Ebstein Anomaly in an Adolescent: A ‘Miranda Warning’ against Blaming Sickle Cell Cardiomyopathy: A Case-Based Scholarly Update
Wala SI
Department of Paediatrics, University of Maiduguri, Teaching Hospital, Nigeria.
Abubakar IG
Department of Internal Medicine, Faculty of Clinical Sciences, College of Medical Sciences, University of Maiduguri, Nigeria.
Ibrahim BA
Department of Paediatrics, Faculty of Clinical Sciences, College of Medical Sciences, University of Health Sciences Azare, Nigeria.
Jelani NI
Department of Paediatrics, University of Maiduguri, Teaching Hospital, Nigeria.
Farouk AG. *
Department of Paediatrics, University of Maiduguri, Teaching Hospital, Nigeria and Department of Paediatrics, Faculty of Clinical Sciences, College of Medical Sciences, University of Maiduguri, Nigeria.
*Author to whom correspondence should be addressed.
Abstract
Background: Ebstein's anomaly (EA) is a rare congenital heart disease characterized by apical displacement of the tricuspid valve associated with atrialisation of the right ventricle. The defect arises from failure of the normal process by which the tricuspid valve is separated from the right ventricular myocardium. Most cases are diagnosed in childhood, but asymptomatic ones may remain undiagnosed until adulthood.
Case Summary: We present a rare case of EA diagnosed for the first time in a 13-year-old female sickle cell anaemia patient when she developed biventricular heart failure with severe tricuspid regurgitation and biventricular thrombi which was managed medically; however, patient died 17 days into admission.
Conclusion: The Ebstein anomaly can presents in adolescents for the first time and is usually associated with pericardial effusion and ventricular thrombi. Due to its similarity in presentation to sickle cell cardiomyopathy, it can be missed in sickle cell anaemia patients. Echocardiography can help unravel this diagnostic dilemma.
Keywords: Biventricular thrombi, congenital heart defects, diagnostic dilemma, ebstein’s anomaly, echocardiography