Tricuspid Valve: Congenital Abnormalities and Stenosis

  • Pei-Ni JoneEmail author
  • Shelby Kutty


Three-dimensional echocardiography (3DE) has incremental diagnostic value over two-dimensional echocardiography in the evaluation of the tricuspid valve. 3DE provides en face view of all leaflets of the tricuspid valve. The understanding of tricuspid valve leaflet morphology and mechanisms of valvular regurgitation from 3DE provides valuable insights into tricuspid valve function, and assists in the planning for surgical interventions. Congenital abnormalities of the tricuspid valve and illustrative clinical cases are presented in this chapter.


Three-dimensional echocardiography Tricuspid valve Tricuspid valve dysplasia Ebstein anomaly Tricuspid valve prolapse Functional abnormalities of the tricuspid valve Tricuspid valve in corrected transposition Tricuspid valve stenosis Tricuspid regurgitation. 

Supplementary material

Video 19.1

Normal tricuspid valve imaged by transesophageal echocardiography from a right atrial perspective (AVI 325 kb)

Video 19.2a

Thickened and dysplastic tricuspid valve leaflets from the apical four chamber view, with the heart rotated and cropped from behind (AVI 4871 kb)

Video 19.2b

This illustrates evaluation of thickened dysplastic tricuspid valve leaflets from the right ventricle up after cropping from a transthoracic three-dimensional full volume (AVI 4042 kb)

Video 19.3a

The tricuspid valve in Ebstein anomaly from a right ventricular perspective. Note the anterior leaflet displacement anteriorly into the right ventricular outflow tract (AVI 1871 kb)

Video 19.3b

Parasternal short axis view in Ebstein anomaly demonstrating the failure of delamination of the septal leaflet and the inability of tricuspid valve to coapt (AVI 1486 kb)

Video 19.4

Right atrial view of moderate to severe tricuspid regurgitation in Ebstein anomaly (AVI 3069 kb)

Video 19.5

Right ventricular view of the Ebstein tricuspid valve and the inability of leaflets to coapt (AVI 11248 kb)

Video 19.6a

Right atrial view of the prolapsing cleft tricuspid valve into the right atrium (AVI 10802 kb)

Video 19.6b

Transesophageal echocardiogram in the midesophageal view demonstrates the prolapse of the tricuspid valve into the right atrium (AVI 1000 kb)

Video 19.7

Severe tricuspid regurgitation of the cleft tricuspid valve with prolapse (AVI 868 kb)

Video 19.8a

This is prolapse of the anterior leaflet of the tricuspid valve demonstrated with three-dimensional transesophageal echocardiography in a patient with corrected transposition of the great arteries (AVI 2212 kb)

Video 19.8b

Straddling of the tricuspid valve shown by transthoracic subcostal three-dimensional echocardiography in a patient with corrected transposition of the great arteries (AVI 4420 kb)

Video 19.9

Three-dimensional transesophageal echocardiography in a stenotic bioprosthetic valve from the right atrial perspective (AVI 75902 kb)

Video 19.10

Three-dimensional transesophageal echocardiography of post-transcatheter tricuspid valve implant of a Melody valve within the Carpentier-Edwards valve (AVI 990 kb)


