An Uncommon Association of Ebstein’s Anomaly and Acute Rhumatic Fever

Abstract

Background: Ebstein’s anomaly is a relatively rare congenital heart disease (atrialisation of the right ventricle). Anomalies of left side of the heart have been described with this condition. We however present a case of Ebstein's anomaly with recurrent acute rheumatic fever (ARF) and mitral stenosis. Case report: A 13-year-old boy with not followed for heart disease and with a history of notion of repetitive angina, is admitted to the pediatric emergency room for fever and polyarthritis. General examination not showed subcutaneous nodules and laboratory investigations revealed leucocytosis, elevated erythrocyte sedimentation rate (ESR) and antistreptolysin O (ASO) titre. Echocardiography revealed Ebstein anomaly with moderate mitral stenosis. A diagnosis of recurrent ARF was made based on the carditis, arthritis, history of recurrence of sore throat and evidence of recent streptococcal infection. He was transferred to the cardiology department and initially put on medical treatment. Its clinical course was marked by the appearance of unresolved ventricular tachycardia despite drug cardioversion. His prognosis was unfavorable with death after 10 days of hospitalization under antiarrhythmic treatment. Conclusion: Ebstein's disease is a rare disease. Cardiac arrhythmias are deleterious and the association of rheumatic fever with co-morbidity increases its lethality.