Skip to main content

Congenital Heart Disease and Right Heart

  • Chapter
  • First Online:
Right Heart Pathology

Abstract

In the field of congenital heart disease (CHD) the right heart is frequently affected, therefore understanding its dysfunction and discovering new modalities of evaluation may have clinical implications. In patients with CHD, the right ventricle (RV) is either considered the subpulmonary ventricle as is the case in atrial septal defects, pulmonary stenosis, and Tetralogy of Fallot, either the systemic ventricle, e.g. in transposition of the great arteries (TGA). Without corrective surgery for these lesions, right-sided heart failure may develop and severely complicate the evolution of these patients. Consequently, finding new means to evaluate the right ventricle is highly important and has prognostic relevance. The anatomy and shape of the right ventricle (RV) are complex, making its assessment more difficult. Generally, several imaging modalities can be utilized, mainly echocardiography, but also radionuclide imaging and, more recently, computed tomography (CT) and cardiac magnetic resonance (CMR). As mentioned before, in CHD the RV can functionally serve as the sub-pulmonary ventricle, however, it can also function as the systemic ventricle, in order to support pressure or volume overload or both.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 219.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 279.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 379.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Davlouros PA, Niwa K, Webb G, Gatzoulis MA. The right ventricle in congenital heart disease. Heart. 2006;92(Suppl 1):i27–38. https://doi.org/10.1136/hrt.2005.077438.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Guihaire J, Haddad F, Mercier O, Murphy DJ, Wu JC, Fadel E. The right heart in congenital heart disease, mechanisms and recent advances. J Clin Exp Cardiolog. 2012;8(10):1–11. https://doi.org/10.4172/2155-9880.S8-010.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Pascotto M, Caso P, Santoro G, et al. Analysis of right ventricular Doppler tissue imaging and load dependence in patients undergoing percutaneous closure of atrial septal defect. Am J Cardiol. 2004;94(9):1202–5. https://doi.org/10.1016/j.amjcard.2004.07.098.

    Article  PubMed  Google Scholar 

  4. Vasquez AF, Lasala JM. Atrial septal defect closure. Cardiol Clin. 2013;31(3):385–400. https://doi.org/10.1016/j.ccl.2013.05.003.

    Article  PubMed  Google Scholar 

  5. Kort HW, Balzer DT, Johnson MC. Resolution of right heart enlargement after closure of secundum atrial septal defect with transcatheter technique. J Am Coll Cardiol. 2001;38(5):1528–32. https://doi.org/10.1016/S0735-1097(01)01547-9.

    Article  PubMed  CAS  Google Scholar 

  6. Schussler JM, Anwar A, Phillips SD, Roberts BJ, Vallabhan RC, Grayburn PA. Effect on right ventricular volume of percutaneous Amplatzer closure of atrial septal defect in adults. Am J Cardiol. 2005;95(8):993–5. https://doi.org/10.1016/j.amjcard.2004.12.046.

    Article  PubMed  Google Scholar 

  7. Baumgartner H, Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC), et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31:2915–57. https://doi.org/10.1093/eurheartj/ehq249.

    Article  PubMed  Google Scholar 

  8. Du ZD, Hijazi ZM, Kleinman CS, Silverman NH, Larntz K. Comparison between transcatheter and surgical closure of secundum atrial septal defect in children and adults: results of a multicenter nonrandomized trial. J Am Coll Cardiol. 2002;39(11):1836–44. https://doi.org/10.1016/S0735-1097(02)01862-4.

    Article  PubMed  Google Scholar 

  9. Alonso-González R, Dimopoulos K, Ho S, Oliver JM, Gatzoulis MA. The right heart in adults with congenital heart disease. Rev Esp Cardiol. 2010;63(9):1070–86. https://doi.org/10.1016/S1885-5857(10)70211-5.

    Article  PubMed  Google Scholar 

  10. Li W, Davlouros PA, Kilner PJ, et al. Doppler-echocardiographic assessment of pulmonary regurgitation in adults with repaired tetralogy of Fallot: comparison with cardiovascular magnetic resonance imaging. Am Heart J. 2004;147(1):165–72. https://doi.org/10.1016/s0002-8703(03)00527-1.

