Dilated Cardiopathy Associated with Sickle Cell Disease in a 68 Years Old Female: An Emerging Complication in Sub-sahara Africa

Abstract

Sickle Cell Anemia (SCA) is an autosomal recessive disease caused by a point mutation in the hemoglobin beta gene found on chromosome 11p15.5 [1]. Specifically, it occurs when a single base from A to T in the codon for glutamic acid at position 6 is changed to valine of the beta globin and thus disrupts the tertiary structure and stability of the hemoglobin molecule [2]. Sickle hemoglobin is responsible for wide spectrum of disorders which vary with respect to severity of anemia, frequency of crises and duration of survival [3]. We present the case of a dilated cardiopathy in an elderly female sickle cell patient. A 68 years old female sickle cell patient with no known major cardiovascular risk factor presented with progressive onset of dyspnea. Clinical examination showed signs of left ventricular failure; an electrocardiogram showed a sinus regular rhythm, left ventricular hypertrophy with systolic overload, Q waves in the anteroseptal leads. Cardiac ultrasound showed a dilated cardiopathy with a preserved left ventricular systolic function at 57%, normal regional wall motion and normal pulmonary pressure. We concluded of heart failure due to probable chronic anemia or anischemic cardiopathy with a conserved systolic function in an elderly sickle cell patient but we were not able to confirm the main etiology without CT Coronary Angiogram or coronarography. Sickle cell anemia is a common genetic condition in sub-Saharan Africa associated with early death. This case is special because we have an elderly female presenting with heart failure on a dilated cardiopathy. This enhances the necessity of strict cardiovascular follow up of Sickle cell patient.