Tricuspid Valve Abnormalities

  • Daniel Tobler
  • Laura Jimenez-Juan
  • Andrew M. Crean
  • Rachel M. WaldEmail author
Part of the Medical Radiology book series (MEDRAD)


Tricuspid valve abnormalities are relatively rare lesions in the spectrum of congenital heart disease which can affect the leaflets, chordal apparatus, and/or papillary muscles of the tricuspid valve. Ebstein anomaly is the most common form of congenital tricuspid valve disease. Although outcomes remain poor in neonates with important tricuspid valve disease, Ebstein anomaly can be managed successfully in children, adolescents, and young adults with recent surgical advances and current interventional techniques. Comprehensive cardiac imaging is essential to allow for accurate diagnosis and appropriate decision-making, and should always be performed by an advanced cardiac imaging specialist with expertise in congenital heart disease. In this chapter, anatomical features pertaining to the range of congenital heart defects which affect the tricuspid valve are reviewed, current imaging techniques are detailed, and contemporary approaches to management are discussed.


  1. Allwork SP, Bentall HH, Becker AE, Cameron H, Gerlis LM, Wilkinson JL, Anderson RH (1976) Congenitally corrected transposition of the great arteries: morphologic study of 32 cases. Am J Cardiol 38:910–923CrossRefGoogle Scholar
  2. Anderson KR, Danielson GK, McGoon DC, Lie JT (1978) Ebstein’s anomaly of the left-sided tricuspid valve: pathological anatomy of the valvular malformation. Circulation 58:187–191CrossRefGoogle Scholar
  3. Attenhofer Jost CH, Connolly HM, O’Leary PW, Warnes CA, Tajik AJ, Seward JB (2005) Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc 80:361–368CrossRefGoogle Scholar
  4. Attenhofer Jost CH, Edmister WD, Julsrud PR, Dearani JA, Savas Tepe M, Warnes CA, Scott CG, Anavekar NS, Ammash NM, Connolly HM (2012a) Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein’s anomaly. Int J Cardiovasc Imaging 28:1147–1159CrossRefGoogle Scholar
  5. Attenhofer Jost CH, Connolly HM, Scott CG, Burkhart HM, Warnes CA, Dearani JA (2012b) Outcome of cardiac surgery in patients 50 years of age or older with Ebstein anomaly: survival and functional improvement. J Am Coll Cardiol 59:2101–2106CrossRefGoogle Scholar
  6. Attie F, Rosas M, Rijlaarsdam M, Buendia A, Zabal C, Kuri J, Granados N (2000) The adult patient with Ebstein anomaly. Outcome in 72 unoperated patients. Medicine 79:27–36CrossRefGoogle Scholar
  7. Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, Gatzoulis MA, Gohlke-Baerwolf C, Kaemmerer H, Kilner P, Meijboom F, Mulder BJ, Oechslin E, Oliver JM, Serraf A, Szatmari A, Thaulow E, Vouhe PR, Walma E, Task Force on the Management of Grown-Up Congenital Heart Disease of the European Society of C, Association for European Paediatric C and Guidelines ESCCfP (2010) ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 31:2915–2957CrossRefGoogle Scholar
  8. Bove EL, Hirsch JC, Ohye RG, Devaney EJ (2009) How I manage neonatal Ebstein’s anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu:63–65CrossRefGoogle Scholar
  9. Brown ML, Dearani JA, PEF D (2011) Ebstein anomaly. In: Gatzoulis MA, Webb G, PEF D (eds) Diagnosis and management of adult congenital heart disease. Elsevier, Philadelphia, pp 288–294CrossRefGoogle Scholar
  10. Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RK, Deanfield JE (1992) Outcome in neonates with Ebstein’s anomaly. J Am Coll Cardiol 19:1041–1046CrossRefGoogle Scholar
  11. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P, Somerville J, Deanfield JE (1994) Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 23:170–176CrossRefGoogle Scholar
  12. Correa-Villasenor A, Ferencz C, Neill CA, Wilson PD, Boughman JA (1994) Ebstein’s malformation of the tricuspid valve: genetic and environmental factors. The Baltimore-Washington Infant Study Group. Teratology 50:137–147CrossRefGoogle Scholar
  13. Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr (1992) Operative treatment of Ebstein’s anomaly. J Thorac Cardiovasc Surg 104:1195–1202PubMedGoogle Scholar
  14. Edwards WD (1993) Embryology and pathologic features of Ebstein’s anomaly. Prog Pediatr Cardiol 2:5–15CrossRefGoogle Scholar
  15. Fratz S, Janello C, Muller D, Seligmann M, Meierhofer C, Schuster T, Schreiber C, Martinoff S, Hess J, Kuhn A, Vogt M, Stern H (2012) The functional right ventricle and tricuspid regurgitation in Ebstein’s anomaly. Int J Cardiol 167:258–261CrossRefGoogle Scholar
  16. Freedom RM, Mawson JB, Yoo SJ, Benson LN (1997) Ebstein’s malformation of the tricuspid valve. Armonk, NY, Futura Publishing CoGoogle Scholar
  17. 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, DB ME, Tworetzky W (2015) Outcomes and predictors of perinatal mortality in fetuses with Ebstein anomaly or tricuspid valve dysplasia in the current era: a multicenter study. Circulation 132:481–489CrossRefGoogle Scholar
