Added Value of Speckle Tracking in the Evaluation of Patients With Sickle Cell Disease

Study Overview
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Brief Summary
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Sickle Cell Disease is a serious disease that is life-threatening for patients being homozygous for the SS form or heterozygous for the SC or βthal forms. The CHU Brugmann hospital currently regularly treats about 70 homozygous adult patients and this number is in constant augmentation. Sickle cell disease patients may develop a cardiomyopathy due to chronic anemia, the haemosiderosis risk or, less frequently, to coronary vaso-occlusive damages. The hypervolemia in patients with sickle cell disease causes an overestimation of the ejected left ventricular fraction measured by echocardiography, this parameter being very dependent of the blood volume.It has already been shown that the left ventricular ejection fraction was normal in most patients with sickle cell disease, but that its evaluation by parameters independent from the blood volume showed the existence of a dysfunction. Myocardial strain, as measured by speckle tracking, is a echographic evaluation method of the cardiac function, independent of the blood volume. This technique hasn't been used much in sickle cell disease patients. A study using 3D speckle tracking on a limited number of sickle cell disease patients failed to show a strain anomaly. Moreover, the study highlighted a higher global longitudinal strain in this patient population. The investigators find these data hard to explain and in contradiction with previous studies using other cardiac function evaluation techniques, independent from the blood volume. The primary goal of this study is thus to study the longitudinal strain by 2D echography to determine if anomalies of the longitudinal strain exist in sickle cell disease patients with a normal ejected left ventricular fraction, compared to a control group of healthy patients. The secondary goal of this study is to correlate, inside the sickle cell disease group, the possible strain anomalies with biological gravity parameters of the disease.

Official Title
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Added Value of Speckle Tracking in the Evaluation of Patients With Sickle Cell Disease

Conditions
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Sickle Cell Disease

Intervention / Treatment
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* Other: Cardiac echography
* Other: Biological parameters
* Other: Clinical parameters


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: All sickle cell disease patients Exclusion Criteria: Insufficient echogenicity

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Sickle cell disease patients<br>Sickle cell disease patients | Other: Cardiac echography<br>* Ejection fraction measured by Teicholz and planimetry, diastolic function, tissular doppler, myocardiac performance index, global longitudinal strain measured by speckle tracking, arterial pulmonary hypertension, left ventricular hypertrophy.<br>Other: Biological parameters<br>* Hemoglobin levels, red cells, hematocrit, iron, ferritin<br>Other: Clinical parameters<br>* Blood transfusion number, severity of the sickle cell disease damage, evolution duration of the sickness<br>|
| Healthy patients<br>This is the control group for the sickle cell disease patients: each sickle cell disease patient will be matched with a healthy patient of the same sex and of similar age. | Other: Cardiac echography<br>* Ejection fraction measured by Teicholz and planimetry, diastolic function, tissular doppler, myocardiac performance index, global longitudinal strain measured by speckle tracking, arterial pulmonary hypertension, left ventricular hypertrophy.<br>Other: Biological parameters<br>* Hemoglobin levels, red cells, hematocrit, iron, ferritin<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Cardiac ejection fraction | Ejection fraction measured by Teicholz and planimety. | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Cardiac diastolic function |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Cardiac tissular doppler |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Myocardiac performance index |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Global longitudinal strain | Global longitudinal strain measured by speckle tracking. | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| arterial pulmonary hypertension |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| left ventricular hypertrophy |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Biological parameters: hemoglobin levels |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Biological parameters: ferritin levels |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Biological parameters: red cells count |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Biological parameters: hematocrit levels |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Biological parameters: iron levels |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Clinical parameters: severity of the illness | Sickle cell disease organ damages. | once per year, at the annual medical visit planned according to the standart of care for this pathology | 
| Clinical parameters: sanguine transfusion numbers |  | once per year, at the annual medical visit planned according to the standart of care for this pathology | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Sickle Cell Disease, Echocardiography, Speckle tracking