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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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References

Keith JD, Rowe RD, Vlad P. Heart disease in infancy and childhood. Macmillan, NewYork, 1958, p 314.
Fyler DC, Bernard WF. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980; 65: 375–461.Google Scholar
Uyan C, Yazici M, Uyan AP, Akdemir R, Imirzalioglu N, Dokumaci B. Ebstein's anomaly in siblings: an original observation. Int J Cardiovasc Imaging 2002; 18: 435–438.Google Scholar
Lo KS, Loventhal JP, Walton JA Jr. Familial Ebstein's anomaly. Cardiology 1979; 64: 246–255.Google Scholar
Johnson CD, Ortiz-Colom PM, Sainz de la Pena H, Barroso E. Ebstein's anomaly in a patient with Down's syndrome. Bol Asoc Med P R 1989; 81: 221–222.Google Scholar
Hannoush H, Tamim H, Younes H, et al. Patterns of congenital heart disease in unoperated adults: a 20-year experience in a developing country. Clin Cardiol 2004; 27: 236–240.Google Scholar
Correa-Villasenor A, McCarter R, Downing J, Ferencz C. White-black differences in cardiovascular malformations in infancy and socioeconomic factors. The Baltimore-Washington Infant Study Group. Am J Epidemiol 1991; 134: 393–402.Google Scholar
Shiina A, Seward JB, Edwards WD, Hagler DJ, Tajik AJ. Two dimensional Echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment. J Am Coll Cardiol 1984; 3: 356–370.Google Scholar
Khan IA, Cohen RA. Ebstein's anomaly of the tricuspid valve associated with congenital deafmutism. Int J Cardiol 1999; 70: 219–221.Google Scholar
Rusconi PG, Zuberbuhler JR, Anderson RH, Rigby ML. Morphologic-echocardiographic correlates of Ebstein's malformation. Eur Heart J 1991; 12: 784–790.Google Scholar
Leung MP, Baker EJ, Anderson RH, Zuberbuhler JR. Cineangiographic spectrum of Ebstein's malformation: its relevance to clinical presentation and outcome. J Am Coll Cardiol 1988; 11: 154–161.Google Scholar
Chauvaud S. Ebstein's malformation. Surgical treatment and results. Thorac Cardiovasc Surg 2000; 48: 220–223.Google Scholar