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Clinical and echocardiographic features of Ebstein's malformation in Sudanese patients

Published online by Cambridge University Press:  22 March 2006

Sulafa K. M. Ali  and
Nuha A. G. M. Nimeri
Sulafa K. M. Ali
Affiliation:
Department of Paediatric Cardiology, Sudan Heart Centre, Khartoum, Sudan
Nuha A. G. M. Nimeri
Affiliation:
Department of Paediatric Cardiology, Sudan Heart Centre, Khartoum, Sudan
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Abstract

Ebstein's malformation is a rare congenital cardiac malformation, accounting for about 0.5% of all congenital cardiac lesions. We report our experience with the anomaly as encountered at the Sudan Heart Centre from July 2004 to April 2005. Diagnosis was based on the echocardiographic demonstration of displacement of the septal leaflet of the tricuspid valve towards the ventricular apex by greater than 8 millimetres per metre squared. Results: In a period of 10 months, we identified 12 patients with the malformation, this number constituting 2% of all patients seen with congenital cardiac disease. The age ranged from 2 weeks to 35 years. Of the patients, half were asymptomatic, while the other half presented with congestive cardiac failure, 4 of these having cyanosis in addition to heart failure. Associated diseases included chronic renal failure, impaired hearing, stunted growth, and developmental delay. Electrocardiographic abnormalities included peaked and tall P waves, seen in four-fifths, an Rsr pattern, first degree atrioventricular block, atrial fibrillation, and Wolf–Parkinson–White syndrome, including the Mahaim pattern of pre-excitation. Associated abnormalities diagnosed echocardiographically included atrial septal defects, prolapse of the leaflets of the mitral valve, left ventricular dysfunction, atrial septal aneurysm, pulmonary valvar stenosis, and pericardial effusion. Conclusions: Ebstein's malformation was seen four times more frequently at the Sudan Heart Centre when compared to the average frequency reported in the Western literature. Many of the associated diseases encountered in Sudan had not previously been reported.

Keywords

Tricuspid valveincidencecongenital heart disease
Type
Original Article
Information
Cardiology in the Young , Volume 16 , Issue 2 , April 2006 , pp. 147 - 151
Copyright
© 2006 Cambridge University Press

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