    Article  PubMed  Google Scholar 

  11. Silversides CK, Veldtman GR, Crossin J, et al. Pressure half-time predicts hemodynamically significant pulmonary regurgitation in adult patients with repaired tetralogy of Fallot. J Am Soc Echocardiogr. 2003;16(10):1057–62. https://doi.org/10.1016/S0894-7317(03)00553-4.

    Article  PubMed  Google Scholar 

  12. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). Circulation. 2008;118(23):2395–451. https://doi.org/10.1161/CIRCULATIONAHA.108.190811.

    Article  PubMed  Google Scholar 

  13. Discigil B, Dearani JA, Puga FJ, et al. Late pulmonary valve replacement after repair of tetralogy of Fallot. J Thorac Cardiovasc Surg. 2001;121(2):344–51. https://doi.org/10.1067/mtc.2001.111209.

    Article  PubMed  CAS  Google Scholar 

  14. d’Udekem Y, Rubay J, Shango-Lody P, et al. Late homograft valve insertion after transannular patch repair of tetralogy of Fallot. J Heart Valve Dis. 1998;7(4):450–4.

    PubMed  Google Scholar 

  15. Vliegen HW, Van Straten A, De Roos A, et al. Magnetic resonance imaging to assess the hemodynamic effects of pulmonary valve replacement in adults late after repair of tetralogy of Fallot. Circulation. 2002;106(13):1703–7. https://doi.org/10.1161/01.CIR.0000030995.59403.F8.

    Article  PubMed  Google Scholar 

  16. Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000;356(9234):975–81. https://doi.org/10.1016/S0140-6736(00)02714-8.

    Article  CAS  PubMed  Google Scholar 

  17. Nollert GDA, Däbritz SH, Schmoeckel M, Vicol C, Reichart B. Risk factors for sudden death after repair of tetralogy of Fallot. Ann Thorac Surg. 2003;76(6):1901–5. https://doi.org/10.1016/S0003-4975(03)01065-8.

    Article  PubMed  Google Scholar 

  18. Gatzoulis MA, Till JA, Somerville J, Redington AN. Mechanoelectrical interaction in tetralogy of Fallot: QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation. 1995;92(2):231–7. https://doi.org/10.1161/01.CIR.92.2.231.

    Article  CAS  PubMed  Google Scholar 

  19. Khairy P, Landzberg MJ, Gatzoulis MA, et al. Value of programmed ventricular stimulation after tetralogy of Fallot repair: a multicenter study. Circulation. 2004;109(16):1994–2000. https://doi.org/10.1161/01.CIR.0000126495.11040.BD.

    Article  PubMed  Google Scholar 

  20. Babu-Narayan SV, Kilner PJ, Li W, et al. Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of Fallot and its relationship to adverse markers of clinical outcome. Circulation. 2015;113(3):405–14. https://doi.org/10.1161/CIRCULATIONAHA.105.548727.

    Article  CAS  Google Scholar 

  21. Ghai A, Silversides C, Harris L, Webb GD, Siu SC, Therrien J. Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot. J Am Coll Cardiol. 2002;40(9):1675–80. https://doi.org/10.1016/S0735-1097(02)02344-6.

    Article  PubMed  Google Scholar 

  22. Oechslin E, Buchholz S, Jenni R. Ebstein’s anomaly in adults: Doppler-echocardiographic evaluation. Thorac Cardiovasc Surg. 2000;48(4):209–13. https://doi.org/10.1055/s-2000-6900.

    Article  PubMed  CAS  Google Scholar 

  23. Nihoyannopoulos P, McKenna WJ, Smith G, Foale R. Echocardiographic assessment of the right ventricle in Ebstein’s anomaly: relation to clinical outcome. J Am Coll Cardiol. 1986;8(3):627–35. https://doi.org/10.1016/S0735-1097(86)80193-0.

