Advertisement

Congenital Heart Disease and Right Heart

  • Silvia Iancovici
  • Maria Dorobanţu
Chapter

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.

Keywords

Congenital heart disease Right heart Right-sided heart failure Atrial septal defect Ventricular septal defect Ebstein anomaly Fallot tetralogy Pulmonary regurgitation Tricuspid regurgitation Transposition of the great arteries 

References

  1. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 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.CrossRefPubMedGoogle Scholar
  4. 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.CrossRefPubMedGoogle Scholar
  5. 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.CrossRefPubMedGoogle Scholar
  6. 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.CrossRefPubMedGoogle Scholar
  7. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 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.CrossRefPubMedGoogle Scholar
  9. 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.CrossRefPubMedGoogle Scholar
  10. 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.CrossRefPubMedGoogle Scholar
  11. 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.CrossRefPubMedGoogle Scholar
  12. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  13. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 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.PubMedPubMedCentralGoogle Scholar
  15. 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.CrossRefPubMedGoogle Scholar
  16. 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.CrossRefPubMedGoogle Scholar
  17. 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.CrossRefPubMedGoogle Scholar
  18. 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.CrossRefPubMedGoogle Scholar
  19. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  20. 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.CrossRefGoogle Scholar
  21. 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.CrossRefPubMedGoogle Scholar
  22. 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.CrossRefPubMedGoogle Scholar
  23. 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.CrossRefPubMedGoogle Scholar
  24. 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.CrossRefPubMedGoogle Scholar
  25. 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.CrossRefPubMedGoogle Scholar
  26. 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.CrossRefPubMedGoogle Scholar
  27. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 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.CrossRefPubMedGoogle Scholar
  29. 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.CrossRefPubMedGoogle Scholar
  30. 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.CrossRefPubMedGoogle Scholar
  31. 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.CrossRefPubMedGoogle Scholar
  32. 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.CrossRefPubMedGoogle Scholar
  33. 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.CrossRefPubMedGoogle Scholar
  34. 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.CrossRefPubMedGoogle Scholar
  35. 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.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Silvia Iancovici
    • 1
  • Maria Dorobanţu
    • 1
    • 2
  1. 1.Cardiology DepartmentEmergency Clinical HospitalBucharestRomania
  2. 2.Cardiology“Carol Davila” University of Medicine and PharmacyBucharestRomania

Personalised recommendations