Abstract
Objectives
To test the hypothesis that myocardial scars cause systolic dysfunction in patients with transposition of the great arteries and a systemic right ventricle.
Methods
We retrospectively analyzed 20 consecutive patients (10 male, mean age 27.3 years) with a systemic right ventricle who underwent cardiac magnetic resonance imaging with 1.5 T. Cine steady-state free-precession sequences were performed to obtain volumes and function. Phase-sensitive inversion-recovery (PSIR) delayed-enhancement imaging was performed to detect myocardial scars. Tricuspid insufficiency was detected with echocardiography. Furthermore, the presence of arrhythmias and New York Heart Association (NYHA) class were assessed.
Results
Mean ejection fraction of systemic right ventricles was 43 ± 11 %, mean end-diastolic volume index was 111 ± 37 ml/m2. Delayed-enhancement imaging revealed only one myocardial scar in the wall of a right ventricular aneurysm. All patients but one (95 %) presented with tricuspid insufficiency. Clinically relevant arrhythmias were present in 13/20 patients (65 %). The majority of patients (90 %) were NYHA class I or II. Arrhythmias, tricuspid insufficiency and NYHA class were not associated with right ventricular ejection fraction.
Conclusions
Although right ventricular function was clearly impaired in our patient cohort, there was only one myocardial scar. Our results show that myocardial scarring assessed by PSIR delayed-enhancement imaging is not the underlying pathology of systemic right ventricular failure.
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References
Warnes CA (2006) Transposition of the great arteries. Circulation 114(24):2699–2709
Bogers AJ, Head SJ, de Jong PL, Witsenburg M, Kappetein AP (2010) Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation. J Cardiothorac Surg 5:74
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
Babu-Narayan SV, Goktekin O, Moon JC, Broberg CS, Pantely GA, Pennell DJ et al (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
Fratz S, Hauser M, Bengel FM, Hager A, Kaemmerer H, Schwaiger M et al (2006) Myocardial scars determined by delayed-enhancement magnetic resonance imaging and positron emission tomography are not common in right ventricles with systemic function in long-term follow up. Heart 92(11):1673–1677
Plaisier AS, Burgmans MC, Vonken EP, Prakken NH, Cox MG, Hauer RN et al (2011) Image quality assessment of the right ventricle with three different delayed enhancement sequences in patients suspected of ARVC/D. Int J Cardiovasc Imaging 28(3):505–601
Dickstein K, Cohen-Solal A, Filippatos G, McMurray JJ, Ponikowski P, Poole-Wilson PA et al (2008) ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure 2008: the Task Force for the Diagnosis and Treatment of Acute and Chronic Heart Failure 2008 of the European Society of Cardiology. Developed in collaboration with the Heart. Eur Heart J 29(19):2388–2442
Beerbaum P, Barth P, Kropf S, Sarikouch S, Kelter-Kloepping A, Franke D et al (2009) Cardiac function by MRI in congenital heart disease: impact of consensus training on interinstitutional variance. J Magn Reson Imaging 30(5):956–966
Desch S, Engelhardt H, Meissner J, Eitel I, Sareban M, Fuernau G et al (2011) Reliability of myocardial salvage assessment by cardiac magnetic resonance imaging in acute reperfused myocardial infarction. Int J Cardiovasc Imaging 28(2):267–272
Winter MM, Bernink FJ, Groenink M, Bouma BJ, van Dijk AP, Helbing WA et al (2008) Evaluating the systemic right ventricle by CMR: the importance of consistent and reproducible delineation of the cavity. J Cardiovasc Magn Reson 10:40
Bondarenko O, Beek AM, Hofman MB, Kühl HP, Twisk JW, van Dockum WG et al (2005) Standardizing the definition of hyperenhancement in the quantitative assessment of infarct size and myocardial viability using delayed contrast-enhanced CMR. J Cardiovasc Magn Reson 7(2):481–485
Moon JC, McKenna WJ, McCrohon JA, Elliott PM, Smith GC, Pennell DJ (2003) Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol 41(9):1561–1567
Giardini A, Lovato L, Donti A, Formigari R, Oppido G, Gargiulo G et al (2006) 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 98(9):1277–1282
Kayser HW, van der Wall EE, Sivananthan MU, Plein S, Bloomer TN, de Roos A (2002) Diagnosis of arrhythmogenic right ventricular dysplasia: a review. Radiographics 22(3):639–648 (Discussion 649–650)
Taylor AJ, Cerqueira M, Hodgson JM, Mark D, Min J, O’Gara P et al (2010) ACCF/SCCT/ACR/AHA/ASE/ASNC/NASCI/SCAI/SCMR 2010 Appropriate Use Criteria for Cardiac Computed Tomography. A Report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, the Society of Cardiovascular Computed Tomography. Circulation 122(21):e525–e555
Park SJ, On YK, Kim JS, Park SW, Yang JH, Jun TG et al (2012) Relation of fragmented QRS complex to right ventricular fibrosis detected by late gadolinium enhancement cardiac magnetic resonance in adults with repaired tetralogy of fallot. Am J Cardiol 109(1):110–115
