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A 6-year follow-up study of adult patients with congenitally corrected transposition

Published online by Cambridge University Press:  19 December 2014

Mirta Koželj*
Affiliation:
Department of Cardiology, University Medical Centre Ljubljana, Zaloška 7, 1525 Ljubljana, Slovenia Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1000 Ljubljana, Slovenia
Marta Cvijić
Affiliation:
Department of Cardiology, University Medical Centre Ljubljana, Zaloška 7, 1525 Ljubljana, Slovenia
Pavel Berden
Affiliation:
Institute of Radiology, University Medical Centre Ljubljana, Zaloška 7, 1525 Ljubljana, Slovenia Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1000 Ljubljana, Slovenia
Tomaž Podnar
Affiliation:
Department of Paediatrics, University Medical Centre Ljubljana, Zaloška 7, 1525 Ljubljana, Slovenia Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1000 Ljubljana, Slovenia
*
Correspondence to: Prof. Mirta Koželj, MD, Department of Cardiology, University Medical Centre Ljubljana, Zaloška 7, 1525 Ljubljana, Slovenia. Tel:+38 601 522 2844; Fax:+38 601 522 2828; E-mail: [email protected]

Abstract

The aims of this study were to assess the development of heart failure in patients with congenitally corrected transposition of the great arteries in a medium-term follow-up, to identify the impact of tricuspid regurgitation on the development of heart failure, and to determine the most reliable marker for its identification. The prospective 6-year follow-up study included 19 adult patients. All patients were evaluated clinically by the determination of N-terminal pro-hormone brain natriuretic peptide levels, exercise stress testing, echocardiography magnetic resonance, or CT. Among them, two patients died of heart failure. There was a decline in exercise capacity and systolic systemic ventricular function (p=0.011). Systemic ventricular ejection fraction decreased (48.3±13.7 versus 42.7±12.7%, p=0.001). Tissue Doppler imaging showed a decline in peak tricuspid systolic annular velocity (10.3±2.0 versus 8.3±2.5 cm/second, p=0.032) and peak tricuspid early diastolic annular velocity (14.6±4.3 versus 12.0±4.5 cm/second, p=0.048). The tricuspid regurgitation did not increase significantly. N-terminal pro-hormone brain natriuretic peptide levels increased (127.0 ng/L(82.3–305.8) versus 226.0 ng/L(112.5–753.0), p=0.022). Progressive exercise intolerance in congenitally corrected transposition of the great arteries appears to be driven mainly by a progression in systemic right ventricular dysfunction. Tricuspid regurgitation is likely to play a role, especially in patients with structural abnormalities of the tricuspid valve – Ebstein anomaly. The N-terminal pro-hormone brain natriuretic peptide levels and tissue Doppler parameters appear sensitive in detecting changes over time and may guide management.

