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The Right Ventricle in Congenital Heart Diseases

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The Right Heart

Abstract

Right ventricular function is an important determinant of prognosis and outcome in congenital heart diseases. Right ventricular (RV) adaptation to congenital heart diseases (CHD) has many faces as there is a wide variety in defects involving the right ventricle as well as in treatment strategies. This variety induces differences in loading conditions and also changes over time as a result of surgical interventions. Also, treatment practice has evolved changing the nature and outcome of survivors of CHD. Lastly, several lesions also affect the left ventricle (LV) that may interact with RV function and thereby change the RV function.

Although in practice sometimes artificially, for educational and conceptual purposes the effects on the RV can be divided into three types of abnormal loading conditions, i.e., increased preload (e.g., shunts or valvular insufficiency), increased afterload (e.g., stenosis or connection to systemic circulation), or a mixture of both. During the process of maturation and aging and as a result of interventions loading conditions can shift from increased afterload to increased preload. In this chapter we describe the different faces of the RV with a focus on the functional capacity. We differentiate between lesions affecting preload, afterload, and a mixture of those. Special attention is given to the RV in corrected tetralogy of Fallot and the systemic RV in congenitally corrected transposition of the great arteries, after the atrial switch procedure for transposition of the great arteries or in hypoplastic left-heart syndrome.

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Abbreviations

ACE:

Angiotensin-converting enzyme

ARB:

Angiotensin receptor blocker

ASD:

Atrial septal defect

ccTGA:

Congenitally corrected transposition of the great arteries

CHD:

Congenital heart diseases

CMR:

Cardiac magnetic resonance imaging

EF:

Ejection fraction

FAC:

Fractional area change

LV:

Left ventricle

PAH:

Pulmonary arterial hypertension

PH:

Pulmonary hypertension

RV:

Right ventricle

RVOT:

Right ventricular outflow tract

TAPSE:

Tricuspid annular plane systolic excursion

TGA-as:

Transposition of the great arteries with atrial switch procedure

TI:

Tricuspid insufficiency

TOF:

Tetralogy of Fallot

VSD:

Ventricular septal defect

References

  1. Norozi K, Wessel A, Alpers V, Arnhold JO, Geyer S, Zoege M, et al. Incidence and risk distribution of heart failure in adolescents and adults with congenital heart disease after cardiac surgery. Am J Cardiol. 2006;97:1238–43.

    Article  PubMed  Google Scholar 

  2. Anderson R, Baker E, Penny D, Redington A, Rigby M, Wernovsky G, editors. Pediatric cardiology. 3rd ed. Philadelphia: Churchill Livingstone; 2009.

    Google Scholar 

  3. Allen H, Driscoll D, Shaddy R, Feltes T, editors. Heart disease in infants, children, and adolescents. Philadelphia: Lippincott Williams & Wilkins; 2012.

    Google Scholar 

  4. Webb G, Gatzoulis MA. Atrial septal defects in the adult. Circulation. 2006;114:1645–53. https://doi.org/10.1161/CIRCULATIONAHA.105.592055.

    Article  PubMed  Google Scholar 

  5. Rudolph AM. Congenital diseases of the heart: clinical-physiological considerations. 3rd ed. New York: John Wiley & Sons; 2011.

    Google Scholar 

  6. Borgdorff MAJJ, Bartelds B, Dickinson MG, Steendijk P, de Vroomen M, Berger RMFF. Distinct loading conditions reveal various patterns of right ventricular adaptation. Am J Physiol Heart Circ Physiol. 2013;305:H354–64. Available from: https://pubmed.ncbi.nlm.nih.gov/23729212.

    Article  CAS  PubMed  Google Scholar 

  7. Eyskens B, Ganame J, Claus P, Boshoff D, Gewillig M, Mertens L. Ultrasonic strain rate and strain imaging of the right ventricle in children before and after percutaneous closure of an atrial septal defect. J Am Soc Echocardiogr. 2006;19:994–1000.

    Article  PubMed  Google Scholar 

  8. Van De Bruaene A, Buys R, Vanhees L, Delcroix M, Voigt JU, Budts W. Regional right ventricular deformation in patients with open and closed atrial septal defect. Eur J Echocardiogr. 2011;12:206–13.

    Article  Google Scholar 

  9. Bartelds B, Borgdorff MA, Smit-van Oosten A, Takens J, Boersma B, Nederhoff MG, et al. Differential responses of the right ventricle to abnormal loading conditions in mice: pressure vs. volume load. Eur J Heart Fail. 2011;13:1275–82. Available from: https://pubmed.ncbi.nlm.nih.gov/22024026.

    Article  CAS  PubMed  Google Scholar 

  10. Cuypers JAAE, Opić P, Menting ME, Utens EMWJ, Witsenburg M, Helbing WA, et al. The unnatural history of an atrial septal defect: longitudinal 35 year follow up after surgical closure at young age. Heart. 2013;99:1346–52.

    Article  PubMed  Google Scholar 

  11. Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet. 2009;374:1462–71.

    Article  CAS  PubMed  Google Scholar 

  12. Bouzas B, Kilner PJ, Gatzoulis MA. Pulmonary regurgitation: not a benign lesion. Eur Heart J. 2005;26:433–9. Available from: https://pubmed.ncbi.nlm.nih.gov/15640261/.

    Article  PubMed  Google Scholar 

  13. Annavajjhala V, Punn R, Tacy TA, Hanley FL, McElhinney DB. Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: comparison of transannular patch and valve-sparing repair. Congenit Heart Dis. 2019;14:691–9. Available from: https://pubmed.ncbi.nlm.nih.gov/30989806/.

