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Ebstein’s Disease of the Tricuspid Valve

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Critical Care of Children with Heart Disease

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Abstract

Ebstein’s disease of the tricuspid valve is a congenital cardiac anomaly that occurs with an incidence of approximately 1–5 in every 200,000 live newborns, therefore representing 1% of congenital cardiac defects. It is the most common etiology for congenital tricuspid regurgitation in the neonatal period as well as later in life. This chapter will summarize the main aspects related to diagnosis and management of the Ebstein’s disease of the tricuspid valve.

Steven P. Goldberg is deceased at the time of publication of this chapter.

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References

  1. Hardy KL, et al. Ebstein's anomaly: a functional concept and successful definitive repair. J Thorac Cardiovasc Surg. 1964;48:927–940.

    Article  CAS  PubMed  Google Scholar 

  2. McElhinney DB, et al. Improving outcomes in fetuses and neonates with congenital displacement (Ebstein's malformation) or dysplasia of the tricuspid valve. Am J Cardiol. 2005;96(4):582–586.

    Article  PubMed  Google Scholar 

  3. van Son JA, Konstantinov IE, Zimmermann V. Wilhelm Ebstein and Ebstein's malformation. Eur J Cardiothorac Surg. 2001;20(5):1082–1085.

    Article  PubMed  Google Scholar 

  4. Attenhofer Jost CH, et al. Ebstein's anomaly. Circulation. 2007;115(2):277–285.

    Article  PubMed  Google Scholar 

  5. Martinez RM, O'Leary PW, Anderson RH. Anatomy and echocardiography of the normal and abnormal tricuspid valve. Cardiol Young. 2006;16(Suppl 3):4–11.

    Article  PubMed  Google Scholar 

  6. Carpentier A, et al. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1988;96(1):92–101.

    Article  CAS  PubMed  Google Scholar 

  7. Celermajer DS, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23(1):170–176.

    Article  CAS  PubMed  Google Scholar 

  8. Danielson GK, et al. Operative treatment of Ebstein's anomaly. J Thorac Cardiovasc Surg. 1992;104(5):1195–1202.

    Article  CAS  PubMed  Google Scholar 

  9. Lillehei CW, Kalke BR, Carlson RG. Evolution of corrective surgery for Ebstein's anomaly. Circulation. 1967;35(4 Suppl):I111–I118.

    CAS  PubMed  Google Scholar 

  10. Holmes D, Kern MJ. Simplified intracardiac electrocardiography for Ebstein's anomaly. Catheter Cardiovasc Interv. 2002;55(3):367–368.

    Article  CAS  PubMed  Google Scholar 

  11. Khositseth A, et al. Supraventricular tachyarrhythmias in Ebstein anomaly: management and outcome. J Thorac Cardiovasc Surg. 2004;128(6):826–833.

    Article  PubMed  Google Scholar 

  12. Ahmed S, et al. Transesophageal three-dimensional echocardiographic demonstration of Ebstein's anomaly. Echocardiography. 2003;20(3):305–307.

    Article  PubMed  Google Scholar 

  13. Chauvaud SM, et al. Ventricular volumes in Ebstein's anomaly: x-ray multislice computed tomography before and after repair. Ann Thorac Surg. 2006;81(4):1443–1449.

    Article  PubMed  Google Scholar 

  14. Marino JP, et al. Echocardiography and color-flow mapping evaluation of a new reconstructive surgical technique for Ebstein's anomaly. Circulation. 1989;80(3 Pt 1):I197–I202.

    CAS  PubMed  Google Scholar 

  15. Daliento L, et al. Angiographic and morphologic features of the left ventricle in Ebstein's malformation. Am J Cardiol. 1997;80(8):1051–1059.

    Article  CAS  PubMed  Google Scholar 

  16. Atz AM, et al. Diagnostic and therapeutic uses of inhaled nitric oxide in neonatal Ebstein's anomaly. Am J Cardiol. 2003;91(7):906–908.

    Article  CAS  PubMed  Google Scholar 

  17. Bruckheimer E, et al. Inhaled nitric oxide therapy in a critically ill neonate with Ebstein's anomaly. Pediatr Cardiol. 1998;19(6):477–479.

    Article  CAS  PubMed  Google Scholar 

  18. Wald RM, et al. Relation of limiting ductal patency to survival in neonatal Ebstein's anomaly. Am J Cardiol. 2005;96(6):851–856.

    Article  PubMed  Google Scholar 

  19. Chauvaud S, et al. Ebstein's anomaly: repair based on functional analysis. Eur J Cardiothorac Surg. 2003;23(4):525–531.

    Article  CAS  PubMed  Google Scholar 

  20. Hetzer R, et al. A modified repair technique for tricuspid incompetence in Ebstein's anomaly. J Thorac Cardiovasc Surg. 1998;115(4):857–868.

    Article  CAS  PubMed  Google Scholar 

  21. Hiramatsu Y, et al. Biventricular repair of Ebstein's anomaly with pulmonary atresia in a low birth weight neonate. J Card Surg. 2006;21(4):421–422.

    Article  PubMed  Google Scholar 

  22. Hunter SW, Lillehei CW. Ebstein's malformation of the tricuspid valve. study of a case together with suggestion of a new form of surgical therapy. Dis Chest. 1958;33(3):297–304.

    Article  CAS  PubMed  Google Scholar 

  23. Marianeschi SM, et al. Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading. Ann Thorac Surg. 1998;66(5):1546–1550.

    Article  CAS  PubMed  Google Scholar 

  24. Quaegebeur JM, et al. Surgery for Ebstein's anomaly: the clinical and echocardiographic evaluation of a new technique. J Am Coll Cardiol. 1991;17(3):722–728.

    Article  CAS  PubMed  Google Scholar 

  25. Reemtsen BL, et al. Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg. 2006;132(6):1285–1290.

    Article  PubMed  Google Scholar 

  26. Sarris GE, et al. Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association. J Thorac Cardiovasc Surg. 2006;132(1):50–57.

    Article  PubMed  Google Scholar 

  27. Starnes VA, et al. Ebstein's anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg. 1991;101(6):1082–1087.

    Article  CAS  PubMed  Google Scholar 

  28. Sano S, et al. Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg. 2002;123(4):640–647.

    Article  PubMed  Google Scholar 

  29. Vargas FJ, et al. Tricuspid annuloplasty and ventricular plication for Ebstein's malformation. Ann Thorac Surg. 1998;65(6):1755–1757.

    Article  CAS  PubMed  Google Scholar 

  30. Wu Q, Huang Z. A new procedure for Ebstein's anomaly. Ann Thorac Surg. 2004;77(2):470–476. discussion 476

    Article  PubMed  Google Scholar 

  31. Knott-Craig CJ, et al. Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium-term follow-up. Ann Thorac Surg. 2000;69(5):1505–1510.

    Article  CAS  PubMed  Google Scholar 

  32. Weinberg M Jr, et al. Surgical palliation in patients with Ebstein's anomaly and congenital hypoplasia of the right ventricle. J Thorac Cardiovasc Surg. 1960;40:310–320.

    Article  PubMed  Google Scholar 

  33. Chauvaud S, 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(5):514–519.

    Article  CAS  PubMed  Google Scholar 

  34. Endo M, et al. Blalock-Taussig shunt for a neonatal case of Ebstein's anomaly with unobstructed right ventricular outflow tract. Pediatr Cardiol. 2004;25(1):56–57.

    Article  CAS  PubMed  Google Scholar 

  35. Kiziltan HT, et al. Late results of bioprosthetic tricuspid valve replacement in Ebstein's anomaly. Ann Thorac Surg. 1998;66(5):1539–1545.

    Article  CAS  PubMed  Google Scholar 

  36. Boston US, et al. Tricuspid valve repair for Ebstein's anomaly in young children: a 30-year experience. Ann Thorac Surg. 2006;81(2):690–695; discussion 695-6.

    Article  PubMed  Google Scholar 

  37. Danielson GK, Maloney JD, Devloo RA. Surgical repair of Ebstein's anomaly. Mayo Clin Proc. 1979;54(3):185–192.

    CAS  PubMed  Google Scholar 

  38. Dearani JA, Danielson GK. Surgical management of Ebstein's anomaly in the adult. Semin Thorac Cardiovasc Surg. 2005;17(2):148–154.

    Article  PubMed  Google Scholar 

  39. Chauvaud S. Ebstein's malformation. surgical treatment and results. Thorac Cardiovasc Surg. 2000;48(4):220–223.

    Article  CAS  PubMed  Google Scholar 

  40. da Silva JP, 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(1):215–223.

    Article  PubMed  Google Scholar 

  41. Bove EL, et al. How I manage neonatal Ebstein's anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009;63–65.

    Google Scholar 

  42. Pizarro C, Bhat MA, Temple J. Cone reconstruction and ventricular septal defect closure for neonatal Ebstein's anomaly. Multimed Man Cardiothorac Surg. 2012;2012:mms014

    PubMed  Google Scholar 

  43. Silva JP, et al. Cone reconstruction in Ebstein's anomaly repair: early and long-term results. Arq Bras Cardiol. 2011;97(3):199–208.

