Diastolic dysfunction in sickle cell disease and thallassamia at Ibn Rochd University hospital

Introduction Cardiovascular complications of hemoglobinopathies are more and more frequent and therefore constitute a prognostic factor. Purpose Assess the impact of hemoglobinopathies on left ventricular diastolic function Methodes Prospective cross-sectional study conducted between May and October 2018, including patients above 14 years old, with sickle cell disease and/or thalassemia followed in the adult and pediatric hematology department at IbnRochd University Hospital-Casablanca-Morocco. Diastolic function was studies using TDI at the mitral annulus, Pulsed wave Doppler of the mitral valve, left atrial (LA) volume, and Velocity of tricuspid regurgitation (Vmax TR). Evaluation of left ventricular filling pressure were decided on the basis of recommandations of ASE/EACVI 2016. Results 52 patients were included, the average age was 24,19 ± 9,55 years. Females accounted for 49.1% of patients. 30.2% of patients had homozygous thalassemia, 24.5% had heterozygous thalassemia, 22.6% had homozygous sickle cell disease, 17% had heterozygous sickle cell disease, and 5.7% had sickle cell disease and thalassemia. median of ferritine was 3128 [411; 10124]. Echocardiographic analysis of diastolic function revealed an average value of the E/A ratio of 1.57 ± 0.46 [0.7; 3]. The mitral profile was normal in 76% of our patients, restrictive in 20%, relaxation abnormality in 2% and uninterpretable in 2% of patients that had atrial fibrillation. Average volume of LA was 42.88 ± 16.15 ml/m2. LA was dilated in 36.5%. Vmax TR was >2.8 m/s in 17,30%. LV filling pressure were considered elevated in 2 patients ( Fig. 1 ). Conclusion Systolic and diastolic heart dysfunction remains a common complication of hemoglobinopathies; The progressive dilation of the left ventricle leads to an increase in its mass and consequently an alteration in diastolic function.This diastolic heart failure can also be explained by damage to the vascular microcirculation and iron overload.