Ebstein's Anomaly in Adults: Doppler-Echocardiographic Evaluation

A systematic approach and segmental analysis are required for comprehensive assessment including both morphologic and functional abnormalities associated with Ebstein's anomaly. The essence of the disease is an apical displacement of both the septal and the posterior tricuspid leaflets, exceeding 20 mm or 8 mm/m² in adults. As a consequence, the right heart consists of three components including the true right atrium, the functional right ventricle (RV) and an intervening zone that is anatomically ventricular but functionally right atrial (atrialized RV). The thin wall of the atrialized RV may result in an aneurysm between the anatomic tricuspid annulus and the apically displaced posterior leaflet. The annular attachment of the anterior leaflet is normal, which may be dysplastic and adherent to the RV wall. Tricuspid regurgitation is usually moderate to severe. Size, shape and function of the functional RV must be described. The paradoxical motion of the interventricular septum causes alterations in left ventricular geometry and function. An interatrial communication is frequently present. Echocardiography is the method of choice to diagnose Ebstein's anomaly on its own or in association with other heart defects.

Key words:

Ebstein's anomaly - Doppler-echocardiography - evaluation

References

• 1 Ebstein W. Über einen sehr seltenen Fall von Insuffizienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Arch Anat Physiol Wissensch Med 1866: 238-254
• 2 Celermajer D S, Dodd S M, Greenwald S E, Wyse R K, Deanfield J E. Morbid anatomy in neonates with Ebstein's anomaly of the tricuspid valve: pathophysiologic and clinical implications.  J Am Coll Cardiol. 1992;  19 1049-1053
• 3 Leung M P, Baker E J, Anderson R H, Zuberbuhler J R. Cineangiographic spectrum of Ebstein's malformation: its relevance to clinical presentation and outcome.  J Am Coll Cardiol. 1988;  11 154-161
• 4 Rusconi P G, Zuberbuhler J R, Anderson R H, Rigby M L. Morphologic-echocardiographic correlates of Ebstein's malformation.  Eur Heart J. 1991;  12 784-790
• 5 Zuberbuhler J R, Becker A E, Anderson R H, Lenox C C. Ebstein's malformation and the embryological development of the tricuspid valve. With a note on the nature of “clefts” in the atrioventricular valves.  Pediatr Cardiol. 1984;  5 289-295
• 6 Roberson D A, Silverman N H. Ebstein's anomaly: echocardiographic and clinical features in the fetus and neonate.  J Am Coll Cardiol. 1989;  14 1300-1307
• 7 Zuberbuhler J R, Allwork S P, Anderson R H. The spectrum of Ebstein's anomaly of the tricuspid valve.  J Thorac Cardiovasc Surg. 1979;  77 202-211
• 8 Anderson K R, Lie J T. Pathologic anatomy of Ebstein's anomaly of the heart revisited.  Am J Cardiol. 1978;  41 739-745
• 9 Anderson K R, Zuberbuhler J R, Anderson R H, Becker A E, Lie J T. Morphologic spectrum of Ebstein's anomaly of the heart: a review.  Mayo Clin Proc. 1979;  54 174-180
• 10 Freedom R M, Mawson J B, Yoo S J, Benson L N. Ebstein's malformation of the tricuspid valve. In: Freedom RM, Mawson JB, Yoo SJ, Benson LN, eds. Congenital Heart Disease: Textbook of Angiocardiography, First ed. Armonk, NY; Futura Publishing Company, Inc. 1997: 349-366
• 11 Perloff J K. Ebstein's anomaly of the tricuspid valve. In: Perloff JK, ed. The clinical recognition of congenital heart disease. Philadelphia; W. B. Saunders Company 1994: 247-272
• 12 Hauck A J, Freeman D P, Ackermann D M, Danielson G K, Edwards W D. Surgical pathology of the tricuspid valve: a study of 363 cases spanning 25 years.  Mayo Clin Proc. 1988;  63 851-863
• 13 Shiina A, Seward J B, Edwards W D, Hagler D J, Tajik A J. Two-dimensional echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment.  J Am Coll Cardiol. 1984;  3 356-370
