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Ebstein malformation of the tricuspid valve: Current concepts in management and outcomes

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Opinion statement

Ebstein anomaly is a myopathy of the right ventricle that results in variable degrees of failure of delamination of the tricuspid valve leaflets from the underlying endocardium, leading to severe tricuspid valve regurgitation and some degree of right ventricular dysfunction. In neonates or infants who remain in congestive heart failure or profoundly cyanotic while receiving appropriate medical therapy, operation is required. Current strategies include biventricular or single-ventricle repair. In children and adults, medical management may be used, but most patients eventually require surgery. Tricuspid valve repair is preferred; we believe the cone repair is the most anatomic repair and is the operation of choice. Tricuspid valve replacement may be necessary in cases in which the valve is not repairable. A bidirectional cavopulmonary shunt is useful in patients with severe right ventricular dilatation and/or dysfunction. Transplantation rarely is necessary.

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References and Recommended Reading

  1. Ebstein W: Ueber einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Arch Anat Physiol 1866, 238–254.

  2. Celermajer DS, Bull C, Till JA, et al.: Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994, 23:170–176.

    PubMed  CAS  Google Scholar 

  3. Knott-Craig CJ, Overholt ED, Ward KE, et al.: Repair of Ebstein’s anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up. Ann Thorac Surg 2002, 73:1786–1793.

    Article  PubMed  Google Scholar 

  4. Knott-Craig CJ, Overhold ED, Ward KE, Razook JD: Neonatal repair of Ebstein’s anomaly: indications, surgical technique, and medium-term follow-up. Ann Thorac Surg 2000, 69:1505–1510.

    Article  PubMed  CAS  Google Scholar 

  5. 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, 84:587–593.

    Article  PubMed  Google Scholar 

  6. Knott-Craig CJ: Management of neonatal Ebstein’s anomaly. Oper Tech 2008, 13:101–108.

    Google Scholar 

  7. Starnes VA, Pitlick PT, Bernstein D, et al.: Ebstein’s anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg 1991, 101:1082–1087.

    PubMed  CAS  Google Scholar 

  8. Reemtsen BL, Fagan BT, Wells WJ, et al.: Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg 2006, 132:1285–1290.

    Article  PubMed  Google Scholar 

  9. Reemtsen BL, Polimenakos AC, Fagan BT, et al.: Fate of the right ventricle after fenestrated right ventricular exclusion for severe neonatal Ebstein anomaly. J Thorac Cardiovasc Surg 2007, 134:1406–1412.

    Article  PubMed  Google Scholar 

  10. Starnes VA, Reemtsen BL: Fenestrated right ventricular exclusion (Starne’s procedure) for severe neonatal Ebstein’s anomaly. Oper Tech 2008, 13:91–100.

    Google Scholar 

  11. Sano S, Ishino K, Kawada M, et al.: Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg 2002, 123:640–647.

    Article  PubMed  Google Scholar 

  12. Attie F, Rosas M, Rijlaarsdam M, et al.: The adult patient with Ebstein anomaly. Outcome in 72 unoperated patients. Medicine (Baltimore) 2000, 79:27–36.

    Article  CAS  Google Scholar 

  13. Brown ML, Dearani JA, Danielson GK, et al.: The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg 2008, 135:1120–1136.

    Article  PubMed  Google Scholar 

  14. Carpentier A, Chavaud S, Macé L, et al.: A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1998, 96:92–101.

    Google Scholar 

  15. Hetzer R, Nagdyman N, Ewart P, et al.: A modified repair technique for tricuspid incompetence in Ebstein’s anomaly. J Thorac Cardiovasc Surg 1998, 115:857–868.

    Article  PubMed  CAS  Google Scholar 

  16. 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, 17:722–728.

    Article  PubMed  CAS  Google Scholar 

  17. Wu Q, Huang Z: A new procedure for Ebstein’s anomaly. Ann Thorac Surg 2004, 77:470–476.

    Article  PubMed  Google Scholar 

  18. Chen JM, Mosca RS, Altmann K, et al.: Early and mediumterm results for repair of Ebstein anomaly. J Thorac Cardiovasc Surg 2004, 127:990–999.

    Article  PubMed  Google Scholar 

  19. Chauvaud S, Fuzellier JF, Berrebi A, et al.: Bi-directional cavopulmonary shunt associated with ventriculo and valvuloplasty in Ebstein’s anomaly: benefits in high risk patients. Eur J Cardiothorac Surg 1998, 13:514–519.

    Article  PubMed  CAS  Google Scholar 

  20. Chauvaud S: Ebstein’s malformation. Surgical treatment and results. J Thorac Cardiovasc Surg 2000, 48:220–223.

    Article  CAS  Google Scholar 

  21. Hancock Friesen CL, Chen R, Howlett JG, et al.: Posterior annular plication: tricuspid valve repair in Ebstein’s anomaly. Ann Thorac Surg 2004, 77:2167–2171.

    Article  PubMed  Google Scholar 

  22. van Son JAM, Kinzel P, Mohr FW: Pericardial patch augmentation of anterior tricuspid leaflet in Ebstein’s anomaly. Ann Thorac Surg 1998, 66:1831–1832.

    Article  PubMed  Google Scholar 

  23. Vargas FJ, Mengo G, Granja MA, et al.: Tricuspid annuloplasty and ventricular plication for Ebstein’s malformation. Ann Thorac Surg 1998, 65:1755–1757.

    Article  PubMed  CAS  Google Scholar 

  24. da Silva JP, Baumgratz FJ, 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, 133:215–223.

    Article  PubMed  Google Scholar 

  25. Dearani JA, Bacha E, da Silva JP: Cone reconstruction of the tricuspid valve for Ebstein’s anomaly: anatomic repair. Oper Tech Thorac Cardiovasc Surg 2008, 13:109–125.

    Article  Google Scholar 

  26. Kiziltan HT, Theodoro DA, Warnes CA, et al.: Late results of bioprosthetic tricuspid valve replacement in Ebstein’s anomaly. Ann Thorac Surg 1998, 66:1539–1545.

    Article  PubMed  CAS  Google Scholar 

  27. Brown ML, Dearani JA, Danielson GK, et al.: Comparison of the outcome of porcine bioprosthetic versus mechanical prosthetic replacement of the tricuspid valve in the Ebstein anomaly. Am J Cardiol 2009, 103:555–561.

    Article  PubMed  Google Scholar 

  28. 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, 66:1546–1550.

    Article  PubMed  CAS  Google Scholar 

  29. Quiñonez 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, 133:1303–1310.

    Article  PubMed  Google Scholar 

  30. Malhotra SP, Reddy VM, Qiu K, et al.: Right ventricular unloading promotes durability of tricuspid valve repair in Ebstein’s anomaly: indications for a selective one and a half ventricle strategy [abstract]. Presented at the 45th Annual Meeting of the Society of Thoracic Surgeons. San Francisco, CA; January 26–28, 2009.

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Authors and Affiliations

  1. Division of Cardiovascular Surgery, Mayo Clinic, 200 First Street Southwest, Rochester, MN, 55905, USA

    Joseph A. Dearani

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  1. Morgan L. Brown
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  2. Joseph A. Dearani
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Correspondence to Joseph A. Dearani.

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Brown, M.L., Dearani, J.A. Ebstein malformation of the tricuspid valve: Current concepts in management and outcomes. Curr Treat Options Cardio Med 11, 396–402 (2009). https://doi.org/10.1007/s11936-009-0041-5

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