TITLE:
Electrical and Echocardiographic Abnormalities in Sickle Cell Disease Followed up at the National Sickle Cell Disease Reference Centre (CNRD)
AUTHORS:
Idrissa Hama, Maman Ali Rahamatou, Djibrilla Almoustapha Amadou, Dodo Boubacar, Maliki Abdoulaye Moctar, Beidari Ali, Harouna Habibou, Mbaye Seck, Hamidou Laouan, Bonkano Ali, Akilou Abdouramane, Malam Abdou Badé, Touré Ibrahim Ali
KEYWORDS:
Electrocardiogram, Echocardiography, Anomalies, Sickle Cell Diseases and CNRD
JOURNAL NAME:
Open Journal of Blood Diseases,
Vol.15 No.2,
June
30,
2025
ABSTRACT: 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.