Cardiac manifestations of sickle cell anaemia in Sudanese children.

Sickle cell anaemia (SCA) is one of the commonest chronic hemolytic anaemias in the Sudan; it is a disease with high mortality and morbidity. This study was conducted aiming to observe the clinical pattern of cardiac abnormalities in children with sickle cell anaemia, and to assess the relationship between the cardiac abnormalities and the severity of the disease. The study was conducted in sickle cell disease clinic at Khartoum Children Emergency Hospital. The study group consisted of 289 patients with sickle cell anaemia, age range from 6 months to 18 years. Data were collected using a questionnaire which include full history, clinical examination findings, chest x-rays, and Electro-cardiography. Tachycardia, systolic murmurs, and cardiomegaly were detected in 28%, 61%, and 54% of patients with SCA respectively. Left ventricular dilatation was observed in 51% of the study group, while right ventricular dilatation was observed in 22% of the patients. Left and right atrial dilatations were observed in 16% and 6% of the patients respectively. Contractility, ejection fraction (EF) were found almost always normal in all study subjects. Chamber dilatations were not associated with any abnormality in Left ventricular functions. Hemglobin (Hb) levels correlated negatively with cardiomegaly. Left Ventricular End Diastolic Dimension (LVEDD) correlates negatively with Hb levels and positively with the severity index. Only four patients (1%) had abnormal valves. In conclusion, cardiac abnormalities in patients with SCA correlate with the age of the patients and the severity of the disease.