    Article  PubMed  Google Scholar 

  14. Hoelscher M, Bonassin F, Oxenius A, Seifert B, Leonardi B, Kellenberger CJ, et al. Right ventricular dilatation in patients with pulmonary regurgitation after repair of tetralogy of Fallot: how fast does it progress? Ann Pediatr Cardiol. 2020;13:294–300. Available from: https://pubmed.ncbi.nlm.nih.gov/33311917/.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Freling HG, Pieper PG, Vermeulen KM, van Swieten JM, Sijens PE, van Veldhuisen DJ, et al. Improved cardiac MRI volume measurements in patients with tetralogy of Fallot by independent end-systolic and end-diastolic phase selection. PLoS One. 2013;8:e55462.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Freling HG, Van Wijk K, Jaspers K, Pieper PG, Vermeulen KM, Van Swieten JM, et al. Impact of right ventricular endocardial trabeculae on volumes and function assessed by CMR in patients with tetralogy of Fallot. Int J Cardiovasc Imaging. 2013;29:625–31.

    Article  PubMed  Google Scholar 

  17. Jaspers K, Freling HG, Van Wijk K, Romijn EI, Greuter MJW, Willems TP. Improving the reproducibility of MR-derived left ventricular volume and function measurements with a semi-automatic threshold-based segmentation algorithm. Int J Cardiovasc Imaging. 2013;29:617–23.

    Article  PubMed  Google Scholar 

  18. Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000;356:975–81.

    Article  CAS  PubMed  Google Scholar 

  19. Redington AN. Physiopathology of right ventricular failure. Pediatr Card Surg Annu. 2006;9:3–10.

    Google Scholar 

  20. Giardini A, Specchia S, Tacy TA, Coutsoumbas G, Gargiulo G, Donti A, et al. Usefulness of cardiopulmonary exercise to predict long-term prognosis in adults with repaired tetralogy of Fallot. Am J Cardiol. 2007;99:1462–7.

    Article  PubMed  Google Scholar 

  21. Roest AAW, Helbing WA, Kunz P, Van den Aardweg JG, Lamb HJ, Vliegen HW, et al. Exercise MR imaging in the assessment of pulmonary regurgitation and biventricular function in patients after tetralogy of Fallot repair. Radiology. 2002;223:204–11.

    Article  PubMed  Google Scholar 

  22. Fogel MA, Sundareswaran KS, De Zelicourt D, Dasi LP, Pawlowski T, Rome J, et al. Power loss and right ventricular efficiency in patients after tetralogy of Fallot repair with pulmonary insufficiency: clinical implications. J Thorac Cardiovasc Surg. 2012;143:1279–85.

    Article  PubMed  Google Scholar 

  23. Krupickova S, Risch J, Gati S, Caliebe A, Sarikouch S, Beerbaum P, et al. Cardiovascular magnetic resonance normal values in children for biventricular wall thickness and mass. J Cardiovasc Magn Reson. 2021;23:1. Available from: https://pubmed.ncbi.nlm.nih.gov/33390185/.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Van Der Ven JPG, Sadighy Z, Valsangiacomo Buechel ER, Sarikouch S, Robbers-Visser D, Kellenberger CJ, et al. Multicentre reference values for cardiac magnetic resonance imaging derived ventricular size and function for children aged 0–18 years. Eur Heart J Cardiovasc Imaging. 2020;21:102–13. Available from: /pmc/articles/PMC6923680/?report=abstract.

    Article  PubMed  Google Scholar 

  25. Hagdorn QAJ, Beurskens NEG, Gorter TM, Eshuis G, Hillege HL, Lui GK, et al. Sex differences in patients with repaired tetralogy of Fallot support a tailored approach for males and females: a cardiac magnetic resonance study. Int J Cardiovasc Imaging. 2020;36:1997–2005. Available from: https://pubmed.ncbi.nlm.nih.gov/32472300/.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Van Den Berg J, Wielopolski PA, Meijboom FJ, Witsenburg M, Bogers AJJC, Pattynama PMT, et al. Diastolic function in repaired tetralogy of Fallot at rest and during stress: assessment with MR imaging. Radiology. 2007;243:212–9.

    Article  PubMed  Google Scholar 

  27. Fernandes FP, Manlhiot C, Roche SL, Grosse-Wortmann L, Slorach C, McCrindle BW, et al. Impaired left ventricular myocardial mechanics and their relation to pulmonary regurgitation, right ventricular enlargement and exercise capacity in asymptomatic children after repair of tetralogy of Fallot. J Am Soc Echocardiogr. 2012;25:494–503.

    Article  PubMed  Google Scholar 

  28. Frigiola A, Tsang V, Bull C, Coats L, Khambadkone S, Derrick G, et al. Biventricular response after pulmonary valve replacement for right ventricular outflow tract dysfunction: is age a predictor of outcome? Circulation. 2008;118:S182–90.

    Article  PubMed  Google Scholar 

  29. Knauth AL, Gauvreau K, Powell AJ, Landzberg MJ, Walsh EP, Lock JE, et al. Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair. Heart. 2008;94:211–6.

    Article  CAS  PubMed  Google Scholar 

  30. Babu-Narayan SV, Uebing A, Davlouros PA, Kemp M, Davidson S, Dimopoulos K, et al. Randomised trial of ramipril in repaired tetralogy of Fallot and pulmonary regurgitation: the APPROPRIATE study (Ace inhibitors for Potential PRevention of the deleterious effects of Pulmonary Regurgitation in Adults with repaired TEtralogy of Fallot). Int J Cardiol. 2012;154:299–305.

