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Right Ventricular Function in Ebstein’s Malformation

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Right Ventricular Physiology, Adaptation and Failure in Congenital and Acquired Heart Disease

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Abstract

Ebstein’s anomaly is a developmental anomaly of the tricuspid valve that primarily involves failure of delamination of the leaflets. However this label encompasses a huge range of valve and ventricular components, in terms of morphology, cellular composition and function, and therefore a great spectrum of haemodynamic situations. On pathologic analysis, increased fibrosis of the right and left ventricular myocardium is a common finding. This is related to intrinsic disease and is progressive, resulting from the abnormal loading conditions for both ventricles. Assessing the underlying function of the RV myocardium is confounded by the loading conditions, and the load-dependency of all conventional echocardiographic and cardiac MRI parameters. Surgical repair of the tricuspid valve, with procedures such as the cone reconstruction, promises to improve TV function and ventricular mechanics. Prudent case selection for this type of surgical repair is crucial and long-term comparative outcome is not yet available, but this type of intervention offers a unique opportunity to examine RV function within the complex haemodynamics of this valve and ventricular anomaly.

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References

  1. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein’s anomaly. Circulation. 2007;115:277–85.

    Article  PubMed  Google Scholar 

  2. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P, Somerville J, Deanfield JE. Ebsteim’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23(1):170–6.

    Article  CAS  PubMed  Google Scholar 

  3. Schreiber C, Cook A, Ho SY, Augustin N, Anderson RH. Morphologic spectrum of Ebstein’s malformation: revisitation relative to surgical repair. J Thorac Cardiovasc Surg. 1999;117:148–55.

    Article  CAS  PubMed  Google Scholar 

  4. Edwards WD. Embryology and pathologic features of Ebstein’s anomaly. Prog Pediatr Cardiol. 1993;2:5–15.

    Article  Google Scholar 

  5. Anderson KR, Lie JT. Pathologic anatomy of Ebstein’s anomaly of the heart revisited. Am J Cardiol. 1978;41:739–45.

    Article  CAS  PubMed  Google Scholar 

  6. Anderson KR, Zuberbuhler JR, Anderson RH, Becker AE, Lie JT. Morphologic spectrum of Ebstein’s anomaly of the heart: a review. Mayo Clin Proc. 1979;54:174–80.

    CAS  PubMed  Google Scholar 

  7. Lee AH, Moore IE, Fagg NL, Cook AC, Kakadekar AP, Allan LD, Keeton BR, Anderson RH. Histological changes in the left and right ventricle in hearts with Ebstein’s malformation and tricuspid valvar dysplasia: A morphometric study of patients dying in the fetal and perinatal periods. Cardiovasc Pathol. 1995;4:19–24.

    Article  CAS  PubMed  Google Scholar 

  8. Luo C, Ware DL, Zwischenberger JB, Clark JW Jr. A mechanical model of the human heart relating septal function to myocardial work and energy. Cardiovasc Eng. 2008;8:174–84.

    Article  CAS  PubMed  Google Scholar 

  9. Goleski PJ, Sheehan FH, Chen SSM, Kilner PJ, Gatyoulis MA. The shape and function of the left ventricle in Ebstein’s anomaly. Int J Cardiol. 2014;171:404–12.

    Article  PubMed  Google Scholar 

  10. Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RK, Deanfield JE. Outcome in neonates with Ebstein’s anomaly. J Am Coll Cardiol. 1992;19:1041–6.

    Article  CAS  PubMed  Google Scholar 

  11. Yater WM, Shapiro MJ. Congenital displacement of the tricuspidvalve (Ebstein’s disease): review and report of a case with electrocardiographic abnormalities and detailed histologic study of the conduction system. Ann Intern Med. 1937;11:1043–62.

    Article  Google Scholar 

  12. Rossi L, Thiene G. Mild Ebstein’s anomaly associated with supraventricular tachycardia and sudden death: clinicomorphologic features in 3 patients. Am J Cardiol. 1984;53:332–4.

    Article  CAS  PubMed  Google Scholar 

  13. Chauvaud SM, Brancaccio G, Alain F, Carpentier AF. Cardiac arrhythmia in patients undergoing surgical repair of Ebstein’s anomaly. Ann Thorac Surg. 2001;71:1547–5.

    Article  CAS  PubMed  Google Scholar 

  14. Assenza GE, Valente AM, Geva T, Graham D, Pluchinotta FR, Sanders SP, Autore C, Volpe M, Landzberg MJ, Cecchin F. QRS duration and QRS fractionation on surface electrocardiogram are markers of right ventricular dysfunction and atrialization in patients with Ebstein anomaly. Eur Heart J. 2013;34:191–200.