• 14 Gussenhoven E J, Stewart P A, Becker A E, Essed C E, Ligtvoet K M, De V V. “Offsetting” of the septal tricuspid leaflet in normal hearts and in hearts with Ebstein's anomaly. Anatomic and echographic correlation.  Am J Cardiol. 1984;  54 172-176
• 15 Chauvaud S M, Mihaileanu S A, Gaer J AR, Carpentier A C. Surgical treatment of Ebstein's malformation - the “Hôpital Broussais” experience.  Cardiol Young. 1996;  6 4-11
• 16 Seward J B. Ebstein's anomaly: ultrasound imaging and hemodynamic evaluation.  Echocardiography. 1993;  1 641
• 17 Celermajer D S, Bull C, Till J A. et al . Ebstein's anomaly: presentation and outcome from fetus to adult.  J Am Coll Cardiol. 1994;  23 170-176
• 18 Shiina A, Seward J B, Tajik A J, Hagler D J, Danielson G K. Two-dimensional echocardiographic - surgical correlation in Ebstein's anomaly: preoperative determination of patients requiring tricuspid valve plication vs replacement.  Circulation. 1983;  68 534-544
• 19 Anderson K R, Lie J T. The right ventricular myocardium in Ebstein's anomaly: a morphometric histopathologic study.  Mayo Clin Proc. 1979;  54 181-184
• 20 Benson L N, Child J S, Schwaiger M, Perloff J K, Schelbert H R. Left ventricular geometry and function in adults with Ebstein's anomaly of the tricuspid valve.  Circulation. 1987;  75 353-359
• 21 Carpentier A, Chauvaud S, Mace L. et al . A new reconstructive operation for Ebstein's anomaly of the tricuspid valve.  J Thorac Cardiovasc Surg. 1988;  96 92-101
• 22 Celermajer D S, Cullen S, Sullivan I D, Spiegelhalter D J, Wyse R K, Deanfield J E. Outcome in neonates with Ebstein's anomaly.  J Am Coll Cardiol. 1992;  19 1041-1046
• 23 Takayasu S, Obunai Y, Konno S. Clinical classification of Ebstein's anomaly.  Am Heart J. 1978;  95 154-162
• 24 Saxena A, Fong L V, Tristam M, Ackery D M, Keeton B R. Late noninvasive evaluation of cardiac performance in mildly symptomatic older patients with Ebstein's anomaly of tricuspid valve: role of radionuclide imaging.  J Am Coll Cardiol. 1991;  17 182-186
• 25 Monibi A A, Neches W H, Lenox C C, Park S C, Mathews R A, Zuberbuhler J R. Left ventricular anomalies associated with Ebstein's malformation of the tricuspid valve.  Circulation. 1978;  57 303-306
• 26 Saxena A, Fong L V, Tristam M, Ackery D M, Keeton B R. Left ventricular function in patients greater than 20 years of age with Ebstein's anomaly of the tricuspid valve.  Am J Cardiol. 1991;  67 217-219
• 27 Cabin H S, Roberts W C. Ebstein's anomaly of the tricuspid valve and prolapse of the mitral valve.  Am Heart J. 1981;  101 177-180
• 28 Castaneda Z W, Nath H P, Moller J H, Edwards J E. Left-sided anomalies in Ebstein's malformation of the tricuspid valve.  Pediatr Cardiol. 1982;  3 181-185
• 29 Roberts W C, Glancy D L, Seningen R P, Maron B J, Epstein S E. Prolapse of the mitral valve is described in two patients with the Ebstein's anomaly of the tricuspid.  Am J Cardiol. 1976;  38 377-382
• 30 Sharma S, Rajani M, Mukhopadhyay S, Aggarwal S, Shrivastava S, Tandon R. Angiographic abnormalities of the morphologically left ventricle in the presence of Ebstein's malformation.  Int J Cardiol. 1989;  22 109-113
• 31 Gerlis L M, Anderson R H. Cor triatriatum dexter with imperforate Ebstein's anomaly.  Br Heart J. 1976;  38 108-111
• 32 Lang D, Oberhoffer R, Cook A. et al . Pathologic spectrum of malformations of the tricuspid valve in prenatal and neonatal life.  J Am Coll Cardiol. 1991;  17 1161-1167
• 33 Eichhorn P, Ritter M, Suetsch G, von S L, Turina M, Jenni R. Congenital cleft of the anterior tricuspid leaflet with severe tricuspid regurgitation in adults.  J Am Coll Cardiol. 1992;  2 1175-1179