    Article  PubMed  Google Scholar 

  31. Bonello B, Kempny A, Uebing A, Li W, Kilner PJ, Diller GP, et al. Right atrial area and right ventricular outflow tract akinetic length predict sustained tachyarrhythmia in repaired tetralogy of Fallot. Int J Cardiol. 2013;168:3280–6.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Greutmann M, Tobler D, Biaggi P, Mah ML, Crean A, Oechslin EN, et al. Echocardiography for assessment of right ventricular volumes revisited: a cardiac magnetic resonance comparison study in adults with repaired tetralogy of Fallot. J Am Soc Echocardiogr. 2010;23:905–11.

    Article  PubMed  Google Scholar 

  33. Davlouros PA, Kilner PJ, Hornung TS, Li W, Francis JM, Moon JCC, et al. Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow aneurysms or akinesia and adverse right-to-left ventricular interaction. J Am Coll Cardiol. 2002;40:2044–52.

    Article  PubMed  Google Scholar 

  34. Kempny A, Diller GP, Orwat S, Kaleschke G, Kerckhoff G, Bunck AC, et al. Right ventricular-left ventricular interaction in adults with tetralogy of Fallot: a combined cardiac magnetic resonance and echocardiographic speckle tracking study. Int J Cardiol. 2012;154:259–64.

    Article  PubMed  Google Scholar 

  35. Geva T, Gauvreau K, Powell AJ, Cecchin F, Rhodes J, Geva J, et al. Randomized trial of pulmonary valve replacement with and without right ventricular remodeling surgery. Circulation. 2010;122:S201–8.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Geva T. Indications for pulmonary valve replacement in repaired tetralogy of Fallot: the quest continues. Circulation. 2013;128:1855–7.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Bokma JP, Winter MM, Van Dijk AP, Vliegen HW, Van Melle JP, Meijboom FJ, et al. Effect of losartan on right ventricular dysfunction: results from the double-blind, randomized REDEFINE trial (right ventricular dysfunction in tetralogy of Fallot: inhibition of the renin-angiotensin-aldosterone system) in adults with repaired tetralogy of Fallot. Circulation. 2018;137:1463–71. Available from: https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.117.031438.

    Article  CAS  PubMed  Google Scholar 

  38. Diller GP, Dimopoulos K, Okonko D, Uebing A, Broberg CS, Babu-Narayan S, et al. Heart rate response during exercise predicts survival in adults with congenital heart disease. J Am Coll Cardiol. 2006;48:1250–6.

    Article  PubMed  Google Scholar 

  39. Diller GP, Dimopoulos K, Okonko D, Li W, Babu-Narayan SV, Broberg CS, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828–35.

    Article  PubMed  Google Scholar 

  40. Koestenberger M, Nagel B, Ravekes W, Avian A, Heinzl B, Fandl A, et al. Tricuspid annular peak systolic velocity (S’) in children and young adults with pulmonary artery hypertension secondary to congenital heart diseases, and in those with repaired tetralogy of Fallot: echocardiography and MRI data. J Am Soc Echocardiogr. 2012;25:1041–9.

    Article  PubMed  Google Scholar 

  41. Li VW-Y, Yu CK-M, So EK-F, Wong WH-S, Cheung Y-F. Ventricular myocardial deformation imaging of patients with repaired tetralogy of Fallot. J Am Soc Echocardiogr. 2020;33:788–801. Available from: https://pubmed.ncbi.nlm.nih.gov/32624088/.

    Article  PubMed  Google Scholar 

  42. Buechel ERV, Mertens LL. Imaging the right heart: the use of integrated multimodality imaging. Eur Heart J. 2012:949–60. Available from: https://pubmed.ncbi.nlm.nih.gov/22408035/.

  43. Van den Eynde J, Sá MPBO, Vervoort D, Roever L, Meyns B, Budts W, et al. Pulmonary valve replacement in tetralogy of Fallot: an updated meta-analysis. Ann Thorac Surg. 2020. Available from: https://pubmed.ncbi.nlm.nih.gov/33378694/.

  44. Dragulescu A, Grosse-Wortmann L, Fackoury C, Riffle S, Waiss M, Jaeggi E, et al. Echocardiographic assessment of right ventricular volumes after surgical repair of tetralogy of Fallot: clinical validation of a new echocardiographic method. J Am Soc Echocardiogr. 2011;24:1191–8.

    Article  PubMed  Google Scholar 

  45. Eyskens B, Brown SC, Claus P, Dymarkowski S, Gewillig M, Bogaert J, et al. The influence of pulmonary regurgitation on regional right ventricular function in children after surgical repair of tetralogy of Fallot. Eur J Echocardiogr. 2010;11:341–5. Available from: https://pubmed.ncbi.nlm.nih.gov/20085920/.

    Article  PubMed  Google Scholar 

  46. Bodhey NK, Beerbaum P, Sarikouch S, Kropf S, Lange P, Berger F, et al. Functional analysis of the components of the right ventricle in the setting of tetralogy of Fallot. Circ Cardiovasc Imaging. 2008;1:141–7.

    Article  PubMed  Google Scholar 

  47. 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;25:682–9.

    Article  PubMed  Google Scholar 

  48. O’Meagher S, Munoz PA, Alison JA, Young IH, Tanous DJ, Celermajer DS, et al. Exercise capacity and stroke volume are preserved late after tetralogy repair, despite severe right ventricular dilatation. Heart. 2012;98:1595–9.

    Article  PubMed  Google Scholar 

  49. Beurskens NEG, Hagdorn QAJ, Gorter TM, Berger RMF, Vermeulen KM, van Melle JP, et al. Risk of cardiac tachyarrhythmia in patients with repaired tetralogy of Fallot: a multicenter cardiac MRI based study. Int J Cardiovasc Imaging. 2019;35:143–51. Available from: https://pubmed.ncbi.nlm.nih.gov/30094564.