  1. 1.
    Silver MD, Lam JH, Ranganathan N, Wigle ED. Morphology of the human tricuspid valve. Circulation. 1971;43(3):333–48.CrossRefGoogle Scholar
  2. 2.
    Lamers WH, Viragh S, Wessels A, Moorman AF, Anderson RH. Formation of the tricuspid valve in the human heart. Circulation. 1995;91(1):111–21.CrossRefGoogle Scholar
  3. 3.
    Addetia K, Yamat M, Mediratta A, Medvedofsky D, Patel M, Ferrara P, et al. Comprehensive two-dimensional interrogation of the tricuspid valve using knowledge derived from three-dimensional echocardiography. J Am Soc Echocardiogr. 2016;29(1):74–82.CrossRefGoogle Scholar
  4. 4.
    Stankovic I, Daraban AM, Jasaityte R, Neskovic AN, Claus P, Voigt JU. Incremental value of the en face view of the tricuspid valve by two-dimensional and three-dimensional echocardiography for accurate identification of tricuspid valve leaflets. J Am Soc Echocardiogr. 2014;27(4):376–84.CrossRefGoogle Scholar
  5. 5.
    Ammash NM, Warnes CA, Connolly HM, Danielson GK, Seward JB. Mimics of Ebstein’s anomaly. Am Heart J. 1997;134(3):508–13.CrossRefGoogle Scholar
  6. 6.
    Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein’s anomaly. Circulation. 2007;115(2):277–85.CrossRefGoogle Scholar
  7. 7.
    Lev M, Liberthson RR, Joseph RH, Seten CE, Eckner FA, Kunske RD, et al. The pathologic anatomy of Ebstein’s disease. Arch Pathol. 1970;90(4):334–43.PubMedGoogle Scholar
  8. 8.
    Ho SY, Goltz D, McCarthy K, Cook AC, Connell MG, Smith A, et al. The atrioventricular junctions in Ebstein malformation. Heart. 2000;83(4):444–9.CrossRefGoogle Scholar
  9. 9.
    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(3):174–80.PubMedGoogle Scholar
  10. 10.
    Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, et al. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23(1):170–6.CrossRefGoogle Scholar
  11. 11.
    Eichhorn P, Ritter M, Suetsch G, von Segesser LK, Turina M, Jenni R. Congenital cleft of the anterior tricuspid leaflet with severe tricuspid regurgitation in adults. J Am Coll Cardiol. 1992;20(5):1175–9.CrossRefGoogle Scholar
  12. 12.
    Song JM, Fukuda S, Lever HM, Daimon M, Agler DA, Smedira NG, et al. Asymmetry of systolic anterior motion of the mitral valve in patients with hypertrophic obstructive cardiomyopathy: a real-time three-dimensional echocardiographic study. J Am Soc Echocardiogr. 2006;19(9):1129–35.CrossRefGoogle Scholar
  13. 13.
    Sukmawan R, Watanabe N, Ogasawara Y, Yamaura Y, Yamamoto K, Wada N, et al. Geometric changes of tricuspid valve tenting in tricuspid regurgitation secondary to pulmonary hypertension quantified by novel system with transthoracic real-time 3-dimensional echocardiography. J Am Soc Echocardiogr. 2007;20(5):470–6.CrossRefGoogle Scholar
  14. 14.
    Ton-Nu TT, Levine RA, Handschumacher MD, Dorer DJ, Yosefy C, Fan D, et al. Geometric determinants of functional tricuspid regurgitation: insights from 3-dimensional echocardiography. Circulation. 2006;114(2):143–9.CrossRefGoogle Scholar
  15. 15.
    Park YH, Song JM, Lee EY, Kim YJ, Kang DH, Song JK. Geometric and hemodynamic determinants of functional tricuspid regurgitation: a real-time three-dimensional echocardiography study. Int J Cardiol. 2008;124(2):160–5.CrossRefGoogle Scholar
  16. 16.
    Ring L, Rana BS, Kydd A, Boyd J, Parker K, Rusk RA. Dynamics of the tricuspid valve annulus in normal and dilated right hearts: a three-dimensional transoesophageal echocardiography study. Eur Heart J Cardiovasc Imaging. 2012;13(9):756–62.CrossRefGoogle Scholar
  17. 17.
    Spinner EM, Buice D, Yap CH, Yoganathan AP. The effects of a three-dimensional, saddle-shaped annulus on anterior and posterior leaflet stretch and regurgitation of the tricuspid valve. Ann Biomed Eng. 2012;40(5):996–1005.CrossRefGoogle Scholar
  18. 18.
    Takahashi K, Inage A, Rebeyka IM, Ross DB, Thompson RB, Mackie AS, et al. Real-time 3-dimensional echocardiography provides new insight into mechanisms of tricuspid valve regurgitation in patients with hypoplastic left heart syndrome. Circulation. 2009;120(12):1091–8.CrossRefGoogle Scholar
  19. 19.
    Kutty S, Colen T, Thompson RB, Tham E, Li L, Vijarnsorn C, et al. Tricuspid regurgitation in hypoplastic left heart syndrome: mechanistic insights from 3-dimensional echocardiography and relationship with outcomes. Circ Cardiovasc Imaging. 2014;7(5):765–72.CrossRefGoogle Scholar
  20. 20.
    Kutty S, Graney BA, Khoo NS, Li L, Polak A, Gribben P, et al. Serial assessment of right ventricular volume and function in surgically palliated hypoplastic left heart syndrome using real-time transthoracic three-dimensional echocardiography. J Am Soc Echocardiogr. 2012;25(6):682–9.CrossRefGoogle Scholar
  21. 21.
    Khoo NS, Smallhorn JF. Mechanism of valvular regurgitation. Curr Opin Pediatr. 2011;23(5):512–7.CrossRefGoogle Scholar
  22. 22.
    Svane S. Congenital tricuspid stenosis. A report on six autopsied cases. Scand J Thorac Cardiovasc Surg. 1971;5(3):232–8.CrossRefGoogle Scholar
  23. 23.
    Lewis T. Congenital tricuspid stenosis. Clin Sci. 1945;5(3–4):261–73.PubMedGoogle Scholar
  24. 24.
    Williams DB, Danielson GK, McGoon DC, Puga FJ, Mair DD, Edwards WD. Porcine heterograft valve replacement in children. J Thorac Cardiovasc Surg. 1982;84(3):446–50.PubMedGoogle Scholar
  25. 25.
    Zoghbi WA, Chambers JB, Dumesnil JG, Foster E, Gottdiener JS, Grayburn PA, et al. Recommendations for evaluation of prosthetic valves with echocardiography and doppler ultrasound: a report From the American Society of Echocardiography’s Guidelines and Standards Committee and the Task Force on Prosthetic Valves, developed in conjunction with the American College of Cardiology Cardiovascular Imaging Committee, Cardiac Imaging Committee of the American Heart Association, the European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography and the Canadian Society of Echocardiography, endorsed by the American College of Cardiology Foundation, American Heart Association, European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography, and Canadian Society of Echocardiography. J Am Soc Echocardiogr. 2009;22(9):975–1014, quiz 1082–4CrossRefGoogle Scholar
  26. 26.
    Connolly HM, Miller FA Jr, Taylor CL, Naessens JM, Seward JB, Tajik AJ. Doppler hemodynamic profiles of 82 clinically and echocardiographically normal tricuspid valve prostheses. Circulation. 1993;88(6):2722–7.CrossRefGoogle Scholar
  27. 27.
    Kobayashi Y, Nagata S, Ohmori F, Eishi K, Nakano K, Miyatake K. Serial doppler echocardiographic evaluation of bioprosthetic valves in the tricuspid position. J Am Coll Cardiol. 1996;27(7):1693–7.CrossRefGoogle Scholar
  28. 28.
    Aoyagi S, Nishi Y, Kawara T, Oryoji A, Kosuga K, Ohishi K. Doppler echocardiographic evaluation of St. Jude Medical valves in the tricuspid position. J Heart Valve Dis. 1993;2(3):279–86.PubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Pediatric CardiologyChildren’s Hospital Colorado, University of Colorado School of MedicineAuroraUSA
  2. 2.Taussig Heart Center, The Johns Hopkins Hospital and School of MedicineBaltimoreUSA

Personalised recommendations