    Article  PubMed  Google Scholar 

  44. Anderson HN, et al. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenit Heart Dis. 2014;9(3):266–271.

    Article  PubMed  Google Scholar 

  45. Vogel M, et al. Ebstein's malformation of the tricuspid valve: short-term outcomes of the “cone procedure” versus conventional surgery. Congenit Heart Dis. 2012;7(1):50–58.

    Article  PubMed  Google Scholar 

  46. Therrien J, et al. Right ventricular long axis function in adults and children with Ebstein's malformation. Int J Cardiol. 2000;73(3):243–249.

    Article  CAS  PubMed  Google Scholar 

  47. Greason KL, et al. Surgical management of atrial tachyarrhythmias associated with congenital cardiac anomalies: Mayo Clinic experience. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2003;6:59–71.

    Article  PubMed  Google Scholar 

  48. Theodoro DA, et al. Right-sided maze procedure for right atrial arrhythmias in congenital heart disease. Ann Thorac Surg. 1998;65(1):149–153. discussion 153–4

    Google Scholar 

  49. Bockeria L, et al. Advantages and disadvantages of one-stage and two-stage surgery for arrhythmias and Ebstein's anomaly. Eur J Cardiothorac Surg. 2005;28(4):536–540.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Pediatric Cardiac Critical Care, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA

    Wonshill Koh

  2. Department of Pediatric Cardiothoracic Surgery, UPMC – Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Jose Pedro da Silva

  3. Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver School of Medicine, Aurora, CO, USA

    Jonathan Kaufman & Jill Ibrahim

  4. Cardiac Intensive Care, Heart Institute, Aurora, CO, USA

    Jonathan Kaufman & Jill Ibrahim

  5. Clinique des Grangettes, Chêne-Bougeries, Geneva, Switzerland

    Cécile Tissot

  6. ECMO Porgram, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver School of Medicine, Aurora, CO, USA

    Shannon Buckvold

  7. Pulmonary Hypertension Program, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver School of Medicine, Aurora, CO, USA

    Dunbar Ivy

  8. The Heart Institute, Aurora, CO, USA

    Dunbar Ivy

  9. Perioperative and Pain Medicine (Pediatrics), The Stanford University Medical Center, Palo Alto, CA, USA

    Lisa Wise-Faberowski

  10. Mitera Hospital, Athens, Greece

    Afksendyios Kalangos

  11. Department of Pediatric Cardiothoracic Surgery, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Victor O. Morell

  12. Associate Medical Director, CHCO Heart Institute, Head, Pediatric Cardiac Critical Care Program & Inpatient Services, Director, Cardiac Intensive Care Unit, Children’s Hospital Colorado, Tenured Professor of Pediatrics, Pediatric Cardiology and Intensive Care, University of Colorado Denver, School of Medicine, Aurora, CO, USA

    Eduardo M. da Cruz

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  1. Wonshill Koh
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Corresponding author

Correspondence to Eduardo M. da Cruz .

Editor information

Editors and Affiliations

  1. Chief, Division of Cardiac Critical Care Medicine Executive Director, Telemedicine Co-director, Children’s National Heart Institute, Professor of Pediatrics, George Washington University School of Medicine, Children’s National Health System, Washington, DC, USA

    Ricardo A. Munoz

  2. Department of Pediatric Cardiothoracic Surgery, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Victor O. Morell

  3. Associate Medical Director, CHCO Heart Institute Head, Pediatric Cardiac Critical Care Program & Inpatient Services Director, Cardiac Intensive Care Unit Children’s Hospital Colorado, Tenured Professor of Pediatrics, Pediatric Cardiology and Intensive Care University of Colorado Denver, School of Medicine, Aurora, CO, USA

    Eduardo M. da Cruz

  4. Department of Pharmacy, Pediatric Critical Care, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Carol G. Vetterly

  5. Department of Pediatric Cardiothoracic Surgery, UPMC – Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Jose Pedro da Silva

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Koh, W. et al. (2020). Ebstein’s Disease of the Tricuspid Valve. In: Munoz, R., Morell, V., da Cruz, E., Vetterly, C., da Silva, J. (eds) Critical Care of Children with Heart Disease . Springer, Cham. https://doi.org/10.1007/978-3-030-21870-6_36

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  • DOI: https://doi.org/10.1007/978-3-030-21870-6_36

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-21869-0

  • Online ISBN: 978-3-030-21870-6

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