Sickle cell disease and thalassemia: Significant causes of dilated cardiomyopathy, experience of Ibn Rochd university hospital-Casablanca

Abstract

Cardiovascular complications of hemoglobinopathiesare more and more common, including rhythm and conduction disorders, heart failure (HF), vascular disease and pulmonary hypertension. They constitute a prognostic factor. Study the associatuon between hemoglobinopathies and dilated cardiomyopathy. Prospective cross-sectional study conducted between May and October 2018, including patients > 14 years old, with sickle cell disease and/or thalassemia followed in the adult and pediatric hematology department at Ibn Rochd University Hospital-Casablanca-Morocco. All patients had a complete clinical examination with an electrocardiogram and a cardiac ultrasound. 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. Echocardiographic analysis revealed dilated LV in 27,5% of cases. The mean value of tele-diastolic-diameter of LV was 50,04 (± 6,94)mm, homozygous sickle cell patients had significantly a (57 ± 7,63 mm) and heterozygous sickle cell patients had a larger diameter compared to the other groupes (51,25 ± 5,50 mm) p = 0,008. The mean LVEF was 59.64%(± 4.09) and was impaired in one case with a LVEF of 48%. Mean LGS was -21.23%(± 3.19). SGL was impaired in 5 patients. The RV was dilated in 2 homozygous sickle cell patients with systolic dysfunction in one case.The left atrium(LA) was dilated in 26,7% of cases the average volume of LA was 42,88(± 16,14)ml/m2 with no statistic significant difference between the groupes. RA was dilated in 19,23% of cases. The dilation of the LV is related to the increase in its volume caused by cardiac output elevation, which is explained by the severity of anemia in these patients that can leads to HF.