Ebsteins Anomaly: Anatomo-echocardiographic correlation

Login or register to view PDF.
Abstract

Abstract (provisional)

Objective
The aim of this investigation is to demonstrate that in Ebstein's Anomaly (EA) the right ventricle (RV) is affected in its three portions and to establish an anatomoechocardiographic correlation between the anatomic features and the equivalent echocardiographic images.

Methods
Thirty hearts with EA were studied. The alterations of each portions of the RV were described. Fifty adult patients with this anomaly were studied by echocardiography.

Results
Anatomy: All hearts had atrial situs solitus, 27 had concordant atrioventricular connection and 3 discordant, of these 2 had transposition of the great arteries (TGA) and one double outlet right ventricle (DORV). The degree of tricuspid valve (TV) displacement showed a spectrum from I to III. The inlet of the RV was markedly thin in 27. The trabecular portion had multiples muscular bands in all. The outlet portion was dilated in 20 and stenotic in 5. In 25 atrial septal defects were found. Echocardiography: All patients had atrial situs solitus, 42 with concordant atrioventricular connection and 8 with discordant, of these last patients 5 had TGA and 3 DORV. The degree of TV displacement varied from I to III. The inlet of RV was markedly thin in 42. The trabecular portion had muscular bands in 45. The outlet portion was dilated in 31 and stenotic in 11. In 30 atrial septal defects were found.

Conclusions
The EA affects the whole RV and the anatomoechocardiographic correlation provides an appropriate understanding of echocardiographic images in terms of a precise diagnosis, therapeutic decisions and prognosis.

Pages

Background
The characteristics of Ebstein™s anomaly of the tricuspid valve include tethering of the leaflets to the ventricular wall and redundancy and dysplasiaof the leaflets. The variable degrees of redundancy and dysplasia determine the displacement of the functional tricuspid opening toward the trabecular portion and outflow tract of the right ventricle 1-6. These alterations of the tricuspid valve have traditionally constituted the most conspicuous aspects of this congenital cardiac malformation 1,4,5. The histopathology of the right ventricular wall reveals a decrease or total absence of myocardial fibers in the inlet portion 5-7. The apical trabecular portion of the right ventricular free wall shows a characteristic pattern of anomalous muscular bands that connect the ventricular septum to the free wall 6. Moreover, in the infundibular portion of the ventricular outflow tract, myocardial fibers are diminished in number, making it very thin. Subpulmonary and pulmonary obstructions can also be present 6,7. In addition, 25% of patients with Ebstein™s anomaly also present with a Wolf-Parkinson-White pre-excitation syndrome 8.

The hemodynamic effects are related to the tricuspid regurgitation, the existence of an atrial septal defect and the degree of dysfunction of the atrialized portion of the right ventricle. Some cases have tricuspid stenosis owing to reduction of the functional valve opening secondary to fusion of the leaflets. In such patients an atrial septal defect is obligatory for survival 8./>/>/>

Pages

References
  1. Becker AE, Becker MJ, Edwards JE. Pathologic spectrum and dysplasia of the tricuspid valve: features in common with Ebstein´s malformation. Arch Pathol 1971;91:167-178.
  2. Lev M, Liberthson RR, Joseph RH, Seten CE, Kunshe RD, Echner FAO, Millar RA. The pathologic anatomy of Ebstein´s disease. Arch Pathol 1970;90:334-343.
  3. Anderson KR, Lie JT. Pathologic anatomy of Ebstein´s anomaly of the heart revisited. Am J Cardiol 1978;49:739-745.
  4. Anderson KR, Zuberbuhler JR, Anderson RH, Becker AE, Lie JT.Morphologic spectrum of Ebstein´s anomaly of the Herat. A review. MayoClin Proc 1979;54:174-180.
  5. Muñoz-Castellanos L, Barros W, García F, Salinas HC. Estudio patológico de la displasia y el adosamiento valvulares en la enfermedad de Ebstein. Arch Inst Cardiol Mex 1993;63:101-109.
  6. Kuri Nivón M, Muñoz Castellanos L, Salinas Sánchez C. Estudio morfológico de 23 corazones con anomalía de Ebstein. Arch Inst Cardiol Mex 1995;65:197-206.
  7. Anderson K. The right ventricular myocardium in Ebstein´s anomaly. Amorphometric histopathologic study. Mayo Clin Proc 1979;54:181-184.8.
  8. Attie F, Rosas M, Rijlaarsdam M, Buendía A, Zabal C, Kuri J, Granados N. The adult patient with Ebstein´s anomaly: Outcome in 72 unoperated patients. Medicine 2000;79: 27-36.
  9. Farooki ZQ, Henry JG, Green EW. Echocardiographic spectrum of Ebstein´s anomaly of the tricuspid valve. Circulation 1976;53:63-69.
  10. Shiina A, Seward JA, Edwards WD, Hagler DJ, Tajik AJ. Twodimensional echocardiographic spectrum of Ebstein´s anomaly: detailed anatomic assessment. J Am Coll Cardiol 1984;3:356-370.
  11. Tynan MJ, Becker AE, Macartney FJ, Jim─é┬®nez M, Shinebourne EA, Anderson RH. Nomenclatura and classification of congenital heart disease. Br Heart J 1979;41:544-553.
  12. Bialostozky D, Medrano G, Muñoz-Castellanos L, Contreras R. Vectocardiographic study and anatomic observations in 21 cases of Ebstein´s malformation of the tricuspid valve. Am J Cardiol 1972;33:354-361.
  13. VanMierop LHS, Alley RD, Kausel HW, Stranahan S. The anatomy and embryology of endocardial cushion defects. J Thorac cardiovascular Surg 192;43:71-83.
  14. Celermajer DS, Deanfield JE. Diseases of the tricuspid valve. In Paediatric Cardiology, Second Edition. Edited by Anderson RH, Baker EJ, MaCartney FJ, Rigby ML, Shinebourne EA, Tynan M. Churchill Livingstone, London, 2002;pp 1111-1133.15. Keith JD. Ebstein´s disease. In:
  15. Keith JD, Rowe RD, Vlod P (Eds). Heart disease in infancy and chilhood. 3rd edn. MacMillan New York 1978; pp 817-855.
  16. Rowe RD, Freedom RM, Mehrizi A, Bloom RK. The neonate with congenital heart disease. 2nd edn. Saunders Philadelphia 1981; pp 515-528.
  17. Shardland GK, Chita SK, Allan LD. Tricuspid valve dysplasia or displacement in intrauterine life. J Am Coll Cardiol 1991;17:944-949.
  18. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P, Somerville J, Deanfield. Ebstein´s anomaly: Presentation and outcome from fetus to adult. J Am Coll Cardiol 1994;23:170-
    176.
  19. Zuberbuhler JR, Allwork SP, Anderson RH. The spectrum of Ebstein´s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1979;77:202-211.
  20. Freedom RM. Discordant atrioventricular connection and congenitally corrected transposition. In Paediatric Cardiology. 2nd Edn. Edited by
    Anderson RH, Baker EJ, Macartney F, Rigby ML, Shinebourne EA, Tynan M, Churchill Livingstone 2002; pp 1321-1335.