Ebstein Anomaly Treatment & Management

  • Author: Kamran Riaz, MD; Chief Editor: Park W Willis IV, MD   more...
 
Updated: May 31, 2011
 

Medical Care

Ebstein anomaly presents with a spectrum of congenital abnormalities of the tricuspid valve and the right ventricle. The age of presentation with symptoms is variable, and a wide range of treatment options is available. Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Treatment options include medical therapy, radiofrequency ablation, and surgical therapy.

  • Antibiotic prophylaxis for infective endocarditis
  • Medical therapy for heart failure - Angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin
  • Arrhythmia treatment - Medical treatments such as anti-arrhythmic drugs or radiofrequency ablation of the accessory pathways
    • Curative therapy of SVT with radiofrequency ablation is currently the treatment of choice.
    • The success rate is lower than that in patients without significant structural heart disease.
    • Factors associated with lower likelihood of success include the following:
      • Accessory pathways located along the atrialized right ventricle
      • Multiple accessory pathways
      • Complex geometry of the pathways
      • Abnormal morphology of the endocardial action potentials in this region
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Surgical Care

Surgical care includes correction of the underlying tricuspid valve and right ventricular abnormalities, correction of any associated intracardiac defects, palliative procedures in early days of life as a bridge to more definitive surgical treatment later, and surgical treatment of associated arrhythmias. Complete repair of Ebstein anomaly in symptomatic neonates has been shown to be feasible, with good early and late survival and excellent functional status.[5]

  • Indications for surgery are generally as follows:
    • New York Heart Association (NYHA) class I-II heart failure with worsening symptoms or with a cardiothoracic ratio of 0.65 or greater[6]
    • NYHA class III-IV heart failure
    • History of paradoxical embolism
    • Significant cyanosis with arterial O2 saturation of 80% or less and/or polycythemia with hemoglobin of 16 g/dL or more
    • Arrhythmias refractory to medical and radiofrequency ablation
  • Generally, the trend is to perform surgery earlier rather later in the course of heart failure.
  • Various approaches are available to treat structural abnormalities.
    • Tricuspid valve repair is preferred over valve replacement, and bioprosthetic valves are preferred over mechanical prosthetic valves.
    • The atrialized portion of the right ventricle can be resected surgically, and the markedly dilated, thin-walled right atrium can be resected.
    • Associated septal defects may be closed.
  • Palliative procedures include creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through aortopulmonary shunt. Palliative procedures usually are reserved for severely ill infants with otherwise dire prognosis.
  • Left ventricular dysfunction should not be considered a contraindication to tricuspid valve surgery. In these patients, although early mortality is greater with tricuspid valve surgery, the late results are favorable and left ventricular function seems to improve postoperatively.[7, 8]
  • Functional status improves after surgery.
  • Surgical treatments of arrhythmias include the following:
    • Ablation of the accessory pathways
    • Maze procedure for atrial arrhythmias
  • Cardiac transplantation is appropriate in selected patients.
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Consultations

  • Electrophysiologist
  • Cardiac surgeon
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Diet

A low-sodium diet is recommended for symptomatic relief from fluid overload.

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Activity

Activity may be allowed as tolerated.

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Contributor Information and Disclosures
Author

Kamran Riaz, MD  Clinical Assistant Professor, Department of Internal Medicine, Section of Cardiology, Wright State University School of Medicine

Kamran Riaz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Society of Echocardiography, Ohio State Medical Association, and Royal College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Marschall S Runge, MD, PhD  Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine

Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association

Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

Amer Suleman, MD  Private Practice

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

References

  1. Muñoz-Castellanos L, Espinola-Zavaleta N, Kuri-Nivón M, Keirns C. Ebstein's Anomaly: anatomo-echocardiographic correlation. Cardiovasc Ultrasound. Nov 23 2007;5:43. [Medline].

  2. Armengol Rofes AJ, Serrano Durán M, Albert Brotons DC, Sánchez López C, Casaldáliga Ferrer J, Girona Comas JM. [Ebstein's anomaly of the tricuspid valve. Apropos 35 cases]. An Esp Pediatr. Feb 1996;44(2):139-44. [Medline].

  3. Khositseth A, Khowsathit P. Factors affecting mortality in Ebstein's anomaly of the tricuspid valve. J Med Assoc Thai. Nov 1999;82 Suppl 1:S10-5. [Medline].

  4. Correa-Villaseñor A, Ferencz C, Neill CA, Wilson PD, Boughman JA. Ebstein's malformation of the tricuspid valve: genetic and environmental factors. The Baltimore-Washington Infant Study Group. Teratology. Aug 1994;50(2):137-47. [Medline].

