Skip to main content

Ebstein’s Anomaly of the Tricuspid Valve: Fetal Physiology and Outcomes

  • Chapter
  • 1083 Accesses

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   89.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Ebstein W. Ueber einen sehr seltenen Fall von Insuffizienz der Valvula tricuspidalis, bedingt durch eine angeborene Missbildung derselben. Arch Anat Physiol Wissensch Med. 1866;238–254.

    Google Scholar 

  2. Soloff LA, Stauffer HM, Zatuchni J. Ebstein’s disease: report of the first case diagnosed during life. Am J Med Sci. 1951;222:554–561.

    PubMed  CAS  Google Scholar 

  3. Sharf M, Abinader EG, Shapiro I, Rosenfeld T, Eibschitz I. Prenatal echocardiographic diagnosis of Ebstein's anomaly with pulmonary atresia. Am J Obstet Gynecol. 1983;147:300–303.

    PubMed  CAS  Google Scholar 

  4. Anderson KR, Lie JT. Pathologic anatomy of Ebstein’s anomaly of the heart revisited. Am J Cardiol. 1978;41:739–745.

    Article  PubMed  CAS  Google Scholar 

  5. Zuberbuhler JR, Allwork SP, Anderson RH. The spectrum of Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1979;77:202–211.

    PubMed  CAS  Google Scholar 

  6. 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.

    PubMed  CAS  Google Scholar 

  7. Gussenhoven EJ, Stewart PA, Becker AE, Essed CE, Ligtvoet KM, De Villeneuve VH. “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.

    Article  PubMed  CAS  Google Scholar 

  8. Zuberbuhler JR, Anderson RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J. 1979;41:281–288.

    Article  PubMed  CAS  Google Scholar 

  9. Schreiber C, Cook A, H SY, Augustin N, Anderson RH. Morphological spectrum of Ebstein’s malformation: revisitation relative to surgical repair. J Thorac Cardiovasc Surg. 1999;117:148–155.

    Article  PubMed  CAS  Google Scholar 

  10. Stellin G, Santini F, Thiene G, Bortolotti U, Daliento L, Milanesi O, Sorbara C, Mazzucco A, Casarotto D. Pulmonary atresia, intact ventricular septum, and Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1993;106:255–261.

    PubMed  CAS  Google Scholar 

  11. Freedom RM, Jaeggi E, Perrin D, Yoo SJ, Anderson RH. The “wall-to-wall” heart in patients with pulmonary atresia and intact ventricular septum. Cardiol Young. 2006;16:18–29.

    Article  PubMed  Google Scholar 

  12. Lang D, Oberhoffer R, Cook A, Sharland G, Allan L, Fagg N, Anderson RH. Pathologic spectrum of malformations of the tricuspid valve in prenatal and neonatal life. J Am Coll Cardiol. 1991;17:1161–1167.

    PubMed  CAS  Google Scholar 

  13. Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RK, Deanfield JE. Outcome in neonates with Ebstein's anomaly. J Am Coll Cardiol. 1992;19:1041–1046.

    PubMed  CAS  Google Scholar 

  14. Celermajer DS, Dodd SM, Greenwald SE, Wyse RK, Deanfield JE. Morbid anatomy in neonates with Ebstein's anomaly of the tricuspid valve: pathophysiologic and clinical implications. J Am Coll Cardiol. 1992;19:1049–1053.

    PubMed  CAS  Google Scholar 

  15. Monibi AA, Neches WH, Lenox CC, Park SC, Mathews RA, Zuberbuhler JR. Left ventricular anomalies associated with Ebstein’s malformation of the tricuspid valve. Circulation. 1978;57:303–306.

    PubMed  CAS  Google Scholar 

  16. Benson LN, Child JS, Schwaiger M, Perloff JK, Schelbert HR. Left ventricular geometry and function in adults with Ebstein's anomaly of the tricuspid valve. Circulation. 1987;75:353–359.

    PubMed  CAS  Google Scholar 

  17. Gerlis LM, Ho SY, Sweeney AE. Mitral valve anomalies associated with Ebstein's malformation of the tricuspid valve. Am J Cardiovasc Pathol. 1993;4:294–301.

    PubMed  CAS  Google Scholar 

  18. Attenhofer Jost CH, Connolly HM, O’Leary PW, Warnes CA, Tajik AJ, Seward JB. Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc. 2005;80:361–368.

    PubMed  Google Scholar 

  19. Correa-Villasenor A, Ferencz C, Neill CA, Wilson PD, Boughman JA. Ebstein’s malformation of the tricuspid valve: genetic and environmental factors. Teratology. 1994;50:137–147.

