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

Overview

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.

Study Type

  • Study Type: Observational
  • Study Design
    • Time Perspective: Prospective
  • Study Primary Completion Date: June 2016

Interventions

  • Other: Cardiac echography
    • 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.
  • Other: Biological parameters
    • Hemoglobin levels, red cells, hematocrit, iron, ferritin
  • Other: Clinical parameters
    • Blood transfusion number, severity of the sickle cell disease damage, evolution duration of the sickness

Arms, Groups and Cohorts

  • Sickle cell disease patients
    • Sickle cell disease patients
  • Healthy patients
    • 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.

Clinical Trial Outcome Measures

Primary Measures

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

Secondary Measures

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

Participating in This Clinical Trial

Inclusion Criteria

  • All sickle cell disease patients

Exclusion Criteria

  • Insufficient echogenicity

Gender Eligibility: All

Minimum Age: 18 Years

Maximum Age: N/A

Are Healthy Volunteers Accepted: No

Investigator Details

  • Lead Sponsor
    • Brugmann University Hospital
  • Provider of Information About this Clinical Study
    • Principal Investigator: Dr Marielle Morissens, Deputy Head of Clinic – Brugmann University Hospital
  • Overall Official(s)
    • Marielle MORISSENS, MD, Principal Investigator, CHU Brugmann

References

Ahmad H, Gayat E, Yodwut C, Abduch MC, Patel AR, Weinert L, Desai A, Tsang W, Garcia JG, Lang RM, Mor-Avi V. Evaluation of myocardial deformation in patients with sickle cell disease and preserved ejection fraction using three-dimensional speckle tracking echocardiography. Echocardiography. 2012 Sep;29(8):962-9. doi: 10.1111/j.1540-8175.2012.01710.x. Epub 2012 May 8.

Knight-Perry JE, de Las Fuentes L, Waggoner AD, Hoffmann RG, Blinder MA, Dávila-Román VG, Field JJ. Abnormalities in cardiac structure and function in adults with sickle cell disease are not associated with pulmonary hypertension. J Am Soc Echocardiogr. 2011 Nov;24(11):1285-90. doi: 10.1016/j.echo.2011.07.009. Epub 2011 Aug 27.

Sachdev V, Machado RF, Shizukuda Y, Rao YN, Sidenko S, Ernst I, St Peter M, Coles WA, Rosing DR, Blackwelder WC, Castro O, Kato GJ, Gladwin MT. Diastolic dysfunction is an independent risk factor for death in patients with sickle cell disease. J Am Coll Cardiol. 2007 Jan 30;49(4):472-9. Epub 2007 Jan 16.

Hankins JS, McCarville MB, Hillenbrand CM, Loeffler RB, Ware RE, Song R, Smeltzer MP, Joshi V. Ventricular diastolic dysfunction in sickle cell anemia is common but not associated with myocardial iron deposition. Pediatr Blood Cancer. 2010 Sep;55(3):495-500. doi: 10.1002/pbc.22587.

Voskaridou E, Christoulas D, Terpos E. Sickle-cell disease and the heart: review of the current literature. Br J Haematol. 2012 Jun;157(6):664-73. doi: 10.1111/j.1365-2141.2012.09143.x. Epub 2012 Apr 25. Review.

Poludasu S, Ramkissoon K, Salciccioli L, Kamran H, Lazar JM. Left ventricular systolic function in sickle cell anemia: a meta-analysis. J Card Fail. 2013 May;19(5):333-41. doi: 10.1016/j.cardfail.2013.03.009. Review.

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