Abstract
There are two predominant scenarios where the right ventricle acts as the systemic ventricle, namely congenitally corrected transposition of the great arteries (CC-TGA) and patients with D-transposition of the great arteries (D-TGA) who have undergone an atrial switch operation. There is evidence that having a right ventricle in the systemic position predisposes to the development of heart failure. In this review, we aim to explore the proposed mechanisms and pathophysiology for heart failure in patients with a systemic right ventricle in these two subsets of patients.
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Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A (2008) Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr 153(6):807–813. https://doi.org/10.1016/j.jpeds.2008.05.059
Senning A (1966) Surgical correction of transposition of the great vessels. Surgery 59(2):334–336
WT MUSTARD (1964) Successful two-stage correction of transposition of the great vessels. Surgery 55:469–472
Jatene AD, Fontes VF, Paulista PP, Souza LC, Neger F, Galantier M, Sousa JE (1976) Anatomic correction of transposition of the great vessels. J Thorac Cardiovasc Surg 72(3):364–370
Atallah J, Rutledge JM, Dyck JD (2013) Congenitally corrected transposition of the great arteries (Atrioventricular and Ventriculoarterial discordance). In: Hugh D. Allen RES, David J. Driscoll, Timothy F. Feltes (ed) Moss and Adams’ Heart Disease in Infants, Children, and Adolescents, vol II. 8 edn. Lippincott Williams & Wilkins, Philadelphia, Pennsylvania, pp 1147-1160
Wallis GA, Debich-Spicer D, Anderson RH (2011) Congenitally corrected transposition. Orphanet J Rare Dis 6:22. https://doi.org/10.1186/1750-1172-6-22
Allwork SP (1976) The morphogenesis of transposition of the great arteries, 1875-1975. Eur J Cardiol 4(1):3–11
ANDERSON RC, LILLEHEI CW, LESTER RG (1957) Corrected transposition of the great vessels of the heart: a review of 17 cases. Pediatrics 20(4):626–646
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(3 Pt 2):I87–I91
Hagler DJ, Ritter DG, Mair DD, Tajik AJ, Seward JB, Fulton RE, Ritman EL (1979) Right and left ventricular function after the Mustard procedure in transposition of the great arteries. Am J Cardiol 44(2):276–283. https://doi.org/10.1016/0002-9149(79)90317-5
Benson LN, Bonet J, McLaughlin P, Olley PM, Feiglin D, Druck M, Trusler G, Rowe RD, Morch J (1982) Assessment of right ventricular function during supine bicycle exercise after Mustard’s operation. Circulation 65(6):1052–1059. https://doi.org/10.1161/01.cir.65.6.1052
Moons P, Gewillig M, Sluysmans T, Verhaaren H, Viart P, Massin M, Suys B, Budts W, Pasquet A, De Wolf D, Vliers A (2004) Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart 90(3):307–313. https://doi.org/10.1136/hrt.2002.007138
Szymański P, Klisiewicz A, Lubiszewska B, Lipczyńska M, Michałek P, Janas J, Hoffman P (2009) Application of classic heart failure definitions of asymptomatic and symptomatic ventricular dysfunction and heart failure symptoms with preserved ejection fraction to patients with systemic right ventricles. Am J Cardiol 104(3):414–418. https://doi.org/10.1016/j.amjcard.2009.03.057
Dos L, Teruel L, Ferreira IJ, Rodriguez-Larrea J, Miro L, Girona J, Albert DC, Gonçalves A, Murtra M, Casaldaliga J (2005) Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries. Heart 91(5):652–656. https://doi.org/10.1136/hrt.2003.029769
Vejlstrup N, Sørensen K, Mattsson E, Thilén U, Kvidal P, Johansson B, Iversen K, Søndergaard L, Dellborg M, Eriksson P (2015) Long-term outcome of Mustard/Senning correction for transposition of the great arteries in Sweden and Denmark. Circulation 132(8):633–638. https://doi.org/10.1161/CIRCULATIONAHA.114.010770
Yap SC, Harris L (2009) Sudden cardiac death in adults with congenital heart disease. Expert Rev Cardiovasc Ther 7(12):1605–1620. https://doi.org/10.1586/erc.09.153
Cuypers JA, Eindhoven JA, Slager MA, Opić P, Utens EM, Helbing WA, Witsenburg M, van den Bosch AE, Ouhlous M, van Domburg RT, Rizopoulos D, Meijboom FJ, Bogers AJ, Roos-Hesselink JW (2014) The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years. Eur Heart J 35(25):1666–1674. https://doi.org/10.1093/eurheartj/ehu102
Piran S, Veldtman G, Siu S, Webb GD, Liu PP (2002) Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation 105(10):1189–1194
Norozi K, Wessel A, Alpers V, Arnhold JO, Geyer S, Zoege M, Buchhorn R (2006) Incidence and risk distribution of heart failure in adolescents and adults with congenital heart disease after cardiac surgery. Am J Cardiol 97(8):1238–1243. https://doi.org/10.1016/j.amjcard.2005.10.065
Connelly MS, Liu PP, Williams WG, Webb GD, Robertson P, McLaughlin PR (1996) Congenitally corrected transposition of the great arteries in the adult: functional status and complications. J Am Coll Cardiol 27(5):1238–1243. https://doi.org/10.1016/0735-1097(95)00567-6
Bolger AP, Sharma R, Li W, Leenarts M, Kalra PR, Kemp M, Coats AJ, Anker SD, Gatzoulis MA (2002) Neurohormonal activation and the chronic heart failure syndrome in adults with congenital heart disease. Circulation 106(1):92–99
Lubiszewska B, Gosiewska E, Hoffman P, Teresińska A, Rózański J, Piotrowski W, Rydlewska-Sadowska W, Kubicka K, Ruzyłło W (2000) Myocardial perfusion and function of the systemic right ventricle in patients after atrial switch procedure for complete transposition: long-term follow-up. J Am Coll Cardiol 36(4):1365–1370
Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, van Dijk AP, Vliegen HW, Grobbee DE, Mulder BJ (2010) Mortality in adult congenital heart disease. Eur Heart J 31(10):1220–1229. https://doi.org/10.1093/eurheartj/ehq032
Huhta JC, Maloney JD, Ritter DG, Ilstrup DM, Feldt RH (1983) Complete atrioventricular block in patients with atrioventricular discordance. Circulation 67(6):1374–1377. https://doi.org/10.1161/01.cir.67.6.1374
Masden R, Franch R (1980) Isolated congenitally corrected transposition of the great arteries. In: Hurst J (ed) The heart, vol III. McGraw-Hill, New York, pp 59–83
Hauser M, Bengel FM, Hager A, Kuehn A, Nekolla SG, Kaemmerer H, Schwaiger M, Hess J (2003) 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 89(10):1231–1235. https://doi.org/10.1136/heart.89.10.1231
Graham TP, Bernard YD, Mellen BG, Celermajer D, Baumgartner H, Cetta F, Connolly HM, Davidson WR, Dellborg M, Foster E, Gersony WM, Gessner IH, Hurwitz RA, Kaemmerer H, Kugler JD, Murphy DJ, Noonan JA, Morris C, Perloff JK, Sanders SP, Sutherland JL (2000) Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol 36(1):255–261
Prieto LR, Hordof AJ, Secic M, Rosenbaum MS, Gersony WM (1998) Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries. Circulation 98(10):997–1005
Anderson RH, Becker AE, Arnold R, Wilkinson JL (1974) The conducting tissues in congenitally corrected transposition. Circulation 50(5):911–923
Lundstrom U, Bull C, Wyse RK, Somerville J (1990) The natural and “unnatural” history of congenitally corrected transposition. Am J Cardiol 65(18):1222–1229
Armour JA, Randall WC (1970) Structural basis for cardiac function. Am J Phys 218(6):1517–1523. https://doi.org/10.1152/ajplegacy.1970.218.6.1517
Rüssel IK, Götte MJ, Bronzwaer JG, Knaapen P, Paulus WJ, van Rossum AC (2009) Left ventricular torsion: an expanding role in the analysis of myocardial dysfunction. JACC Cardiovasc Imaging 2(5):648–655. https://doi.org/10.1016/j.jcmg.2009.03.001
Vitarelli A, Terzano C (2010) Do we have two hearts? New insights in right ventricular function supported by myocardial imaging echocardiography. Heart Fail Rev 15(1):39–61. https://doi.org/10.1007/s10741-009-9154-x
Damiano RJ, La Follette P, Cox JL, Lowe JE, Santamore WP (1991) Significant left ventricular contribution to right ventricular systolic function. Am J Phys 261(5 Pt 2):H1514–H1524. https://doi.org/10.1152/ajpheart.1991.261.5.H1514
Santamore WP, Dell'Italia LJ (1998) Ventricular interdependence: significant left ventricular contributions to right ventricular systolic function. Prog Cardiovasc Dis 40(4):289–308
Redington AN, Gray HH, Hodson ME, Rigby ML, Oldershaw PJ (1988) Characterisation of the normal right ventricular pressure-volume relation by biplane angiography and simultaneous micromanometer pressure measurements. Br Heart J 59(1):23–30. https://doi.org/10.1136/hrt.59.1.23
Theissen P, Kaemmerer H, Sechtem U, Luhmer I, Smolarz K, Kallfelz HC, Schicha H (1991) Magnetic resonance imaging of cardiac function and morphology in patients with transposition of the great arteries following Mustard procedure. Thorac Cardiovasc Surg 39(Suppl 3):221–224. https://doi.org/10.1055/s-2007-1020023
Pettersen E, Helle-Valle T, Edvardsen T, Lindberg H, Smith HJ, Smevik B, Smiseth OA, Andersen K (2007) Contraction pattern of the systemic right ventricle shift from longitudinal to circumferential shortening and absent global ventricular torsion. J Am Coll Cardiol 49(25):2450–2456. https://doi.org/10.1016/j.jacc.2007.02.062
Hornung TS, Bernard EJ, Jaeggi ET, Howman-Giles RB, Celermajer DS, Hawker RE (1998) Myocardial perfusion defects and associated systemic ventricular dysfunction in congenitally corrected transposition of the great arteries. Heart 80(4):322–326. https://doi.org/10.1136/hrt.80.4.322
Hornung TS, Bernard EJ, Celermajer DS, Jaeggi E, Howman-Giles RB, Chard RB, Hawker RE (1999) Right ventricular dysfunction in congenitally corrected transposition of the great arteries. Am J Cardiol 84(9):1116–1119 A1110
Benson LN, Burns R, Schwaiger M, Schelbert HR, Lewis AB, Freedom RM, Olley PM, McLaughlin P, Rowe RD (1986) Radionuclide angiographic evaluation of ventricular function in isolated congenitally corrected transposition of the great arteries. Am J Cardiol 58(3):319–324
Babu-Narayan SV, Goktekin O, Moon JC, Broberg CS, Pantely GA, Pennell DJ, Gatzoulis MA, Kilner PJ (2005) Late gadolinium enhancement cardiovascular magnetic resonance of the systemic right ventricle in adults with previous atrial redirection surgery for transposition of the great arteries. Circulation 111(16):2091–2098. https://doi.org/10.1161/01.CIR.0000162463.61626.3B
Singh TP, Humes RA, Muzik O, Kottamasu S, Karpawich PP, Di Carli MF (2001) Myocardial flow reserve in patients with a systemic right ventricle after atrial switch repair. J Am Coll Cardiol 37(8):2120–2125. https://doi.org/10.1016/s0735-1097(01)01283-9
Lowensohn HS, Khouri EM, Gregg DE, Pyle RL, Patterson RE (1976) Phasic right coronary artery blood flow in conscious dogs with normal and elevated right ventricular pressures. Circ Res 39(6):760–766. https://doi.org/10.1161/01.res.39.6.760
Parrish MD, Graham TP, Bender HW, Jones JP, Patton J, Partain CL (1983) Radionuclide angiographic evaluation of right and left ventricular function during exercise after repair of transposition of the great arteries. Comparison with normal subjects and patients with congenitally corrected transposition. Circulation 67(1):178–183. https://doi.org/10.1161/01.cir.67.1.178
Wernovsky G (2013) Transposition of the great arteries. In: Hugh D. Allen RES, David J. Driscoll, Timothy F. Feltes (ed) Moss and Adams’ Heart Disease in Infants, Children, and Adolescents, vol II. 8 edn. Lippincott Williams & Wilkins, Philadelphia, Pennsylvania, pp 1097-1146
Khairy P (2017) Sudden cardiac death in transposition of the great arteries with a Mustard or Senning baffle: the myocardial ischemia hypothesis. Curr Opin Cardiol 32(1):101–107
Rog B, Salapa K, Okolska M, Dluzniewska N, Werynski P, Podolec P, Tomkiewicz-Pajak L (2019) Clinical evaluation of exercise capacity in adults with systemic right ventricle. Tex Heart Inst J 46(1):14–20. https://doi.org/10.14503/THIJ-17-6408
Reybrouck T, Mertens L, Brown S, Eyskens B, Daenen W, Gewillig M (2001) Long-term assessment and serial evaluation of cardiorespiratory exercise performance and cardiac function in patients with atrial switch operation for complete transposition. Cardiol Young 11(1):17–24
Paul MH, Wessel HU (1999) Exercise studies in patients with transposition of the great arteries after atrial repair operations (Mustard/Senning): a review. Pediatr Cardiol 20(1):49–55; discussion 56. https://doi.org/10.1007/s002469900395
Meijboom F, Szatmari A, Deckers JW, Utens EM, Roelandt JR, Bos E, Hess J (1996) Long-term follow-up (10 to 17 years) after Mustard repair for transposition of the great arteries. J Thorac Cardiovasc Surg 111(6):1158–1168
Sterrett LE, Ebenroth ES, Montgomery GS, Schamberger MS, Hurwitz RA (2011) Pulmonary limitation to exercise after repair of D-transposition of the great vessels: atrial baffle versus arterial switch. Pediatr Cardiol 32(7):910–916. https://doi.org/10.1007/s00246-011-0013-x
Müller J, Hess J, Hörer J, Hager A (2013) Persistent superior exercise performance and quality of life long-term after arterial switch operation compared to that after atrial redirection. Int J Cardiol 166(2):381–384. https://doi.org/10.1016/j.ijcard.2011.10.116
Derrick GP, Narang I, White PA, Kelleher A, Bush A, Penny DJ, Redington AN (2000) 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 102(19 Suppl 3):III154–III159
Matthys D, De Wolf D, Verhaaren H (1996) Lack of increase in stroke volume during exercise in asymptomatic adolescents in sinus rhythm after intraatrial repair for simple transposition of the great arteries. Am J Cardiol 78(5):595–596. https://doi.org/10.1016/s0002-9149(96)00375-x
Presbitero P, Somerville J, Rabajoli F, Stone S, Conte MR (1995) Corrected transposition of the great arteries without associated defects in adult patients: clinical profile and follow up. Br Heart J 74(1):57–59. https://doi.org/10.1136/hrt.74.1.57
Acar P, Sidi D, Bonnet D, Aggoun Y, Bonhoeffer P, Kachaner J (1998) Maintaining tricuspid valve competence in double discordance: a challenge for the paediatric cardiologist. Heart 80(5):479–483. https://doi.org/10.1136/hrt.80.5.479
Talwar S, Ahmed T, Saxena A, Kothari SS, Juneja R, Airan B (2013) Morphology, surgical techniques, and outcomes in patients above 15 years undergoing surgery for congenitally corrected transposition of great arteries. World J Pediatr Congenit Heart Surg 4(3):271–277. https://doi.org/10.1177/2150135113476717
Filippov AA, Del Nido PJ, Vasilyev NV (2016) Management of systemic right ventricular failure in patients with congenitally corrected transposition of the great arteries. Circulation 134(17):1293–1302. https://doi.org/10.1161/CIRCULATIONAHA.116.022106
van der Bom T, Winter MM, Knaake JL, Cervi E, de Vries LS, Balducci A, Meregalli PG, Pieper PG, van Dijk AP, Bonvicini M, Mulder BJ, Bouma BJ (2015) Long-term benefits of exercise training in patients with a systemic right ventricle. Int J Cardiol 179:105–111. https://doi.org/10.1016/j.ijcard.2014.10.042
Jauvert G, Rousseau-Paziaud J, Villain E, Iserin L, Hidden-Lucet F, Ladouceur M, Sidi D (2009) Effects of cardiac resynchronization therapy on echocardiographic indices, functional capacity, and clinical outcomes of patients with a systemic right ventricle. Europace 11(2):184–190. https://doi.org/10.1093/europace/eun319
Diller GP, Okonko D, Uebing A, Ho SY, Gatzoulis MA (2006) Cardiac resynchronization therapy for adult congenital heart disease patients with a systemic right ventricle: analysis of feasibility and review of early experience. Europace 8(4):267–272. https://doi.org/10.1093/europace/euj048
Puley G, Siu S, Connelly M, Harrison D, Webb G, Williams WG, Harris L (1999) Arrhythmia and survival in patients >18 years of age after the mustard procedure for complete transposition of the great arteries. Am J Cardiol 83(7):1080–1084
Rutledge JM, Nihill MR, Fraser CD, Smith OE, McMahon CJ, Bezold LI (2002) Outcome of 121 patients with congenitally corrected transposition of the great arteries. Pediatr Cardiol 23(2):137–145. https://doi.org/10.1007/s00246-001-0037-8
Sarkar D, Bull C, Yates R, Wright D, Cullen S, Gewillig M, Clayton R, Tunstill A, Deanfield J (1999) Comparison of long-term outcomes of atrial repair of simple transposition with implications for a late arterial switch strategy. Circulation 100(Suppl 2):II-176–II-181
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Andrade, L., Carazo, M., Wu, F. et al. Mechanisms for heart failure in systemic right ventricle. Heart Fail Rev 25, 599–607 (2020). https://doi.org/10.1007/s10741-019-09902-1
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DOI: https://doi.org/10.1007/s10741-019-09902-1