    Article  PubMed  CAS  Google Scholar 

  20. Freedom RM, Yoo SJ. Ebstein malformation of the tricuspid valve. In: Freedom RM, Yoo SJ, Mikailian H, Williams WG, editors. The natural and modified history of congenital heart disease. New York: Blackwell Publishing; USA. 2004. p 91–96.

    Google Scholar 

  21. Hornberger LK, Sahn DJ, Kleinman CS, Copel JA, Reed KL. Tricuspid valve disease with significant insufficiency in the fetus: diagnosis and outcome. J Am Coll Cardiol. 1991;17:167–173

    PubMed  CAS  Google Scholar 

  22. Sharland GK, Chita SK, Allan LD. Tricuspid valve dysplasia or displacement in intrauterine life. J Am Coll Cardiol. 1991;17:944–949.

    PubMed  CAS  Google Scholar 

  23. Miller MS, Rao PN, Dudovitz RN, Falk RE. Ebstein anomaly and duplication of the distal arm of chromosome 15: report of two patients. Am J Med Genet A. 2005;139:141–145.

    Google Scholar 

  24. Nakagawa M, Kato H, Aotani H, Kondo M. Ebstein’s anomaly associated with trisomy 9p. Clin Genet. 1999;55:383–385.

    PubMed  CAS  Google Scholar 

  25. Attenhofer Jost CH, Connolly HM, Warnes CA, O’Leary P, Tajik AJ, Pelikka SA, Seward JB. Noncompacted myocardium in Ebstein’s anomaly: initial description in three patients. J Am Soc Echocardiography. 2004;17:677–680.

    Article  Google Scholar 

  26. McIntosh N, Chitayat D, Bardanis M, Fouron JC. Ebstein anomaly: report of a familial occurrence and prenatal diagnosis. Am J Med Genet. 1992;42:307–309.

    Article  PubMed  CAS  Google Scholar 

  27. Balaji S, Dennis NR, Keeton BR. Familial Ebstein’s anomaly: a report of six cases in two generations associated with mild skeletal abnormalities. Br Heart J. 1991;66:28–28.

    Google Scholar 

  28. Rosenmann A, Arad I, Simcha A, Schaap T. Familial Ebstein’s anomaly. J Med Genet. 1976;13:532–535.

    Article  PubMed  CAS  Google Scholar 

  29. Donegon CC, Jr., Moore MM, Wiley TM, Jr., Hernandez FA, Green JR, Jr., Schiebler GL. Familial Ebstein’s anomaly of the tricuspid valve. Am Heart J. 1968;75:375–379.

    Article  Google Scholar 

  30. McElhinney DB, Salvin JW, Colan SD, Thiagarajan R, Crawford EC, Marcus EN, del Nido PJ, Tworetzky W. Improved outcomes in fetuses and neonates with congenital displacement (Ebstein’s malformation) or dysplasia of the tricuspid valve. Am J Cardiol. 2005;96:582–586.

    Article  PubMed  Google Scholar 

  31. Shipp TD, Bromley B, Hornberger LK, Nadel A, Benacerraf BR. Levorotation of the fetal cardiac axis: a clue for the presence of congenital heart disease. Obstet Gynecol. 1995;85:97–102.

    Article  PubMed  CAS  Google Scholar 

  32. Berning RA, Silverman NH, Villegas M, Sahn DJ, Martin GR, Rice MJ. Reversed shunting across the ductus arteriosus or atrial septum in utero heralds severe congenital heart disease. J Am Coll Cardiol. 1996;27:481–486.

    Article  PubMed  CAS  Google Scholar 

  33. Berg C, Kremer C, Geipel A, Kohl T, Germer U, Gembruch U. Ductus venosus blood flow alterations in fetuses with obstructive lesions of the right heart. Ultrasound Obstet Gynecol. 2006;28:137–1342.

    Article  PubMed  CAS  Google Scholar 

  34. Schwartz ML. Fetal progression of Ebstein’s anomaly. Circulation. 2003;108:c86–c87.

    Article  Google Scholar 

  35. Todros T, Paladini D, Chiappa E, Russo MG, Gaglioti P, Pacileo G, Cau MA, Martinelli P. Pulmonary stenosis and atresia with intact ventricular septum during prenatal life. Ultrasound Obstet Gynecol. 2003; 21:228–233.