Type
Original Articles
Copyright
© Cambridge University Press 2014 

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References

1. Graham, TP Jr, Bernard, YD, Mellen, BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multiinstitutional study. J Am Coll Cardiol 2000; 36: 255261.CrossRefGoogle Scholar
2. Mongeon, FP, Connolly, HM, Dearani, JA, Li, Z, Warnes, C. Congenitally corrected transposition of the great arteries. J Am Coll Cardiol 2011; 57: 20082017.CrossRefGoogle ScholarPubMed
3. Rutledge, JM, Nihill, MR, Frazer, CD, Smith, EO, Bezold, LI. Outcome of 121 patients with congenitally corrected transposition of the great arteries. Pediatr Cardiol 2002; 23: 137145.CrossRefGoogle ScholarPubMed
4. Kozelj, M, Prokselj, K, Berden, P, et al. The syndrome of cardiac failure in adults with congenitally corrected transposition. Cardiol Young 2008; 18: 599607.CrossRefGoogle ScholarPubMed
5. Baumgartner, H, Bonhoeffer, P, De Groot, N, de Haan, F, Deanfield, JE, Galie, N. ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31: 29152957.Google ScholarPubMed
6. Bruce, RA, Blackmon, JR, Jones, JW, Strait, G. Exercising testing in adult normal subjects and cardiac patients. Ann Noninvasive Electrocardiol 2004; 9: 291303.CrossRefGoogle ScholarPubMed
7. Lang, RM, Bierig, M, Devereux, RB, Flachskampf, FA, Foster, E, Pellikka, PA. Recommendations for chamber quantification: a report from the American Society of Echocarardiography’s Guidelines and Standards Committee and Chamber Quantification Writing Group, developed in conjunction with European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 2005; 18: 14401463.CrossRefGoogle Scholar
8. Alam, M, Wardell, J, Andersson, E, Samad, BA, Nordlander, R. Characteristics of mitral and tricuspid annular velocities determined by pulsed wave Doppler tissue imaging in healthy subjects. J Am Soc Echocardiogr 1999; 12: 618628.CrossRefGoogle ScholarPubMed
9. Suty-Selton, C, Mock, L, Piquemal, R, Vancon, A-C, Nippert, M, Juilliere, Y. Doppler tissue imaging of the right ventricle. Arch Mal Coeur Vaiss 2002; 95: 933937.Google Scholar
10. Gustafsson, F, Badskjaer, J, Stensgaard, H, Poulsen, AH, Hildebrandt, P. Value of N-terminal proBNP in the diagnosis of left ventricular systolic dysfunction in primary care patients referred for echocardiography. Heart Drug 2003; 3: 141146.CrossRefGoogle Scholar
11. Lorenz, CH. The range of normal values of cardiovascular structures in infants, children and adolescents by MRI. Pediatr Cardiol 2000; 21: 3746.CrossRefGoogle Scholar
12. Alfakih, K, Plein, S, Thiele, H, Jones, T, Ridgway, JP, Sivananthan, MU. Normal human left and right ventricular dimensions for MRI as assessed by turbo gradient echo and steady-state free precession imaging sequences. J Magn Reson Imaging 2003; 17: 323329.CrossRefGoogle ScholarPubMed
13. Maffei, E, Messalli, G, Martini, C, et al. Left and right ventricular assessment with cardiac CT: validation study vs Cardiac MR. Eur Radiol 2012; 22: 10411049.CrossRefGoogle ScholarPubMed
14. Grewal, J, Crean, A, Garceau, P, et al. Subaortic right ventricular characteristics and relationship to exercise capacity in congenitally corrected transposition of the great arteries. J Am Soc Echocardiogr 2012; 12: 12151221.CrossRefGoogle Scholar
15. Bonello, B, Kilner, PJ. Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment. Arch Cardiovasc Dis 2012; 105: 605613.CrossRefGoogle ScholarPubMed
16. Dimas, AP, Moodie, DS, Sterba, R, Gill, CC. Long-term function of the morphologic right ventricle in adult patients with corrected transposition of the great arteries. Am Heart J 1989; 118: 526530.CrossRefGoogle ScholarPubMed
17. Dobson, R, Danton, M, Nicola, W, Hamish, W. The natural and unnatural history of the systemic right ventricle in adult survivors. J Thorac Surg 2013; 145: 14931501.CrossRefGoogle ScholarPubMed
18. Diller, GP, Dimopoulos, K, Okonko, D, et al Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005; 112: 828835.CrossRefGoogle ScholarPubMed
19. Prieto, LR, Hordof, AL, Secic, M, Rosembaum, MS, Gersony, WM. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries. Circulation 1998; 98: 9971005.CrossRefGoogle ScholarPubMed
20. Lewis, M, Ginns, J, Rosenbaum, M. Is systemic right ventricular function by cardiac MRI related to the degree of tricuspid regurgitation in congenitally corrected transposition of the great arteries. Int J Cardiol 2014; 174: 586589.CrossRefGoogle Scholar
21. Szymański, P, Klisiewicz, A, Lubiszewska, B, et al. Functional anatomy of tricuspid regurgitation in patients with systemic right ventricles. Am Soc Echocardiogr 2010; 23: 504510.CrossRefGoogle ScholarPubMed
22. Tay, EL, Frogoudaki, A, Inuzuka, R, et al. Exercise intolerance in patients with congenitally corrected transposition of the great arteries relates to right ventricular filling pressures. Int J Cardiol 2011; 147: 219223.CrossRefGoogle ScholarPubMed