Clinical trial NCT02394431: Added Value of Speckle Tracking in the Evaluation of Patients With Sickle Cell Disease
Official title: Added Value of Speckle Tracking in the Evaluation of Patients With Sickle Cell Disease
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Sickle Cell Disease
Keywords: Sickle Cell Disease
Echocardiography
Speckle tracking
Lead sponsor: Brugmann University Hospital
Sponsor class: OTHER
First posted: 2015-03-20
Enrollment: 62
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Sickle cell disease patients

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.


Eligibility criteria: Inclusion Criteria:

* All sickle cell disease patients

Exclusion Criteria:

* Insufficient echogenicity

Primary outcomes:
measure: Cardiac ejection fraction
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Cardiac diastolic function
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Cardiac tissular doppler
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Myocardiac performance index
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Global longitudinal strain
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: arterial pulmonary hypertension
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: left ventricular hypertrophy
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
Secondary outcomes:
measure: Biological parameters: hemoglobin levels
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Biological parameters: ferritin levels
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Biological parameters: red cells count
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Biological parameters: hematocrit levels
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Biological parameters: iron levels
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Clinical parameters: severity of the illness
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
measure: Clinical parameters: sanguine transfusion numbers
timeFrame: once per year, at the annual medical visit planned according to the standart of care for this pathology
Other outcomes:
Locations:
city: Brussels
country: Belgium
facility: CHU Brugmann
Study officials:
affiliation: CHU Brugmann
name: Marielle MORISSENS, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: 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.
citation: 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.
citation: 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.
citation: 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.
citation: 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.
citation: 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.