    Article  PubMed  CAS  Google Scholar 

  36. Roman KS, Fouron JC, Nii M, Smallhorn JF, Chaturvedi R, Jaeggi ET. Determinants of outcome in pulmonary valve stenosis or atresia with intact ventricular septum. Am J Cardiol. 2007; In Press.

    Google Scholar 

  37. Haworth SG, Shinebourne EA, Miller GA. Right-to-left interatrial shunting with normal right ventricular pressure. A puzzling haemodynamic picture associated with some rare congenital malformations of the right ventricle and tricuspid valve. Br Heart J. 1975;37:386–391.

    Article  PubMed  CAS  Google Scholar 

  38. Yetman AT, Freedom RM, McCrindle BW. Outcome in cyanotic neonates with Ebstein’s anomaly. Am J Cardiol. 1998;81:749–754.

    Article  PubMed  CAS  Google Scholar 

  39. Atz AM, Munoz RA, Adatia I, Wessel DL. Diagnostic and therapeutic uses of inhaled nitric oxide in neonatal Ebstein’s anomaly. Am J Cardiol. 2003;91:906–908.

    Article  PubMed  CAS  Google Scholar 

  40. Wald RM, Adatia I, Van Arsdell GS, Hornberger LK. Relation of limiting ductal patency to survival in neonatal Ebstein's anomaly. Am J Cardiol. 2005;96:851–856.

    Article  PubMed  Google Scholar 

  41. Andrews RE, Tibby SM, Sharland G, Simpson JM. Outcome following diagnosis of isolated tricuspid valve malformations in the fetus. Cardiol Young. 2006;16 Suppl 2:6–19.

    Google Scholar 

  42. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P, Somerville J, Deanfield JE. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23:170–176.

    Article  PubMed  CAS  Google Scholar 

  43. Robertson DA, Silverman NH. Ebstein’s anomaly: echocardiographic and clinical features in the fetus and neonate. J Am Coll Cardiol. 1989;14:1300–1307.

    Article  Google Scholar 

  44. Pavlova M, Fouron JC, Drblik SP, van Doesburg NH, Bigras JL, Smallhorn J, Harder J, Robertson M. Factors influencing the prognosis of Ebstein’s anomaly during fetal life. Am Heart J. 1998;135:1081–1085.

    Article  PubMed  CAS  Google Scholar 

  45. Watson H. The natural history of Ebstein’s anomaly in childhood and adolescence. A preliminary report on the first 100 cases. Proc Assoc Eur Cardiol. 1970;6:35–39.

    Google Scholar 

  46. Watson H. Natural history of Ebstein’s anomaly of the tricuspid valve in childhood and adolescence: an international cooperative study of 505 cases. Br Heart J. 1974;36:417–427.

    Article  PubMed  CAS  Google Scholar 

  47. Giuliani ER, Fuster V, Brandenburg RO, Mair DD. Ebstein’s anomaly: the clinical features and natural history of Ebstein’s anomaly of the tricuspid valve. Mayo Clin Proc. 1979;54:163–173.

    PubMed  CAS  Google Scholar 

  48. Radford DJ, Graff RF, Neilson GH. Diagnosis and natural history of Ebstein’s anomaly. Br Heart J. 1985;36:517–522.

    Article  Google Scholar 

  49. Kumar AE, Fyler DC, Miettinen OS, Nadas AS. Ebstein’s anomaly: clinical profile and natural history. Am J Cardiol. 1971;28:84–95.

    Article  PubMed  CAS  Google Scholar 

  50. Hong YM, Moller JH. Ebstein’s anomaly: a long term study of survival. Am Heart J. 1993;125:1419–1424.

    Article  PubMed  CAS  Google Scholar 

  51. Arduini D, Rizzo G, Romanini C. Fetal cardiac output measurements in normal and pathological states. In: Copel JA, Reed KL, editors. Doppler ultrasound in obstetrics and gynecology. New York: Raven Press; USA. 1995. p. 271–290.

    Google Scholar 

  52. Friedman WF. The intrinsic physiologic properties of the developing heart. Prog Cardiovasc Dis. 1972;15:87–111.

    Article  PubMed  CAS  Google Scholar 

  53. Kaufman TM, Horton JW, White DJ, Mahoney L. Age-related changes in myocardial relaxation and sarcoplasmatic reticulum function. Am J Physiol. 1990;259:H309–H316.

    PubMed  CAS  Google Scholar 

  54. Rudolph AM. Fetal circulation. In: Yagel S, Silverman NH, Gembruch U, editors. Fetal Cardiology. London: Martin Dunits; UK. 2003. p.107–120.

