eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Ebstein Anomaly: Follow-up

Author: Kamran Riaz, MD, Clinical Assistant Professor, Department of Internal Medicine, Section of Cardiology, Wright State University School of Medicine
Contributor Information and Disclosures

Updated: Nov 11, 2009

Follow-up

Further Outpatient Care

Outpatient follow-up is directed toward the following:

  • Assessment of general symptoms (eg, fatigue, lassitude)
  • Assessment for development of bacterial endocarditis with careful history and examination for signs of bacterial endocarditis, such as fever, weight loss, anorexia, night sweats, splinter hemorrhages, clubbing, Roth spots, Janeway lesions, Osler nodes, splenomegaly, and hematuria
  • Assessment of signs of worsening heart failure, such as weight gain, ankle edema, and rales
  • Assessment for arrhythmias by history of symptoms, such as faintness or syncope
  • Periodic chest radiographs to assess for worsening cardiomegaly
  • Evaluation and adjustment of medications

Transfer

Transfer to a cardiothoracic surgery unit if necessary.

Complications

  • Congestive heart failure
  • Sudden cardiac death
  • Bacterial endocarditis
  • Brain abscess
  • Paradoxical embolism
  • Transient ischemic attacks
  • Stroke

Prognosis

  • Prognosis depends on the severity of the disease and treatment options available.
  • Poor prognostic signs include the following:
    • Male sex
    • Earlier age at presentation8
    • Cardiothoracic ratio of more than 0.65 on chest radiographs
    • Septal leaflet attachment ratio (ie, ratio of distance between AV ring and distal attachment of septal leaflet to length of septal leaflet) of more than 0.45
    • Increasing ratio of combined area of right atrium and atrialized right ventricle to that of the functional right ventricle—grade 1, which is less than 0.5, to grade 4, which is more than 1.5 (increase in the relative risk of 2.7 for each increment in the grade)
    • NYHA class - Also has been linked with mortality rate in some studies9
  • Pregnancy seems to be well tolerated with adequate supervision.10,7

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose Ebstein anomaly in patients with tricuspid regurgitation and right heart failure is a potential pitfall.
  • Failure to identify and treat various arrhythmias associated with Ebstein anomaly may cause problems.
  • Failure to refer the patient for electrophysiological studies is a potential pitfall.
  • Failure to provide endocarditis prophylaxis advice is a potential pitfall.
  • Failure to refer the patient to a cardiothoracic surgeon in a timely fashion is a potential pitfall.
 


More on Ebstein Anomaly

Overview: Ebstein Anomaly
Differential Diagnoses & Workup: Ebstein Anomaly
Treatment & Medication: Ebstein Anomaly
Follow-up: Ebstein Anomaly
References
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References

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  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. 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].

  6. 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].

  7. 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].

  8. 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].

  9. 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].

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  15. 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].

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  20. 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].

  21. 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.

  22. 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.

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  24. 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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Further Reading

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Keywords

Ebstein's anomaly, congenital heart disease, tricuspid regurgitation lithium ingestion during pregnancy, maternal benzodiazepine use, right heart failure, supraventricular tachycardia, accessory conduction pathways, bacterial endocarditis

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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 Medical Association, American Society of Echocardiography, Ohio State Medical Association, and Royal College of Physicians
Disclosure: Nothing to disclose.

Medical 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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
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.

 
 
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