Huber AM, Schoenberg SO, Hayes C, Spannagl B, Engelmann MG, Franz WM et al (2005) Phase-sensitive inversion-recovery MR imaging in the detection of myocardial infarction. Radiology 237(3):854–860
Kellman P, Arai AE, McVeigh ER, Aletras AH (2002) Phase-sensitive inversion recovery for detecting myocardial infarction using gadolinium-delayed hyperenhancement. Magn Reson Med 47(2):372–383
Klein C, Nekolla SG, Bengel FM, Momose M, Sammer A, Haas F et al (2002) Assessment of myocardial viability with contrast-enhanced magnetic resonance imaging: comparison with positron emission tomography. Circulation 105(2):162–167
Reiter T, Ritter O, Prince MR, Nordbeck P, Wanner C, Nagel E et al (2012) Minimizing risk of nephrogenic systemic fibrosis in cardiovascular magnetic resonance. J Cardiovasc Magn Reson 14:31
Fernandez-Teran MA, Hurle JM (1982) Myocardial fiber architecture of the human heart ventricles. Anat Rec 204(2):137–147
Sanchez-Quintana D, Garcia-Martinez V, Hurle JM (1990) Myocardial fiber architecture in the human heart. Anatomical demonstration of modifications in the normal pattern of ventricular fiber architecture in a malformed adult specimen. Acta Anat (Basel) 138(4):352–358
Sanchez-Quintana D, Garcia-Martinez V, Climent V, Hurle JM (1995) Morphological changes in the normal pattern of ventricular myoarchitecture in the developing human heart. Anat Rec 243(4):483–495
Lubiszewska B, Gosiewska E, Hoffman P, Teresinska A, Rózanski J, Piotrowski W et al (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
Hornung TS, Bernard EJ, Celermajer DS, Jaeggi E, Howman-Giles RB, Chard RB et al (1999) Right ventricular dysfunction in congenitally corrected transposition of the great arteries. Am J Cardiol 84(9):1116–1119 A10
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
Hauser M, Bengel FM, Hager A, Kuehn A, Nekolla SG, Kaemmerer H et al (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
Grothoff M, Hoffmann J, Abdul-Khaliq H, Lehmkuhl L, Dähnert I, Berger F et al (2012) Right ventricular hypertrophy after atrial switch operation: normal adaptation process or risk factor? A cardiac magnetic resonance study. Clin Res Cardiol doi: 10.1007/s00392-012-0485-6
Fattori R, Biagini E, Lorenzini M, Buttazzi K, Lovato L, Rapezzi C (2010) Significance of magnetic resonance imaging in apical hypertrophic cardiomyopathy. Am J Cardiol 105(11):1592–1596
Rathod RH, Prakash A, Powell AJ, Geva T (2010) Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation. J Am Coll Cardiol 55(16):1721–1728
Maron MS (2009) The current and emerging role of cardiovascular magnetic resonance imaging in hypertrophic cardiomyopathy. J Cardiovasc Transl Res 2(4):415–425
Weber KT, Brilla CG (1991) Patho logical hypertrophy and cardiac interstitium. Fibrosis and renin–angiotensin–aldosterone system. Circulation 83(6):1849–1865
Schelbert EB, Testa SM, Meier CG, Ceyrolles WJ, Levenson JE, Blair AJ et al (2011) Myocardial extravascular extracellular volume fraction measurement by gadolinium cardiovascular magnetic resonance in humans: slow infusion versus bolus. J Cardiovasc Magn Reson 13:16
Wu E, Judd RM, Vargas JD, Klocke FJ, Bonow RO, Kim RJ (2001) Visualisation of presence, location, and transmural extent of healed Q-wave and non-Q-wave myocardial infarction. Lancet 357(9249):21–28
Ricciardi MJ, Wu E, Davidson CJ, Choi KM, Klocke FJ, Bonow RO et al (2001) Visualization of discrete microinfarction after percutaneous coronary intervention associated with mild creatine kinase-MB elevation. Circulation 103(23):2780–2783
Flett AS, Hayward MP, Ashworth MT, Hansen MS, Taylor AM, Elliott PM et al (2010) Equilibrium contrast cardiovascular magnetic resonance for the measurement of diffuse myocardial fibrosis: preliminary validation in humans. Circulation 122(2):138–144
Ugander M, Oki AJ, Hsu LY, Kellman P, Greiser A, Aletras AH et al (2012) Extracellular volume imaging by magnetic resonance imaging provides insights into overt and sub-clinical myocardial pathology. Eur Heart J 33(10):1268–1278
Grothoff M, Hoffmann J, Lehmkuhl L, Abdul-Khaliq H, Nitzsche S, Mahler A et al (2011) Time course of right ventricular functional parameters after surgical correction of tetralogy of Fallot determined by cardiac magnetic resonance. Clin Res Cardiol 100(4):343–350
Shehata ML, Cheng S, Osman NF, Bluemke DA, Lima JA (2009) Myocardial tissue tagging with cardiovascular magnetic resonance. J Cardiovasc Magn Reson 11:55
Goetschalckx K, Rademakers F, Bogaert J (2010) Right ventricular function by MRI. Curr Opin Cardiol 25(5):451–455
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Preim, U., Hoffmann, J., Lehmkuhl, L. et al. Systemic right ventricles rarely show myocardial scars in cardiac magnetic resonance delayed-enhancement imaging. Clin Res Cardiol 102, 337–344 (2013). https://doi.org/10.1007/s00392-013-0539-4
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DOI: https://doi.org/10.1007/s00392-013-0539-4