0147: Cardiac involvement in hemoglobin SC disease compared to homozygous sickle-cell anemia

Abstract

Hemoglobin SC (HbSC) disease and homozygous sickle-cell anemia (SCA) are the most frequent genotypes (accounting for respectively 25% and 70%) of sickle-cell disease. Although the SCA cardiac involvement was well studied, the cardiac remodeling associated to HbSC has never been specifically investigated. The aim of the study was to describe the HbSC cardiac remodeling versus SCA. Using a case-control design, 61 HbSC (mean age 31.3±10.0 years, 36 women) patients underwent a comprehensive echocardiography and were compared to 61 SCA patients in stable conditions. LV end diastolic volume index and LV ejection fraction were measured by Simpson method. LV mass index, left atrial volume index, septal E/e’ ratio, peak tricuspid regurgitation velocity (TRV) and cardiac index were also measured as recommended. All the parameters were the average of three measures. Both LV morphological and diastolic functional parameters differed dramatically between the two groups of patients (table, mean±SD). Moreover, the pulmonary artery systolic pressure as estimated by TRV was lower in HbSC patients. Cardiac remodeling is very different in HbSC compared to SCA. Clinical interpretation of echocardiography data should be adjusted to each variant of the disease. Moreover, this study is further evidence that the both genotypes of sickle-cell disease have different organ involvements and should not be pooled in clinical studies. Specific guidelines for follow up of HbSC patients should be established. Abstratct 0147 – Table SCA patients (n=61) HbSC patients (n=61) p value Heart rate (beats/min) 70±11 72±10 0.26 LV mass index (g/m²) 103±26 77±16 <0.0001 LV end diastolic volume index (ml/m²) 92±19 64±15 <0.0001 Cardiac index (L/min/m²) 4.2±1.0 3.1±0.6 <0.0001 LV ejection fraction (%) 60±5 61±6 0.88 Septal E/e’ ratio 10.9±2.4 7.2±2.1 <0.0001 Left atrial volume index (ml/m²) 51.1±11.9 33.8±7.5 <0.0001 Tricuspid regurgitation velocity (m/s) 2.57±0.26 2.26±0.23 <0.0001 Tricuspid regurgitation velocity >2.5m/s, n(%) 34 (56) 4 (7) <0.0001