  5. Boston US, Goldberg SP, Ward KE, et al. Complete repair of Ebstein anomaly in neonates and young infants: A 16-year follow-up. J Thorac Cardiovasc Surg. May 2011;141(5):1163-9. [Medline].

  6. Attie F, Casanova JM, Zabal C, Buendía A, Miranda I, Rijlaarsdam M. Ebstein's anomaly. Clinical profile in 174 patients. Arch Inst Cardiol Mex. Jan-Feb 1999;69(1):17-25. [Medline].

  7. Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, et al. Effect of operation for Ebstein anomaly on left ventricular function. Am J Cardiol. Dec 15 2008;102(12):1724-7. [Medline].

  8. Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, et al. Functional status after operation for Ebstein anomaly: the Mayo Clinic experience. J Am Coll Cardiol. Aug 5 2008;52(6):460-6. [Medline].

  9. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. Jan 1994;23(1):170-6. [Medline].

  10. Hong YM, Moller JH. Ebstein's anomaly: a long-term study of survival. Am Heart J. May 1993;125(5 Pt 1):1419-24. [Medline].

  11. Riesgo CA, Gómez LG, Higareda SH, Vargas JR. [Ebstein anomaly and pregnancy]. Ginecol Obstet Mex. Aug 2008;76(8):461-7. [Medline].

  12. Ammash NM, Warnes CA, Connolly HM, Danielson GK, Seward JB. Mimics of Ebstein's anomaly. Am Heart J. Sep 1997;134(3):508-13. [Medline].

  13. Attie F, Rosas M, Rijlaarsdam M, et al. The adult patient with Ebstein anomaly. Outcome in 72 unoperated patients. Medicine (Baltimore). Jan 2000;79(1):27-36. [Medline].

  14. Augustin N, Schreiber C, Lunge R. Valve preserving treatment of Ebstein's anomaly: perioperative and follow-up results. Thorac Cardiovasc Surg. Oct 2000;48(5):316. [Medline].

  15. Augustin N, Schreiber C, Wottke M, Meisner H. [Ebstein's anomaly: when should a patient have operative treatment?]. Herz. Aug 1998;23(5):287-92. [Medline].

  16. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. Second of two parts. N Engl J Med. Feb 3 2000;342(5):334-42. [Medline].

  17. Chauvaud S. Ebstein's malformation. surgical treatment and results. Thorac Cardiovasc Surg. Aug 2000;48(4):220-3. [Medline].

  18. Friedman WF. Congenital heart disease in infancy and childhood. In: Braunwald E, ed. Heart Disease: A Text Book of Cardiovascular Medicine. 5th ed. Philadelphia, Pa: WB Saunders Company; 1997:934-5.

  19. Gerlis LM, Ho SY, Sweeney AE. Mitral valve anomalies associated with Ebstein's malformation of the tricuspid valve. Am J Cardiovasc Pathol. 1993;4(4):294-301. [Medline].

  20. Kaneko Y, Okabe H, Nagata N, Yasui S, Yamada S, Kobayashi J. Repair of septal and posterior tricuspid leaflets in Ebstein's anomaly. J Card Surg. Jul 1998;13(4):229-35. [Medline].

  21. MacLellan-Tobert SG, Driscoll DJ, Mottram CD, Mahoney DW, Wollan PC, Danielson GK. Exercise tolerance in patients with Ebstein's anomaly. J Am Coll Cardiol. Jun 1997;29(7):1615-22. [Medline].

  22. Perloff JK. Ebstein's anomaly of the tricuspid valve. In: Perloff JK, ed. The Clinical Recognition of Congenital Heart Disease. 3rd ed. Philadelphia, Pa: WB Saunders Company; 1991:235-251.

  23. Ronan Jr JA. Congenital heart disease. In: Chizner MA, ed. Classic Teachings in Clinical Cardiology. 2. First ed. Cedar Grove, NJ: Laennec; 1996:1242-44.

  24. Shaughnessy R. Ebstein's anomaly. In: Crawford MH, DiMarco JP, eds. Cardiology. London: Mosby International; 2001:6.13.1-6.

  25. Weyman AE. Right ventricular inflow tract (congenital anomalies associated with tricuspid regurgitation). In: Weyman AE, ed. Principles and Practice of Echocardiography. 1994. 3rd ed. Philadelphia, Pa: Lea & Febiger:840-2.

 
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