    Article  PubMed  Google Scholar 

  50. Gatzoulis MA, Elliott JT, Guru V, Siu SC, Warsi MA, Webb GD, et al. Right and left ventricular systolic function late after repair of tetralogy of Fallot. Am J Cardiol. 2000;86:1352–7.

    Article  CAS  PubMed  Google Scholar 

  51. Lumens J, Fan CS, Walmsley J, Yim D, Manlhiot C, Dragulescu A, et al. Relative impact of right ventricular electromechanical dyssynchrony versus pulmonary regurgitation on right ventricular dysfunction and exercise intolerance in patients after repair of tetralogy of Fallot. J Am Heart Assoc. 2019;8. Available from: https://www.ahajournals.org/doi/10.1161/JAHA.118.010903.

  52. Yim D, Hui W, Larios G, Dragulescu A, Grosse-Wortmann L, Bijnens B, et al. Quantification of right ventricular electromechanical dyssynchrony in relation to right ventricular function and clinical outcomes in children with repaired tetralogy of Fallot. J Am Soc Echocardiogr. 2018;31:822–30. Available from: https://pubmed.ncbi.nlm.nih.gov/29976349/.

    Article  PubMed  Google Scholar 

  53. Hui W, Slorach C, Bradley TJ, Jaeggi ET, Mertens L, Friedberg MK. Measurement of right ventricular mechanical synchrony in children using tissue doppler velocity and two-dimensional strain imaging. J Am Soc Echocardiogr. 2010;23:1289–96.

    Article  PubMed  Google Scholar 

  54. Roche SL, Grosse-Wortmann L, Redington AN, Slorach C, Smith G, Kantor PF, et al. Exercise induces biventricular mechanical dyssynchrony in children with repaired tetralogy of Fallot. Heart. 2010;96:2010–5.

    Article  PubMed  Google Scholar 

  55. Friedberg MK, Fernandes FP, Roche SL, Slorach C, Grosse-Wortmann L, Manlhiot C, et al. Relation of right ventricular mechanics to exercise tolerance in children after tetralogy of Fallot repair. Am Heart J. 2013;165:551–7.

    Article  PubMed  Google Scholar 

  56. Gatzoulis MA, Clark AL, Cullen S, Newman CGH, Redington AN. Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot: restrictive physiology predicts superior exercise performance. Circulation. 1995;91:1775–81.

    Article  CAS  PubMed  Google Scholar 

  57. Babu-Narayan SV, Kilner PJ, Li W, Moon JC, Goktekin O, Davlouros PA, 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. 2006;113:405–13.

    Article  CAS  PubMed  Google Scholar 

  58. Burkhoff D, Mirsky I, Suga H. Assessment of systolic and diastolic ventricular properties via pressure-volume analysis: a guide for clinical, translational, and basic researchers. Am J Physiol Heart Circ Physiol. 2005;289:H501–12.

    Article  CAS  PubMed  Google Scholar 

  59. Ahmad N, Kantor PF, Grosse-Wortmann L, Seller N, Jaeggi ET, Friedberg MK, et al. Influence of RV restrictive physiology on LV diastolic function in children after tetralogy of fallot repair. J Am Soc Echocardiogr. 2012;25:866–73.

    Article  PubMed  Google Scholar 

  60. Hagdorn QAJ, Vos JDL, Beurskens NEG, Gorter TM, Meyer SL, van Melle JP, et al. CMR feature tracking left ventricular strain-rate predicts ventricular tachyarrhythmia, but not deterioration of ventricular function in patients with repaired tetralogy of Fallot. Int J Cardiol. 2019;295:1–6. Available from: https://pubmed.ncbi.nlm.nih.gov/31402156.

    Article  PubMed  Google Scholar 

  61. Frigiola A, Redington AN, Cullen S, Vogel M. Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot. Circulation. 2004;110. Available from: https://pubmed.ncbi.nlm.nih.gov/15364855/.

  62. Diller GP, Kempny A, Liodakis E, Alonso-Gonzalez R, Inuzuka R, Uebing A, et al. Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired tetralogy of Fallot. Circulation. 2012;125:2440–6.

    Article  PubMed  Google Scholar 

  63. de Alba CG, Khan A, Woods P, Broberg CS. Left ventricular strain and fibrosis in adults with repaired tetralogy of Fallot: a case-control study. Int J Cardiol. 2021;323:34–9. Available from: https://pubmed.ncbi.nlm.nih.gov/32882293/.

    Article  PubMed  Google Scholar 

  64. Diller GP, Orwat S, Vahle J, Bauer UMM, Bauer UMM, Urban A, et al. Prediction of prognosis in patients with tetralogy of Fallot based on deep learning imaging analysis. Heart. 2020;106:1007–14. Available from: https://pubmed.ncbi.nlm.nih.gov/32161041/.

    Article  CAS  PubMed  Google Scholar 

  65. Cochet H, Iriart X, Allain-Nicolaï A, Camaioni C, Sridi S, Nivet H, et al. Focal scar and diffuse myocardial fibrosis are independent imaging markers in repaired tetralogy of Fallot. Eur Heart J Cardiovasc Imaging. 2019;20:990–1003. Available from: https://pubmed.ncbi.nlm.nih.gov/30993335/.

    Article  PubMed  PubMed Central  Google Scholar 

  66. Bokma JP, Winter MM, Vehmeijer JT, Vliegen HW, Van Dijk AP, Van Melle JP, et al. QRS fragmentation is superior to QRS duration in predicting mortality in adults with tetralogy of Fallot. Heart. 2016;103:666–71. Available from: https://pubmed.ncbi.nlm.nih.gov/27803032/.