    Article  PubMed  CAS  Google Scholar 

  24. Chauvaud S, Berrebi A, D’Attellis N, Mousseaux E, Hernigou A, Carpentier A. Ebstein’s anomaly: repair based on functional analysis. Eur J Cardiothorac Surg. 2003;23:525–31. https://doi.org/10.1016/S1010-7940(02)00836-9.

    Article  PubMed  CAS  Google Scholar 

  25. Mahle WT, Parks WJ, Fyfe DA, Sallee D. Tricuspid regurgitation in patients with repaired tetralogy of Fallot and its relation to right ventricular dilatation. Am J Cardiol. 2003;92(5):643–5. https://doi.org/10.1016/S0002-9149(03)00746-X.

    Article  PubMed  Google Scholar 

  26. Conte S, Jashari R, Eyskens B, Gewillig M, Dumoulin M, Daenen W. Homograft valve insertion for pulmonary regurgitation late after valveless repair of right ventricular outflow tract obstruction. Eur J Cardiothorac Surg. 1999;15(2):143–9. https://doi.org/10.1016/S1010-7940(98)00306-6.

    Article  PubMed  CAS  Google Scholar 

  27. Warnes CA. Adult congenital heart disease importance of the right ventricle. J Am Coll Cardiol. 2009;54(21):1903–10. https://doi.org/10.1016/j.jacc.2009.06.048.

    Article  PubMed  Google Scholar 

  28. Jarrar M, Betbout F, Farhat MB, et al. Long-term invasive and noninvasive results of percutaneous balloon pulmonary valvuloplasty in children, adolescents, and adults. Am Heart J. 1999;138(5):950–4. https://doi.org/10.1016/S0002-8703(99)70022-0.

    Article  PubMed  CAS  Google Scholar 

  29. Sadr-Ameli MA, Sheikholeslami F, Firoozi I, Azarnik H. Late results of balloon pulmonary valvuloplasty in adults. Am J Cardiol. 1998;82(3):398–400. https://doi.org/10.1016/S0002-9149(98)00302-6.

    Article  PubMed  CAS  Google Scholar 

  30. Lundstrom U, Bull C, Wyse RK, Somerville J. The natural and “unnatural” history of congenitally corrected transposition. Am J Cardiol. 1990;65(18):1222–9. https://doi.org/10.1016/0002-9149(90)90978-a.

    Article  PubMed  CAS  Google Scholar 

  31. Graham TP, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol. 2000;36(1):255–61. https://doi.org/10.1016/S0735-1097(00)00682-3.

    Article  PubMed  Google Scholar 

  32. Daliento L, Corrado D, Buja G, John N, Nava A, Thiene G. Rhythm and conduction disturbances in isolated, congenitally corrected transposition of the great arteries. Am J Cardiol. 1986;58(3):314–8.

    Article  PubMed  CAS  Google Scholar 

  33. Prieto LR, Hordof AJ, Secic M, Rosenbaum MS, Gersony WM. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries. Circulation. 1998;98(10):997–1005. https://doi.org/10.1161/01.CIR.98.10.997.

    Article  PubMed  CAS  Google Scholar 

  34. Roos-Hesselink JW, Meijboom FJ, Spitaels SEC, et al. Excellent survival and low incidence of arrhythmias, stroke and heart failure long-term after surgical ASD closure at young age: a prospective follow-up study of 21-33 years. Eur Heart J. 2003;24(2):190–7. https://doi.org/10.1016/S0195-668X(02)00383-4.

    Article  CAS  PubMed  Google Scholar 

  35. Lissin LW, Li W, Murphy DJ, et al. Comparison of transthoracic echocardiography versus cardiovascular magnetic resonance imaging for the assessment of ventricular function in adults after atrial switch procedures for complete transposition of the great arteries. Am J Cardiol. 2004;93(5):654–7. https://doi.org/10.1016/j.amjcard.2003.11.044.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Iancovici, S., Dorobanţu, M. (2018). Congenital Heart Disease and Right Heart. In: Dumitrescu, S., Ţintoiu, I., Underwood, M. (eds) Right Heart Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-73764-5_14

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-73764-5_14

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-73763-8

  • Online ISBN: 978-3-319-73764-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics