Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Management and prognosis of Ebstein anomaly

Heidi M Connolly, MD, FASE
Muhammad Yasir Qureshi, MBBS, FASE
Joseph Dearani, MD
Section Editors
Matthias Greutmann, MD, FESC
David R Fulton, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC


Ebstein anomaly is a congenital malformation that is characterized primarily by abnormalities of the tricuspid valve and right ventricle (figure 1). The clinical presentation of Ebstein anomaly varies widely, ranging from symptomatic neonate to the asymptomatic adult, depending upon the degree of anatomic abnormality and concomitant lesions (eg, atrial septal defect). The management and prognosis of Ebstein anomaly are discussed here.

The clinical manifestations and diagnosis of Ebstein anomaly are discussed separately. (See "Clinical manifestations and diagnosis of Ebstein anomaly".)


Approach to management — The approach to management of patients with Ebstein anomaly is determined by the patient’s age and clinical presentation including presence of symptoms of heart failure secondary to tricuspid regurgitation and right heart failure, cyanosis, and right ventricular dilation or dysfunction. The components of management are monitoring, medical management (including temporizing relief of symptoms prior to surgery), management of arrhythmias, and surgical or catheter intervention. Many patients with Ebstein anomaly have no symptoms and require only monitoring while others are symptomatic and require supportive medical therapy and surgical intervention.

All patients with Ebstein anomaly should undergo periodic monitoring. (See 'Monitoring' below.)