    Google Scholar 

  55. Morton MJ, Thornburg KL. The pericardium and cardiac transmural filling pressure in the fetal sheep. J Dev Physiol. 1987;9:159–168.

    PubMed  CAS  Google Scholar 

  56. Grant DA, Maloney JE, Tyberg JV, Walker AM. Effects of external constraint on the fetal left ventricular function curve. Am Heart J. 1992;123:1601–1609.

    Article  PubMed  CAS  Google Scholar 

  57. Grant DA, Kondo CS, Maloney JE, Walker AM, Tyberg V. Changes in pericardial pressure during the perinatal period. Circulation. 1992;86:1615–1621.

    PubMed  CAS  Google Scholar 

  58. Grant DA, Walker AM. Pleural and pericardial pressures limit fetal right ventricular output. Circulation. 1996;94:555–561.

    PubMed  CAS  Google Scholar 

  59. Grant DA, Fauchère JC, Eede KJ, Tyberg JV, Walker AM. Left ventricular stroke volume in the fetal sheep is limited by extracardiac constraint and arterial pressure. J Physiol. 2001;535:231–239.

    Article  PubMed  CAS  Google Scholar 

  60. Gilbert RD. Effects of afterload and baroreceptors on cardiac function in fetal sheep. J Dev Physiol. 1982;4:299–309.

    PubMed  CAS  Google Scholar 

  61. Reller MD, Morton MJ, Reid DL, Thornberg KL. Fetal lamb ventricles respond differently to filling and arterial pressures and to in utero ventilation. Pediatr Res. 1987;22:621–626.

    Article  PubMed  CAS  Google Scholar 

  62. Gold PS, Brace RA. Fetal whole-body interstitial compliance and capillary filtration coefficient. Am J Physiol. 1984;247:R800–R805.

    PubMed  Google Scholar 

  63. Johnson SA, Vander Straten MC, Parellada JA, Schnakenberg W, Gest AL. Thoracic duct function in fetal, newborn and adult sheep. Lymphology. 1996;29:50–56.

    PubMed  CAS  Google Scholar 

  64. Gest AL, Blair DK, Vander Straten MC. The effect of outflow pressure upon thoracic duct lymph flow rate in fetal sheep. Pediatr Res. 1992;32:585–588.

    Article  PubMed  CAS  Google Scholar 

  65. Gest AL, Bair DK, Vander Straten MC. Thoracic duct lymph flow in fetal sheep with increased venous pressure from electrically induced tachycardia. Biol Neonate. 1993;64:325–330.

    Article  PubMed  CAS  Google Scholar 

  66. Pinson CW, Morton MJ, Thornburg KL. An anatomic basis for fetal right ventricular dominance and arterial pressure sensitivity. J Dev Physiol. 1987;9:253–269.

    PubMed  CAS  Google Scholar 

  67. Atkins DL, Clark EB, Marvin WJ, Jr. Foramen ovale/atrial septum area ratio: a marker of transatrial blood flow. Circulation. 1982;66:281–283.

    PubMed  CAS  Google Scholar 

  68. Feit LR, Copel JA, Kleinman CS. Foramen ovale size in the normal and abnormal human fetal heart: an indicator of transatrial flow physiology Ultrasound Obstet Gynecol. 1991;1:313–319.

    Article  PubMed  CAS  Google Scholar 

  69. Marshall AC, van der Velde ME, Tworetzky W, Gomez CA, Wilkins-Haug L, Benson CB, Jennings RW, Lock JE. Creation of an atrial septal defect in utero for fetuses with hypoplastic left heart syndrome and intact or highly restrictive atrial septum. Circulation. 2004;110:253–258.

    Article  PubMed  Google Scholar 

  70. Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Baker SS, Razook JD. Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up. Ann Thorac Surg. 2002;73:1786–1792.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2009 Springer-Verlag London Limited

About this chapter

Cite this chapter

Jaeggi, E.T., Cavalle-Garido, T. (2009). Ebstein’s Anomaly of the Tricuspid Valve: Fetal Physiology and Outcomes. In: Redington, A.N., Van Arsdell, G.S., Anderson, R.H. (eds) Congenital Diseases in the Right Heart. Springer, London. https://doi.org/10.1007/978-1-84800-378-1_35

Download citation

  • DOI: https://doi.org/10.1007/978-1-84800-378-1_35

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-84800-377-4

  • Online ISBN: 978-1-84800-378-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics