Main Article Content
Acute rheumatic fever in a child with Ebstein anomaly
Abstract
Background: Ebstein anomaly (atrialisation of the right ventricle ) alone constitutes 0.5 -1% of congenital heart disease and usually presents as an isolated lesion. Association with mitral valve stenosis has been reported previously. We however present a case of Ebstein's anomaly with recurrent acute rheumatic fever (ARF) and mitral and aortic valve disease.
Case report: An 8-year-old girl with Ebstein anomaly presented to a Cardiac hospital in India with fever, polyarthritis and a recent history of sore throat. General examination showed subcutaneous nodules and investigations revealed leucocytosis, elevated erythrocyte sedimentation rate (ESR) and antistreptolysin O (ASO) titre. Echocardiography confirmed Ebstein anomaly with mitral and aortic regurgitation. A diagnosis of recurrent ARF was made based on the carditis, arthritis, subcutaneous nodules and evidence of recent streptococcal infection and a history of recurrence of symptoms in the past year. She was promptly commenced on oral penicillin, steroids, secondary prophylaxis with benzathine penicillin and aspirin. She responded to complete bed rest and these therapy; symptoms abated before discharge 7 days later. She was doing well on monthly follow-up in the past 4 months.
Conclusion: This report supports the need to investigate non cardiac symptoms such as arthritis in children with existing congenital heart disease because co-morbidity with ARF can exacerbate the symptoms, signs and prognosis of a congenital heart disease, if left untreated.