  18. Ho SY, Rigby ML, Anderson RH (2005) Ebstein malformation. In: Ho SY, Rigby ML, Anderson RH (eds) Echocardiography in congenital heart disease made simple. Imperial College Press, London, pp 214–218CrossRefGoogle Scholar
  19. Horvath P, Szufladowicz M, de Leval MR, Elliott MJ, Stark J (1994) Tricuspid valve abnormalities in patients with atrioventricular discordance: surgical implications. Ann Thorac Surg 57:941–945CrossRefGoogle Scholar
  20. Hosch O, Sohns JM, Nguyen TT, Lauerer P, Rosenberg C, Kowallick JT, Kutty S, Unterberg C, Schuster A, Fasshauer M, Staab W, Paul T, Lotz J, Steinmetz M (2014) 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 7:601–609CrossRefGoogle Scholar
  21. Ibrahim M, Tsang VT, Caruana M, Hughes ML, Jenkyns S, Perdreau E, Giardini A, Marek J (2015) Cone reconstruction for Ebstein’s anomaly: patient outcomes, biventricular function, and cardiopulmonary exercise capacity. J Thorac Cardiovasc Surg 149:1144–1150CrossRefGoogle Scholar
  22. Khositseth A, Danielson GK, Dearani JA, Munger TM, Porter CJ (2004) Supraventricular tachyarrhythmias in Ebstein anomaly: management and outcome. J Thorac Cardiovasc Surg 128:826–833CrossRefGoogle Scholar
  23. Kuhn A, Meierhofer C, Rutz T, Rondak IC, Rohlig C, Schreiber C, Fratz S, Ewert P, Vogt M (2016) 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 17:930–935CrossRefGoogle Scholar
  24. Lange R, Burri M, Eschenbach LK, Badiu CC, da Silva JP, Nagdyman N, Fratz S, Horer J, Kuhn A, Schreiber C, Vogt MO (2015) Da Silva’s cone repair for Ebstein’s anomaly: effect on right ventricular size and function. Eur J Cardiothorac Surg 48:316–320. discussion 320–1CrossRefGoogle Scholar
  25. Lee CM, Sheehan FH, Bouzas B, Chen SS, Gatzoulis MA, Kilner PJ (2012) The shape and function of the right ventricle in Ebstein’s anomaly. Int J Cardiol 167:704–710CrossRefGoogle Scholar
  26. Li X, Wang SM, Schreiber C, Cheng W, Lin K, Sun JY, Yang D, Luo SH, An Q, Chen YC (2016) More than valve repair: effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly. Int J Cardiol 206:131–137CrossRefGoogle Scholar
  27. Samanek M, Voriskova M (1999) Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study. Pediatr Cardiol 20:411–417CrossRefGoogle Scholar
  28. Seward JB (1993) Ebstein’s anomaly: ultrasound imaging and hemodynamic evaluation. Echocardiography 10:641–664CrossRefGoogle Scholar
  29. da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM, Soares AM, Moreira LF, Barbero-Marcial M (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
  30. Silverman NH, Gerlis LM, Horowitz ES, Ho SY, Neches WH, Anderson RH (1995) Pathologic elucidation of the echocardiographic features of Ebstein’s malformation of the morphologically tricuspid valve in discordant atrioventricular connections. Am J Cardiol 76:1277–1283CrossRefGoogle Scholar
  31. Tobler D, Yalonetsky S, Crean AM, Granton JT, Burchill L, Silversides CK, Wald RM (2011) Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol 165:146–150CrossRefGoogle Scholar
  32. Wald RM, Adatia I, Van Arsdell GS, Hornberger LK (2005) Relation of limiting ductal patency to survival in neonatal Ebstein’s anomaly. Am J Cardiol 96(6):851CrossRefGoogle Scholar
  33. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules JW, Graham TP Jr, Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GDACC (2008) 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 118:e714–e833PubMedGoogle Scholar
  34. Yalonetsky S, Tobler D, Greutmann M, Crean AM, Wintersperger BJ, Nguyen ET, Oechslin EN, Silversides CK, Wald RM (2011) Cardiac magnetic resonance imaging and the assessment of ebstein anomaly in adults. Am J Cardiol 107:767–773CrossRefGoogle Scholar
  35. Yetman AT, Freedom RM, McCrindle BW (1998) Outcome in cyanotic neonates with Ebstein’s anomaly. Am J Cardiol 81:749–754CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  • Daniel Tobler
    • 1
  • Laura Jimenez-Juan
    • 2
    • 3
  • Andrew M. Crean
    • 4
  • Rachel M. Wald
    • 5
    • 6
    Email author
  1. 1.Department of CardiologyUniversity Hospital Basel, University of BaselBaselSwitzerland
  2. 2.Department of Medical ImagingUniversity of TorontoTorontoCanada
  3. 3.Department of Medical ImagingSunnybrook Health Sciences CentreTorontoCanada
  4. 4.Sanghvi Endowed Chair in Cardiovascular ImagingUniversity of Cincinnati Medical Center and Cincinnati Children’s Hospital Medical CenterCincinnatiUSA
  5. 5.Division of Cardiology, Peter Munk Cardiac CentreUniversity Health NetworkTorontoCanada
  6. 6.Departments of Pediatrics, Medicine, Medical Imaging and Obstetrics & GynecologyUniversity of TorontoTorontoCanada

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