    Article  PubMed  Google Scholar 

  67. Graham TP, Bernard YD, Mellen BG, Celermajer D, Baumgartner H, Cetta F, et al. Long-term outcome in congenitally corrected transposition of the great arteries. J Am Coll Cardiol. 2000;36:255–61.

    Article  PubMed  Google Scholar 

  68. Dobson R, Danton M, Nicola W, Hamish W. The natural and unnatural history of the systemic right ventricle in adult survivors. J Thorac Cardiovasc Surg. 2013;145:1493–503.

    Article  PubMed  Google Scholar 

  69. Grothoff M, Fleischer A, Abdul-Khaliq H, Hoffmann J, Lehmkuhl L, Luecke C, et al. The systemic right ventricle in congenitally corrected transposition of the great arteries is different from the right ventricle in dextro-transposition after atrial switch: a cardiac magnetic resonance study. Cardiol Young. 2013;23:239–47.

    Article  PubMed  Google Scholar 

  70. Roos-Hesselink JW, Meijboom FJ, Spitaels SEC, Van Domburg R, Van Rijen EHM, Utens EMWJ, et al. Decline in ventricular function and clinical condition after mustard repair for transposition of the great arteries (a prospective study of 22-29 years). Eur Heart J. 2004;25:1264–70.

    Article  CAS  PubMed  Google Scholar 

  71. Roubertie F, Thambo JB, Bretonneau A, Iriart X, Laborde N, Baudet E, et al. Late outcome of 132 Senning procedures after 20 years of follow-up. Ann Thorac Surg. 2011;92:2206–14.

    Article  PubMed  Google Scholar 

  72. Norozi K, Wessel A, Buchhorn R, Alpers V, Arnhold JO, Zoege M, et al. Is the ability index superior to the NYHA classification for assessing heart failure? Comparison of two classification scales in adolescents and adults with operated congenital heart defects. Clin Res Cardiol. 2007;96:542–7.

    Article  PubMed  Google Scholar 

  73. Roest AAW, Lamb HJ, Van Der Wall EE, Vliegen HW, Van Den Aerdweg JG, Kunz P, et al. Cardiovascular response to physical exercise in adult patients after atrial correction for transposition of the great arteries assessed with magnetic resonance imaging. Heart. 2004;90:678–84.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  74. Winter MM, Bernink FJP, Groenink M, Bouma BJ, Van Dijk APJ, Helbing WA, et al. Evaluating the systemic right ventricle by CMR: the importance of consistent and reproducible delineation of the cavity. J Cardiovasc Magn Reson. 2008;10:40.

    Article  PubMed  PubMed Central  Google Scholar 

  75. Van Der Bom T, Winter MM, Groenink M, Vliegen HW, Pieper PG, Van Dijk APJ, et al. Right ventricular end-diastolic volume combined with peak systolic blood pressure during exercise identifies patients at risk for complications in adults with a systemic right ventricle. J Am Coll Cardiol. 2013;62:926–36.

    Article  PubMed  Google Scholar 

  76. Fratz S, Hager A, Busch R, Kaemmerer H, Schwaiger M, Lange R, et al. Patients after atrial switch operation for transposition of the great arteries can not increase stroke volume under dobutamine stress as opposed to patients with congenitally corrected transposition. Circ J. 2008;72:1130–5.

    Article  PubMed  Google Scholar 

  77. Pettersen E, Helle-Valle T, Edvardsen T, Lindberg H, Smith HJ, Smevik B, et al. Contraction pattern of the systemic right ventricle. Shift from longitudinal to circumferential shortening and absent global ventricular torsion. J Am Coll Cardiol. 2007;49:2450–6.

    Article  PubMed  Google Scholar 

  78. Khattab K, Schmidheiny P, Wustmann K, Wahl A, Seiler C, Schwerzmann M. Echocardiogram versus cardiac magnetic resonance imaging for assessing systolic function of subaortic right ventricle in adults with complete transposition of great arteries and previous atrial switch operation. Am J Cardiol. 2013;111:908–13.

    Article  PubMed  Google Scholar 

  79. Kukulski T, Hübbert L, Arnold M, Wranne B, Hatle L, Sutherland GR. Normal regional right ventricular function and its change with age: a Doppler myocardial imaging study. J Am Soc Echocardiogr. 2000;13:194–204.

    Article  CAS  PubMed  Google Scholar 

  80. Haber I, Metaxas DN, Geva T, Axel L. Three-dimensional systolic kinematics of the right ventricle. Am J Physiol Heart Circ Physiol. 2005;289:H1826–33.

    Article  CAS  PubMed  Google Scholar 

  81. Haddad F, Doyle R, Murphy DJ, Hunt SA. Right ventricular function in cardiovascular disease, part II: pathophysiology, clinical importance, and management of right ventricular failure. Circulation. 2008;117:1717–31.

    Article  PubMed  Google Scholar 

  82. Eyskens B, Weidemann F, Kowalski M, Bogaert J, Dymarkowski S, Bijnens B, et al. Regional right and left ventricular function after the Senning operation: an ultrasonic study of strain rate and strain. Cardiol Young. 2004;14:255–64.

    Article  PubMed  Google Scholar 

  83. Kalogeropoulos AP, Deka A, Border W, Pernetz MA, Georgiopoulou VV, Kiani J, et al. Right ventricular function with standard and speckle-tracking echocardiography and clinical events in adults with D-transposition of the great arteries post atrial switch. J Am Soc Echocardiogr. 2012;25:304–12.

    Article  PubMed  Google Scholar 

  84. Millane T, Bernard EJ, Jaeggi E, Howman-Giles RB, Uren RF, Cartmill TB, et al. Role of ischemia and infarction in late right ventricular dysfunction after atrial repair of transposition of the great arteries. J Am Coll Cardiol. 2000;35:1661–8.

    Article  CAS  PubMed  Google Scholar 

  85. Giardini A, Lovato L, Donti A, Formigari R, Oppido G, Gargiulo G, et al. Relation between right ventricular structural alterations and markers of adverse clinical outcome in adults with systemic right ventricle and either congenital complete (after Senning operation) or congenitally corrected transposition of the great arteries. Am J Cardiol. 2006;98:1277–82.

    Article  PubMed  Google Scholar 

  86. Hauser M, Bengel FM, Hager A, Kuehn A, Nekolla SG, Kaemmerer H, et al. Impaired myocardial blood flow and coronary flow reserve of the anatomical right systemic ventricle in patients with congenitally corrected transposition of the great arteries. Heart. 2003;89:1231–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  87. Singh TP, Humes RA, Muzik O, Kottamasu S, Karpawich PP, Di Carli MF. Myocardial flow reserve in patients with a systemic right ventricle after atrial switch repair. J Am Coll Cardiol. 2001;37:2120–5.

    Article  CAS  PubMed  Google Scholar 

  88. Broberg CS, Chugh SS, Conklin C, Sahn DJ, Jerosch-Herold M. Quantification of diffuse myocardial fibrosis and its association with myocardial dysfunction in congenital heart disease. Circ Cardiovasc Imaging. 2010;3:727–34.

    Article  PubMed  PubMed Central  Google Scholar 

  89. Rydman R, Gatzoulis MA, Ho SY, Ernst S, Swan L, Li W, et al. Systemic right ventricular fibrosis detected by cardiovascular magnetic resonance is associated with clinical outcome, mainly new-onset atrial arrhythmia, in patients after atrial redirection surgery for transposition of the great arteries. Circ Cardiovasc Imaging. 2015;8. Available from: https://pubmed.ncbi.nlm.nih.gov/25948241/.

  90. 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;98:997–1005.

    Article  CAS  PubMed  Google Scholar 

  91. Saran N, Dearani JA, Said SM, Greason KL, Pochettino A, Stulak JM, et al. Long-term outcomes of patients undergoing tricuspid valve surgery. Eur J Cardio-thoracic Surg. 2019;56:950–8. Available from: https://pubmed.ncbi.nlm.nih.gov/30919898/.

    Article  Google Scholar 

  92. Koolbergen DR, Ahmed Y, Bouma BJ, Scherptong RWC, Bruggemans EF, Vliegen HW, et al. Follow-up after tricuspid valve surgery in adult patients with systemic right ventricles. Eur J Cardio-thoracic Surg. 2016;50:456–63. Available from: https://pubmed.ncbi.nlm.nih.gov/26984988/.

    Article  Google Scholar 

  93. Chow PC, Liang XC, Lam WWM, Cheung EWY, Wong KT, Cheung YF. Mechanical right ventricular dyssynchrony in patients after atrial switch operation for transposition of the great arteries. Am J Cardiol. 2008;101:874–81.

    Article  PubMed  Google Scholar 

  94. Friedberg MK, Mertens L. Deformation imaging in selected congenital heart disease: is it evolving to clinical use? J Am Soc Echocardiogr. 2012;25:919–31.

    Article  PubMed  Google Scholar 

  95. Filippov AA, Del Nido PJ, Vasilyev NV. Management of systemic right ventricular failure in patients with congenitally corrected transposition of the great arteries. Circulation. 2016;134:1293–302. Available from: https://pubmed.ncbi.nlm.nih.gov/27777298/.

    Article  PubMed  Google Scholar 

  96. Jauvert G, Rousseau-Paziaud J, Villain E, Iserin L, Hidden-Lucet F, Ladouceur M, et al. Effects of cardiac resynchronization therapy on echocardiographic indices, functional capacity, and clinical outcomes of patients with a systemic right ventricle. Europace. 2009;11:184–90. Available from: https://pubmed.ncbi.nlm.nih.gov/19038975/.

    Article  PubMed  Google Scholar 

  97. Dubin AM, Janousek J, Rhee E, Strieper MJ, Cecchin F, Law IH, et al. Resynchronization therapy in pediatric and congenital heart disease patients: an international multicenter study. J Am Coll Cardiol. 2005;46:2277–83. Available from: https://pubmed.ncbi.nlm.nih.gov/16360058/.

    Article  PubMed  Google Scholar 

  98. Kempny A, Dimopoulos K, Uebing A, Moceri P, Swan L, Gatzoulis MA, et al. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life single centre experience and review of published data. Eur Heart J. 2012;33:1386–96. Available from: https://pubmed.ncbi.nlm.nih.gov/22199119/.

    Article  PubMed  Google Scholar 

  99. Derrick G, Narang I, White P, Kelleher A, Bush A, Penny D, et al. Failure of stroke volume augmentation during exercise and dobutamine stress is unrelated to load-independent indexes of right ventricular performance after the Mustard operation. Circulation. 2000;102:III154–9. Available from: https://www.ahajournals.org/doi/full/10.1161/circ.102.suppl_3.III-154.

    Article  CAS  PubMed  Google Scholar 

  100. Winter MM, Van Der Plas MN, Bouma BJ, Groenink M, Bresser P, Mulder BJM. Mechanisms for cardiac output augmentation in patients with a systemic right ventricle. Int J Cardiol. 2010;143:141–6.