For the newborn with Ebstein anomaly presenting with cyanosis or heart failure, medical management with advanced neonatal care is preferred to delay surgery as long as possible except when an indication for surgery during the neonatal period is present. (See 'Management of symptomatic newborn' below and 'For neonates' below.)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Feb 06, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008; 118:e714.
  2. 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.
  3. Wilson W, Taubert KA, Gewitz M, et al. Prevention of Infective Endocarditis. Guidelines From the American Heart Association. A Guideline From the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation 2007; 115 published online April 19, 2007. www.circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.106.183095v1 (Accessed on May 04, 2007).
  4. Mair DD. Ebstein's anomaly: natural history and management. J Am Coll Cardiol 1992; 19:1047.
  5. Van Hare GF, Ackerman MJ, Evangelista JA, et al. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 4: Congenital Heart Disease: A Scientific Statement From the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015; 66:2372.
  6. Graham TP Jr, Driscoll DJ, Gersony WM, et al. Task Force 2: congenital heart disease. J Am Coll Cardiol 2005; 45:1326.
  7. Cappato R, Schlüter M, Weiss C, et al. Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly. Circulation 1996; 94:376.
  8. Reich JD, Auld D, Hulse E, et al. The Pediatric Radiofrequency Ablation Registry's experience with Ebstein's anomaly. Pediatric Electrophysiology Society. J Cardiovasc Electrophysiol 1998; 9:1370.
  9. Misaki T, Watanabe G, Iwa T, et al. Surgical treatment of patients with Wolff-Parkinson-White syndrome and associated Ebstein's anomaly. J Thorac Cardiovasc Surg 1995; 110:1702.
  10. Stulak JM, Sharma V, Cannon BC, et al. Optimal surgical ablation of atrial tachyarrhythmias during correction of Ebstein anomaly. Ann Thorac Surg 2015; 99:1700.
  11. Roten L, Lukac P, DE Groot N, et al. Catheter ablation of arrhythmias in ebstein's anomaly: a multicenter study. J Cardiovasc Electrophysiol 2011; 22:1391.
  12. Shivapour JK, Sherwin ED, Alexander ME, et al. Utility of preoperative electrophysiologic studies in patients with Ebstein's anomaly undergoing the Cone procedure. Heart Rhythm 2014; 11:182.
  13. Waickman LA, Skorton DJ, Varner MW, et al. Ebstein's anomaly and pregnancy. Am J Cardiol 1984; 53:357.
  14. Connolly HM, Warnes CA. Ebstein's anomaly: outcome of pregnancy. J Am Coll Cardiol 1994; 23:1194.
  15. Thompson LP, Crimmins S, Telugu BP, Turan S. Intrauterine hypoxia: clinical consequences and therapeutic perspectives. Research and Reports in Neonatology 2015; 5:79.
  16. Kounis NG, Zavras GM, Papadaki PJ, et al. Pregnancy-induced increase of supraventricular arrhythmias in Wolff-Parkinson-White syndrome. Clin Cardiol 1995; 18:137.
  17. Donnelly JE, Brown JM, Radford DJ. Pregnancy outcome and Ebstein's anomaly. Br Heart J 1991; 66:368.
  18. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol 2007; 49:2303.
  19. Houser L, Zaragoza-Macias E, Jones TK, Aboulhosn J. Transcatheter closure of atrial septal communication during pregnancy in women with Ebstein's anomaly of the tricuspid valve and cyanosis. Catheter Cardiovasc Interv 2015; 85:842.
  20. Knott-Craig CJ, Goldberg SP, Overholt ED, et al. Repair of neonates and young infants with Ebstein's anomaly and related disorders. Ann Thorac Surg 2007; 84:587.
  21. Knott-Craig CJ, Goldberg SP, Kirklin JK. Surgical strategy to prevent cardiac injury during reoperation in infants. J Cardiothorac Surg 2008; 3:10.
  22. Starnes VA, Pitlick PT, Bernstein D, et al. Ebstein's anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg 1991; 101:1082.
  23. Dearani JA, Mora BN, Nelson TJ, et al. Ebstein anomaly review: what's now, what's next? Expert Rev Cardiovasc Ther 2015; 13:1101.
  24. Brown ML, Dearani JA, Danielson GK, et al. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg 2008; 135:1120.
  25. Sarris GE, Giannopoulos NM, Tsoutsinos AJ, et al. Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association. J Thorac Cardiovasc Surg 2006; 132:50.
  26. Chauvaud S, Berrebi A, d'Attellis N, et al. Ebstein's anomaly: repair based on functional analysis. Eur J Cardiothorac Surg 2003; 23:525.
  27. Kiziltan HT, Theodoro DA, Warnes CA, et al. Late results of bioprosthetic tricuspid valve replacement in Ebstein's anomaly. Ann Thorac Surg 1998; 66:1539.
  28. Dearani JA, Bacha E, da Silva JP. Cone Reconstruction of the Tricuspid Valve for Ebstein's Anomaly: Anatomic Repair. Op Tech Thorac Cardiovasc Surg 2008; :109.
  29. Augustin N, Schmidt-Habelmann P, Wottke M, et al. Results after surgical repair of Ebstein's anomaly. Ann Thorac Surg 1997; 63:1650.
  30. Anderson HN, Dearani JA, Said SM, et al. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenit Heart Dis 2014; 9:266.
  31. Attenhofer Jost CH, Connolly HM, Scott CG, et al. Outcome of cardiac surgery in patients 50 years of age or older with Ebstein anomaly: survival and functional improvement. J Am Coll Cardiol 2012; 59:2101.
  32. Celermajer DS, Bull C, Till JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994; 23:170.
  33. Vargas FJ, Mengo G, Granja MA, et al. Tricuspid annuloplasty and ventricular plication for Ebstein's malformation. Ann Thorac Surg 1998; 65:1755.
  34. da Silva JP, Baumgratz JF, da Fonseca L, et al. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg 2007; 133:215.
  35. Quaegebeur JM, Sreeram N, Fraser AG, et al. Surgery for Ebstein's anomaly: the clinical and echocardiographic evaluation of a new technique. J Am Coll Cardiol 1991; 17:722.
  36. Hetzer R, Nagdyman N, Ewert P, et al. A modified repair technique for tricuspid incompetence in Ebstein's anomaly. J Thorac Cardiovasc Surg 1998; 115:857.
  37. Marianeschi SM, McElhinney DB, Reddy VM, et al. Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading. Ann Thorac Surg 1998; 66:1546.
  38. Wu Q, Huang Z. A new procedure for Ebstein's anomaly. Ann Thorac Surg 2004; 77:470.
  39. Bove EL, Kirsh MM. Valve replacement for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1979; 78:229.
  40. van Son JA, Falk V, Black MD, et al. Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia. Eur J Cardiothorac Surg 1998; 13:280.
  41. Kaulitz R, Ziemer G. Modified Fontan procedure for Ebstein's anomaly of the tricuspid valve--an alternative surgical approach preserving Ebstein's anatomy. Thorac Cardiovasc Surg 1995; 43:275.
  42. Quinonez LG, Dearani JA, Puga FJ, et al. Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. J Thorac Cardiovasc Surg 2007; 133:1303.
  43. Malhotra SP, Petrossian E, Reddy VM, et al. Selective right ventricular unloading and novel technical concepts in Ebstein's anomaly. Ann Thorac Surg 2009; 88:1975.
  44. Silva M, Teixeira A, Menezes I, et al. Percutaneous closure of atrial right-to-left shunt in patients with Ebstein's anomaly of the tricuspid valve. EuroIntervention 2012; 8:94.
  45. Jategaonkar SR, Scholtz W, Horstkotte D, et al. Interventional closure of atrial septal defects in adult patients with Ebstein's anomaly. Congenit Heart Dis 2011; 6:374.
  46. Kenny D, Hijazi ZM, Walsh KP. Transcatheter tricuspid valve replacement with the Edwards SAPIEN valve. Catheter Cardiovasc Interv 2011; 78:267.
  47. Roberts PA, Boudjemline Y, Cheatham JP, et al. Percutaneous tricuspid valve replacement in congenital and acquired heart disease. J Am Coll Cardiol 2011; 58:117.
  48. Freud LR, Escobar-Diaz MC, Kalish BT, et al. Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study. Circulation 2015; 132:481.