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

NCT02394431 | Completed

• 1 other identifier: CHUB-Echo-Cardio
• Study Type: observational
• Target: 62
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Sickle Cell Disease

Keywords

Sickle Cell Disease; Echocardiography; Speckle tracking

Outcome Measures

Primary Outcomes (7)

• 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 Outcomes (7)

• 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

Study Arms (2)

Sickle cell disease patients

Sickle cell disease patients

Other: Cardiac echography; Other: Biological parameters; Other: Clinical parameters

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.

Other: Cardiac echography; Other: Biological parameters

Interventions

Cardiac echography OTHER

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.

Healthy patients; Sickle cell disease patients

Biological parameters OTHER

Hemoglobin levels, red cells, hematocrit, iron, ferritin

Healthy patients; Sickle cell disease patients

Clinical parameters OTHER

Blood transfusion number, severity of the sickle cell disease damage, evolution duration of the sickness

Sickle cell disease patients

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Sickle cell disease patients

You may qualify if:

• All sickle cell disease patients

You may not qualify if:

• Insufficient echogenicity

Sponsors & Collaborators

• Brugmann University Hospital: lead

Study Sites (1)

CHU Brugmann

Brussels, 1020, Belgium

Location

Related Publications (6)

• 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.

  PMID: 22563937 BACKGROUND

• Knight-Perry JE, de las Fuentes L, Waggoner AD, Hoffmann RG, Blinder MA, Davila-Roman 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.

  PMID: 21873028 BACKGROUND

• 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. doi: 10.1016/j.jacc.2006.09.038. Epub 2007 Jan 16.

  PMID: 17258093 BACKGROUND

• 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.

  PMID: 20658621 BACKGROUND

• 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.

  PMID: 22530942 BACKGROUND

• 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.

  PMID: 23663816 BACKGROUND

MeSH Terms

Conditions

Anemia, Sickle Cell

Condition Hierarchy (Ancestors)

Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Anemia; Hematologic Diseases; Hemic and Lymphatic Diseases; Hemoglobinopathies; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Marielle MORISSENS, MD

  CHU Brugmann

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Deputy Head of Clinic

Study Record Dates

• First Submitted: March 10, 2015
• First Posted: March 20, 2015
• Study Start: November 1, 2013
• Primary Completion: June 1, 2016
• Study Completion: June 1, 2016
• Last Updated: July 26, 2016

Record last verified: 2016-07

Locations

BE (1)