    Article  PubMed  Google Scholar 

  101. Uebing A, Diller GP, Li W, Maskell M, Dimopoulos K, Gatzoulis MA. Optimised rate-responsive pacing does not improve either right ventricular haemodynamics or exercise capacity in adults with a systemic right ventricle. Cardiol Young. 2010;20:485–94.

    Article  PubMed  Google Scholar 

  102. Giardini A, Hager A, Lammers AE, Derrick G, Müller J, Diller GP, et al. Ventilatory efficiency and aerobic capacity predict event-free survival in adults with atrial repair for complete transposition of the great arteries. J Am Coll Cardiol. 2009;53:1548–55.

    Article  PubMed  Google Scholar 

  103. Hechter SJ, Fredriksen PM, Liu P, Veldtman G, Merchant N, Freeman M, et al. Angiotensin-converting enzyme inhibitors in adults after the Mustard procedure. Am J Cardiol. 2001;87:660–3.

    Article  CAS  PubMed  Google Scholar 

  104. Robinson B, Heise CT, Moore JW, Anella J, Sokoloski M, Eshaghpour E. Afterload reduction therapy in patients following intra-atrial baffle operation for transposition of the great arteries. Pediatr Cardiol. 2002;23:618–23.

    Article  CAS  PubMed  Google Scholar 

  105. Therrien J, Provost Y, Harrison J, Connelly M, Kaemmerer H, Webb GD. Effect of angiotensin receptor blockade on systemic right ventricular function and size: a small, randomized, placebo-controlled study. Int J Cardiol. 2008;129:187–92.

    Article  PubMed  Google Scholar 

  106. Dore A, Houde C, Chan KL, Ducharme A, Khairy P, Juneau M, et al. Angiotensin receptor blockade and exercise capacity in adults with systemic right ventricles: a multicenter, randomized, placebo-controlled clinical trial. Circulation. 2005;112:2411–6.

    Article  CAS  PubMed  Google Scholar 

  107. Lester SJ, McElhinney DB, Viloria E, Reddy GP, Ryan E, Tworetzky W, et al. Effects of Losartan in patients with a systemically functioning morphologic right ventricle after atrial repair of transposition of the great arteries. Am J Cardiol. 2001;88:1314–6.

    Article  CAS  PubMed  Google Scholar 

  108. Van Der Bom T, Winter MM, Bouma BJ, Groenink M, Vliegen HW, Pieper PG, et al. Effect of valsartan on systemic right ventricular function: a double-blind, randomized, placebo-controlled pilot trial. Circulation. 2013;127:322–30.

    Article  PubMed  CAS  Google Scholar 

  109. Borgdorff MA, Bartelds B, Dickinson MG, Steendijk P, Berger RMFF. A cornerstone of heart failure treatment is not effective in experimental right ventricular failure. Int J Cardiol. 2013;169:183–9. Available from: https://pubmed.ncbi.nlm.nih.gov/24067600.

    Article  PubMed  Google Scholar 

  110. Zaragoza-Macias E, Zaidi AN, Dendukuri N, Marelli A. Medical therapy for systemic right ventricles: a systematic review (part 1) for the 2018 AHA/ACC Guideline for the Management of Adults with Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019, 139:E801–13. Available from: https://pubmed.ncbi.nlm.nih.gov/30586770/.

  111. Lindenfeld JA, Keller K, Campbell DN, Wolfe RR, Quaife RA. Improved systemic ventricular function after carvedilol administration in a patient with congenitally corrected transposition of the great arteries. J Hear Lung Transplant. 2003;22:198–201. Available from: https://pubmed.ncbi.nlm.nih.gov/12581770/.

    Article  Google Scholar 

  112. Josephson CB, Howlett JG, Jackson SD, Finley J, Kells CM. A case series of systemic right ventricular dysfunction post atrial switch for simple D-transposition of the great arteries: the impact of beta-blockade. Can J Cardiol. 2006;22:769–72. Available from: https://pubmed.ncbi.nlm.nih.gov/16835671/.

    Article  PubMed  PubMed Central  Google Scholar 

  113. Giardini A, Lovato L, Donti A, Formigari R, Gargiulo G, Picchio FM, et al. A pilot study on the effects of carvedilol on right ventricular remodelling and exercise tolerance in patients with systemic right ventricle. Int J Cardiol. 2007;114:241–6.

    Article  PubMed  Google Scholar 

  114. Doughan ARK, McConnell ME, Book WM. Effect of beta blockers (carvedilol or metoprolol XL) in patients with transposition of great arteries and dysfunction of the systemic right ventricle. Am J Cardiol. 2007;99:704–6.

    Article  CAS  PubMed  Google Scholar 

  115. Bouallal R, Godart F, Francart C, Richard A, Foucher-Hossein C, Lions C. Interest of β-blockers in patients with right ventricular systemic dysfunction. Cardiol Young. 2010;20:615–9. Available from: https://pubmed.ncbi.nlm.nih.gov/20519056/.

    Article  PubMed  Google Scholar 

  116. Tutarel O, Meyer GP, Bertram H, Wessel A, Schieffer B, Westhoff-Bleck M. Safety and efficiency of chronic ACE inhibition in symptomatic heart failure patients with a systemic right ventricle. Int J Cardiol. 2012;154:14–6. Available from: https://pubmed.ncbi.nlm.nih.gov/20843567/.

    Article  PubMed  Google Scholar 

  117. Winter MM, Bouma BJ, Groenink M, Konings TC, Tijssen JGP, Van Veldhuisen DJ, et al. Latest insights in therapeutic options for systemic right ventricular failure: a comparison with left ventricular failure. Heart. 2009;95:960–3.

