title: Subclinical left ventricular systolic impairment in steady state young adult patients with sickle-cell anemia.
abstract: Chronic volume overload in sickle-cell anemia (SCA) is associated with left ventricular (LV) enlargement and hypertrophy. The effect of the disease on LV systolic function remains debated. The aim of our study was to investigate LV systolic function in SCA patients using 2D speckle-tracking imaging. We compared 30 steady state asymptomatic adult SCA patients (17 women, mean age 24.7 ± 5.1 years) with 30 age and sex-matched healthy subjects (17 women, mean age 25.0 ± 4.9 years). In addition to conventional echocardiographic parameters including LV ejection fraction (LVEF) and LV mass index (LVMi), global longitudinal strain (GLS) and strain rate (GLSR) were measured. GLS (-17.9 ± 2.0 vs. -19.7 ± 2.5 %, p = 0.004) and GLSR (-0.92 ± 0.09 vs. -1.07 ± 0.17 s(-1), p < 0.0001) values were lower in SCA patients while LVEF values (60.1 ± 3.8 vs. 61.7 ± 4.7 %, p = 0.30) were not different. LVMi was increased in SCA patients (100.7 ± 23.5 vs. 72.4 ± 15.2 g/m(2), p = 0.0001) and GLSR was significantly lower in the subgroup of patients with LV hypertrophy (-0.88 ± 0.09 vs. -0.96 ± 0.08 s(-1), p = 0.02). In SCA patients LVMi was correlated to GLS (r = 0.58, p = 0.001) and GLSR (r = 0.45, p = 0.015) pleading in favor of a pathological LV remodeling. Asymptomatic SCA patients exhibited a subclinical alteration of LV systolic function. Myocardial dysfunction appears to be linked to the degree of LV hypertrophy. 2D speckle-tracking imaging might be useful for long-term follow-up and to study the natural course of LV dysfunction in SCA patients.
authors: Department of Cardiology, Institut de Cardiologie, Pitié-Salpêtrière Hospital (Groupe Hospitalier Pitié-Salpêtrière), Assistance Publique - Hôpitaux de Paris, 47-83, boulevard de l'Hôpital, 75651, Paris Cedex 13, France, nadjib.hammoudi@psl.aphp.fr.
authors: Nadjib
authors: N
authors: Hammoudi
authors: Dimitri
authors: D
authors: Arangalage
authors: Morad
authors: M
authors: Djebbar
authors: Katia Stankovic
authors: KS
authors: Stojanovic
authors: Magali
authors: M
authors: Charbonnier
authors: Richard
authors: R
authors: Isnard
authors: Robert
authors: R
authors: Girot
authors: Pierre-Louis
authors: PL
authors: Michel
authors: François
authors: F
authors: Lionnet
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis; D058070:Asymptomatic Diseases; D016022:Case-Control Studies; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D011237:Predictive Value of Tests; D012307:Risk Factors; D013318:Stroke Volume; D013599:Systole; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D016277:Ventricular Function, Left; D055815:Young Adult