Electrical and Echocardiographic Abnormalities in Sickle Cell Disease Followed up at the National Sickle Cell Disease Reference Centre (CNRD)

Objective: To study the electrical and echocardiographic aspects in patients with major sickle cell syndrome. Methods: This was a prospective descriptive cross-sectional study carried out at the National Reference Center for Sickle Cell Disease and the cardiology department of the National Hospital of Niamey over a period of 6 months (from September 2021 to February 2022). Results: We included 100 patients, including 53 women and 47 men, with a sex ratio of 0.88. The average age was 32.6 ± 12 years, with extremes ranging from 16 to 65 years. The SS phenotype represented 87%, the SC phenotype 11% and only 2% of S beta-thalassemia. The mean baseline hemoglobin level was 8.89 ± 1.6 g/dL. Electrically, we found QT elongation, left ventricular hypertrophy, sinus tachycardia, negative T waves, first-degree atrioventricular block, right ventricular hypertrophy and left atrial hypertrophy in respectively 28%, 15%, 14%, 11%, 4% and 3% of cases. Echocardiographic abnormalities were dominated by valve involvement in 64% of cases with 53% valve leakage, 4% mitral insufficiency, 5% aortic insufficiency, 2% moderate to severe tricuspid insufficiency; followed by dilation of the left atrium and left ventricle in 30% and 13% of cases respectively. Dilation of the right ventricle and right atrium was present in 3% and 4% of cases, respectively. 4 cases of pulmonary arterial hypertension and 4 cases of ventricular communication were observed. We also noted 1 case of systolic dysfunction, 1 case of hypokinetic dilated cardiomyopathy. The homozygous SS form was found to be the most severe. Conclusion: Sickle cell disease is a severe disease whose prognosis is worsened by cardiac involvement.