    Article  CAS  PubMed  Google Scholar 

  118. Bogaard HJ, Natarajan R, Mizuno S, Abbate A, Chang PJ, Chau VQ, et al. Adrenergic receptor blockade reverses right heart remodeling and dysfunction in pulmonary hypertensive rats. Am J Respir Crit Care Med. 2010;182:652–60.

    Article  CAS  PubMed  Google Scholar 

  119. Apitz C, Honjo O, Humpl T, Li J, Assad RS, Cho MY, et al. Biventricular structural and functional responses to aortic constriction in a rabbit model of chronic right ventricular pressure overload. J Thorac Cardiovasc Surg. 2012;144:1494–501.

    Article  PubMed  Google Scholar 

  120. Siffel C, Riehle-Colarusso T, Oster ME, Correa A. Survival of children with hypoplastic left heart syndrome. Pediatrics. 2015;136:e864–70. Available from: https://pubmed.ncbi.nlm.nih.gov/26391936/.

    Article  PubMed  Google Scholar 

  121. Öhman A, El-Segaier M, Bergman G, Hanséus K, Malm T, Nilsson B, et al. Changing epidemiology of hypoplastic left heart syndrome: results of a national Swedish cohort study. J Am Heart Assoc. 2019;8. Available from: https://pubmed.ncbi.nlm.nih.gov/30661430/.

  122. Erikssen G, Aboulhosn J, Lin J, Liestøl K, Estensen ME, Gjesdal O, et al. Survival in patients with univentricular hearts: the impact of right versus left ventricular morphology. Open Heart. 2018;5. Available from: https://pubmed.ncbi.nlm.nih.gov/30364544/.

  123. Metcalf MK, Rychik J. Outcomes in hypoplastic left heart syndrome. Pediatr Clin N Am. 2020;67:945–62.

    Article  Google Scholar 

  124. Hormaza VM, Conaway M, Schneider DS, Vergales JE. The effect of right ventricular function on survival and morbidity following stage 2 palliation: an analysis of the single ventricle reconstruction trial public data set. Congenit Heart Dis. 2019;14:274–9. Available from: https://pubmed.ncbi.nlm.nih.gov/30506893/.

    Article  PubMed  Google Scholar 

  125. Son JS, James A, Fan CPS, Mertens L, McCrindle BW, Manlhiot C, et al. Prognostic value of serial echocardiography in hypoplastic left heart syndrome. Circ Cardiovasc Imaging. 2018;11:e006983. Available from: https://pubmed.ncbi.nlm.nih.gov/30012823/.

    Article  PubMed  Google Scholar 

  126. D’Alto M, Mahadevan VS. Pulmonary arterial hypertension associated with congenital heart disease. Eur Respir Rev. 2012;21:328–37.

    Article  PubMed  Google Scholar 

  127. van der Feen DE, Bartelds B, de Boer RA, Berger RMF. Pulmonary arterial hypertension in congenital heart disease: translational opportunities to study the reversibility of pulmonary vascular disease. Eur Heart J. 2017;38:2034–41. Available from: https://pubmed.ncbi.nlm.nih.gov/28369399.

    Article  PubMed  CAS  Google Scholar 

  128. Galiè N, Humbert M, Vachiery J-L, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endor. Eur Heart J. 2016;37:67–119. Available from: http://www.ncbi.nlm.nih.gov/pubmed/26320113.

    Article  PubMed  Google Scholar 

  129. Dimopoulos K, Diller GP, Koltsida E, Pijuan-Domenech A, Papadopoulou SA, Babu-Narayan SV, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117:2320–8.

    Article  PubMed  Google Scholar 

  130. Gatzoulis MA, Beghetti M, Galiè N, Granton J, Berger RMFF, Lauer A, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008;127:27–32. Available from: https://pubmed.ncbi.nlm.nih.gov/17658633.

    Article  PubMed  Google Scholar 

  131. Van Loon RLE, Roofthooft MTRR, Hillege HL, ten Harkel ADJJ, van Osch-Gevers M, Delhaas T, et al. Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005. Circulation. 2011;124:1755–64. Available from: https://pubmed.ncbi.nlm.nih.gov/21947294.

    Article  PubMed  Google Scholar 

  132. Barst RJ, McGoon MD, Elliott CG, Foreman AJ, Miller DP, Ivy DD. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation. 2012;125:113–22.

    Article  PubMed  Google Scholar 

  133. Berger RMFF, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing Z-CC, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet (London, England). 2012;379:537–46. Available from: https://pubmed.ncbi.nlm.nih.gov/22240409.

    Article  Google Scholar 

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Department of Pediatrics, Erasmus Medical Center, Sophia Children’s Hospital, Rotterdam, The Netherlands

    Beatrijs Bartelds

  2. Department of Pediatric and Congenital Cardiology, Center for Congenital Heart Diseases, Beatrix Children’s Hospital, University Medical Center Groningen, Groningen, The Netherlands

    Johannes M. Douwes & Rolf M. F. Berger

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  1. Beatrijs Bartelds
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  2. Johannes M. Douwes
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  3. Rolf M. F. Berger
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Correspondence to Johannes M. Douwes .

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  1. National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland

    Sean P. Gaine

  2. Free University of Brussels, Brussels, Belgium

    Robert Naeije

  3. Scottish Pulmonary Vascular Unit, Regional Heart and Lung Centre, Glasgow, UK

    Andrew J. Peacock

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Bartelds, B., Douwes, J.M., Berger, R.M.F. (2021). The Right Ventricle in Congenital Heart Diseases. In: Gaine, S.P., Naeije, R., Peacock, A.J. (eds) The Right Heart. Springer, Cham. https://doi.org/10.1007/978-3-030-78255-9_13

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