    Article  Google Scholar 

  15. Freud LR, Escobar-Diaz MC, Kalish BT, Komarlu R, Puchalski MD, Jaeggi ET, Szwast AL, Freire G, Levasseur SM, Kavanaugh-McHugh A, Michelfelder EC, Moon-Grady AJ, Donofrio MT, Howley LW, Tierney ES, Cuneo BF, Morris SA, Pruetz JD, van der Velde ME, Kovalchin JP, Ikemba CM, Vernon MM, Samai C, Satou GM, Gotteiner NL, Phoon CK, Silverman NH, McElhinney DB, Tworetzki W. Outcomes and predictors of perinatal mortality in fetuses with Ebstein anomaly or tricuspid valve dysplasia in the current era: a multicenter study. Circulation. 2015;132:481–9.

    Article  PubMed  Google Scholar 

  16. McElhinney DB, Salvin JW, Colan SD, Thiagarajan R, Crawford EC, Marcus EN, del Nido PJ, Tworetzky W. Improving outcomes in fetuses and neonates with congenital displacement (Ebstein’s malformation) or dysplasia of the tricuspid valve. Am J Cardiol. 2005;96:582–6.

    Article  PubMed  Google Scholar 

  17. Postma AV, van Engelen K, van de Meerakker J, Rahman T, Probst S, Baars MJH, Bauer U, Pickardt T, Sperling SR, Berger F, Moorman AFM, Mulder BJM, Thierfelder L, Keavney B, Goodship J, Klaassen S. Mutations in the sarcomere gene MYH7 in Ebstein anomaly. Circ Cardiovasc Genet. 2011;4:43–50.

    Article  CAS  PubMed  Google Scholar 

  18. Davies RR, Pasquali SK, Jacobs ML, Jacobs JJ, Wallace AS, Pizarro C. Current spectrum of surgical procedures performed for Ebstein’s malformation: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg. 2013;96:1703–10.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Danielson GK, Fuster V. Surgical repair of Ebstein’s anomaly. Ann Surg. 1982;196:499–504.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Carpentier A, Chauvaud S, Mace L, Relland J, Mihaileanu S, Marino JP, Abry B, Buibourt P. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1988;96:92–101.

    CAS  PubMed  Google Scholar 

  21. da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM, Soares AM, Moreira LF, Barbero-Marcial M. The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg. 2007;133:215–23.

    Article  PubMed  Google Scholar 

  22. Anderson HN, Dearani JA, Said SM, Norris MD, Pundi KN, Miller AR, et al. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenit Heart Dis. 2014;9:266–71.

    Article  PubMed  Google Scholar 

  23. Dearani JA, Said SM, O’Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein’s malformation: lessons learned with cone reconstruction. Ann Thorac Surg. 2013;95:220–6. Discussion 6–8.

    Article  PubMed  Google Scholar 

  24. Vogel M, Marx GR, Tworetzky W, Cecchin F, Graham D, Mayer JE, et al. Ebstein’s malformation of the tricuspid valve: short-term outcomes of the “cone procedure” versus conventional surgery. Congenit Heart Dis. 2012;7:50–8.

    Article  PubMed  Google Scholar 

  25. Kostenburger J, Everett AD, Stueger HP, Heinzl B, Gamillscheg A, Cvirn G, Boysen A, Fandl A, Nagel B. Right ventricular function in infants, children and adolescents: reference values of the tricuspid annular plane systolic excursion (TAPSE) in 640 Healthy Patients and Calculation of z Score Values. Am Soc Echocardiogr. 2009;22:715–9.

    Article  Google Scholar 

  26. Mertens LL, Friedberg MK. Imaging the right ventricle-current state of the art. Nat Rev Cardiol. 2010;7:551–63.

    Article  PubMed  Google Scholar 

  27. Kühn A, Meierhofer C, Rutz T, Rondak IC, Röhling C, Schreiber C, Fratz S, Ewert P, Vogt M. Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein’s anomaly: comparison with CMR-derived ejection fraction in 49 patients. Eur Heart J Cardiovasc Imaging. 2016;17(8):930–5.

    Article  PubMed  Google Scholar 

  28. Hosch O, Sohns JM, Nguyen T-T, Lauerer P, Rosenberg C, Kowallick JT, Kutty S, Unterberg C, Schuster A, Martin Fashauer M, Staab W, Paul T, Lotz J, Steinmetz M. The total right/left-volume index: a new and simplified cardiac magnetic resonance measure to evaluate the severity of Ebstein anomaly of the tricuspid valve. A comparison with heart failure markers from various modalities. Circ Cardiovasc Imaging. 2014;7:601–9.

