imaging, presence of an occlusion or Moya-Moya), 33 had a severe vasculopathy according to EchoDoppler criteria defined as a TAMMx ratio >=3 (n=5) or presenting an occlusion (n=11) or a Moya-Moya (n=17). Sensitivity and specificity of EchoDoppler to identify patients with >=50% vasculopathy on MRA is respectively of 87%, 90%. Positive and negative predictive values are respectively of 86% and 87%.
Conclusion
Doppler ultrasound may be used to identify SCD adult patients with vasculopathy.
Session topic: Red blood cells and iron - Clinical
Keyword(s): Sickle cell disease, Stroke
Type: Oral Presentation
Presentation during EHA21: On Saturday, June 11, 2016 from 11:45 - 12:00
Location: Hall C14
Background
Stroke is one of the leading causes of death in both children and adults with sickle-cell disease (SCD) most often related to a specific large-vessel vasculopathy. Prevention of stroke is based on blood exchange transfusion if stenosant vasculopathy is confirmed. However magnetic resonance angiography or angio scanner are not easily available in many AFrican countries instead of Doppler ultrasound which is the main diagnosis strategy in SCD children. Doppler ultrasound has never been demontrated to be effecient in SCD adults.
Aims
The objective of this study was to assess whether Doppler ultrasound is sensitive and specific to identify SCD adult patients with vasculopathy, compared with magnetic resonance angiography.
Methods
80 adult SCD patients likely to have brain attempt were referred to our center for vasculopathy screening. 77 patients could benefit from our protocol including both a 3T brain MR imaging with a 3D time-of-flight MR angiography and a Doppler-Ultrasound examination performed on the same day. 3 patients presented contraindication to MRI.On MR imaging, patients were categorized according to the grade of the highest stenosis and to the presence of Moya-Moya. On EchoDoppler-Utrasound examination, time-averaged mean of the maximum velocity (TAMMx) was measured and a TAMMx ratio was established using the highest TAMMx value recorded on one axis, divided by the TAMMx value of the ipsilateral extracranial internal carotid artery. Presence of Moya-Moya was also recorded.
Results
Among the 38 patients with an intracranial vasculopathy (stenosis >=50% on MR imaging, presence of an occlusion or Moya-Moya), 33 had a severe vasculopathy according to EchoDoppler criteria defined as a TAMMx ratio >=3 (n=5) or presenting an occlusion (n=11) or a Moya-Moya (n=17). Sensitivity and specificity of EchoDoppler to identify patients with >=50% vasculopathy on MRA is respectively of 87%, 90%. Positive and negative predictive values are respectively of 86% and 87%.
Conclusion
Doppler ultrasound may be used to identify SCD adult patients with vasculopathy.
Session topic: Red blood cells and iron - Clinical
Keyword(s): Sickle cell disease, Stroke
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