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Ebstein’s Anomaly

  • Viktor Hraška
  • Peter Murín

Abstract

Ebstein’s anomaly is a rare congenital heart malformation of the right ventricle and tricuspid valve. Failure of delamination of the tricuspid valve, especially of the posterior and septal leaflets, from the underlying myocardium, results in a variable degree of tricuspid valve and right ventricular deformity. The natural history is influenced by a high rate of intrauterine and neonatal death, especially when the anomaly is associated with severe deformity of the tricuspid valve and cyanosis. At the other end of the spectrum, patients with mild dysfunction of the tricuspid valve can remain asymptomatic until late in adult life. Management therefore requires a choice between a conservative approach and operative intervention. Currently available surgical techniques have demonstrated survival and functional benefits to patients with a greater severity of Ebstein’s anomaly.

Keywords

Patent Ductus Arteriosus Atrial Septal Defect Tricuspid Valve Tricuspid Annulus Atrioventricular Septal Defect 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Supplementary material

Clip 1: Preoperative findings.

Clip 2: Assessment of anatomy.

Clip 3: Detachment of the leaflets of the tricuspid valve.

Clip 4: “Correction” of failure of delamination.

Clip 5: Creating a cone.

Clip 6: Exclusion of the atrialized right ventricle.

Clip 7: Attachment of the tricuspid valve to the annulus.

Clip 8: Plication of the annulus.

Clip 9: Final reattachment of the base of the cone. In addition, the atrial septal defect is partially closed, leaving a 3-mm fenestration. The redundant right atrium is excised from each side of the atriotomy, and then the atriotomy is closed.

Clip 10: The echocardiogram before discharge.

Fullversion

Clip 1: Assessment of the extra- and intracardiac anatomy.

Clip 2: Detachment of the leaflets of the tricuspid valve.

Clip 3: Detachment of the linear attachments of the posterior leaflet.

Clip 4: Detachment of the septal leaflet.

Clip 5: Creating a cone.

Clip 6: Attachment of the tricuspid valve to the annulus.

Clip 7: Lateral annuloplasty.

Clip 8: Atrial septal defect closure and reduction atriotomy.

Clip 9: The echocardiogram before discharge.

Fullversion

Recommended Reading

  1. Attenhofer Jost CH, Connolly HM et al (2007) Ebstein’s anomaly. Circulation 115:277–285PubMedCrossRefGoogle Scholar
  2. Badiu CC, Schreiber C, Horer J et al (2010) Early timing of surgical intervention in patients with Ebstein’s anomaly predicts superior long-term outcome. Eur J Cardiothorac Surg 37:186–192PubMedCrossRefGoogle Scholar
  3. Brown ML, Dearani JA, Danielson GK et al (2008) The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg 135:1120–1136PubMedCrossRefGoogle Scholar
  4. Chauvaud S, Carpentier A (2007) Ebstein’s anomaly: the Broussais approach. MMCTS. doi:10.1510/mmcts.2007.003038Google Scholar
  5. Dearani JA, Danielson GK (2005) Surgical management of Ebstein’s anomaly in the adult. Semin Thorac Cardiovasc Surg 17:148–154Google Scholar
  6. Dearani JA, O’Leary PW, Danielson GK (2006) Surgical treatment of Ebstein’s malformation: state of the art in 2006. Cardiol Young 16:12–20CrossRefGoogle Scholar
  7. Dearani JA, Bacha E, da Silva JP (2008) Cone reconstruction of the tricuspid valve for Ebstein’s anomaly: anatomic repair. Oper Tech Thorac Cardiovasc Surg 13:109–125Google Scholar
  8. Ebstein W (1866) Über einen sehr seltenen Fall von Insuffizienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Arch Anat Physiol 7:238–254Google Scholar
  9. Knott-Craig CJ (2008) Management of neonatal Ebstein’s anomaly. Oper Tech Thorac Cardiovasc Surg 13:101–108Google Scholar
  10. Knott-Craig CJ, Goldberg SP, Overholt ED et al (2007) Repair of neonates and young infants with Ebstein’s anomaly and related disorders. Ann Thorac Surg 84:587–593PubMedCrossRefGoogle Scholar
  11. O’Leary PW, Dearani JA, Anderson RH (2010) In: Anderson RH, Baker EJ, Penny D et al (eds) Pediatric cardiology, 3rd edn. Churchill-Livingstone, London, pp 713–730Google Scholar
  12. Reemtsen BL, Fagan BT, Wells WJ et al (2006) Current surgical therapy for Ebstein’s anomaly in neonates. J Thorac Cardiovasc Surg 132:1285–1289PubMedCrossRefGoogle Scholar
  13. Sano S, Ishino K, Kawada M et al (2002) Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg 123:640–647PubMedCrossRefGoogle Scholar
  14. Silva da JP, Baumgratz JF, da Fonseca L et al (2007) The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg 133:215–223CrossRefGoogle Scholar
  15. Ullmann MV, Born S, Sebening C et al (2004) Ventricularization of the atrialized chamber: a concept of Ebstein’s anomaly repair. Ann Thorac Surg 78:918–924PubMedCrossRefGoogle Scholar
  16. Wald RM, Tham EB, McCrindle BW et al (2007) Outcome after prenatal diagnosis of tricuspid atresia: a multicenter experience. Am Heart J 153:772–778PubMedCrossRefGoogle Scholar
  17. Wu Q, Huang Z (2004) A new procedure for Ebstein’s anomaly. Ann Thorac Surg 77:470–476PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Viktor Hraška
    • 1
  • Peter Murín
    • 1
  1. 1.Department of Cardiac SurgeryGerman Pediatric Heart Centre, Sankt AugustinSankt AugustinGermany

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