    Article  PubMed  Google Scholar 

  29. Yalonetsky S, Tobler D, Greutmann M, Crean AM, Wintersperger BJ, Nguyen ET, Oechslin EN, Silversides CK, Wald RM. Cardiac magneticresonance imaging and the assessment of Ebstein anomaly in adults. Am J Cardiol. 2011;107:767–73.

    Article  PubMed  Google Scholar 

  30. Tobler D, Yalonetsky S, Crean AM, Granton JT, Burchill L, Silversides CK, Wald RM. Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol. 2013;165:146–50.

    Article  PubMed  Google Scholar 

  31. Hudsmith LE, Petersen SE, Francis JM, Robson MD, Neubauer S. Normal human left and right ventricular and left atrial dimensions using steady state free precession magnetic resonance imaging. J Cardiovasc Magn Reson. 2005;7:775–82.

    Article  PubMed  Google Scholar 

  32. Sievers B, Addo M, Breuckmann F, Barkhausen J, Erbel R. Reference right atrial function determined by steady-state free precession cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2007;9:807–14.

    Article  PubMed  Google Scholar 

  33. Sarikouch S, Peters B, Gutberlet M, Leismann B, Kelter-Kloepping A, Koerperich H, Kuehne T, Beerbaum P. Sex-specific pediatric percentiles for ventricular size and mass as reference values for cardiac MRI: assessment by steady-state free-precession and phase-contrast MRI flow. Circ Cardiovasc Imaging. 2010;3:65–76.

    Article  PubMed  Google Scholar 

  34. Sarikouch S, Koerperich H, Boethig D, Peters B, Lotz J, Gutberlet M, Beerbaum P, Kuehne T. Reference values for atrial size and function in children and young adults by cardiac MR: a study of the German competence network congenital heart defects. J Magn Reson Imaging. 2011;33:1028–39.

    Article  PubMed  Google Scholar 

  35. Ibrahim M, Tsang VT, Caruana M, Hughes ML, Jenkyns S, Perdreau E, Giardini A, Marek J. Cone reconstruction for Ebstein’s anomaly: patient outcomes, biventricular function, and cardiopulmonary exercise capacity. J Thorac Cardiovasc Surg. 2015;149:1144–50.

    Article  PubMed  Google Scholar 

  36. Lange R, Burri M, Eschenbach LK, Badiu CC, da Silva JP, Nagdyman N, Fratz S, Hörer J, Kühn A, Schreiber C, Vogt MO. Da Silvaś cone repair for Ebsteon’s anomaly: effect on right ventricular size and function. Eur J Cardiothorac Surg. 2015;48(2):316–20. Discussion 320–1.

    Article  PubMed  Google Scholar 

  37. Perdreau E, Tsang V, Hughes M, Ibrahim M, Kataria S, Janagarajan K, Iriart X, Khambadkone S, Marek J. Change in biventricular function after cone repair of Ebstein’s anomaly: an echocardiographic study. Eur Heart J Cardiovasc Imaging. 2017; July 31. doi:10.1093/ehjci/jex186.

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Authors and Affiliations

  1. Paediatric Cardiology, Hospital for Sick Children, Great Ormond St, London, WC13JH, UK

    Jan Marek & Marina L. Hughes

  2. Cardiothoracic Surgery, Hospital for Sick Children, Great Ormond St, London, WC13JH, UK

    Victor Tsang

Authors
  1. Jan Marek
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  2. Marina L. Hughes
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  3. Victor Tsang
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Correspondence to Jan Marek .

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Editors and Affiliations

  1. Paediatric Cardiology, Labatt Family Heart Center Hospital for Sick Children, Toronto, Ontario, Canada

    Mark K. Friedberg

  2. Heart Institute, Cincinnati Children’s Hosp Med Center, Cincinnati, Ohio, USA

    Andrew N. Redington

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Marek, J., Hughes, M.L., Tsang, V. (2018). Right Ventricular Function in Ebstein’s Malformation. In: Friedberg, M., Redington, A. (eds) Right Ventricular Physiology, Adaptation and Failure in Congenital and Acquired Heart Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-67096-6_12

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  • DOI: https://doi.org/10.1007/978-3-319-67096-6_12

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  • Publisher Name: Springer, Cham

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  • Online ISBN: 978-3-319-67096-6

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