CONGENITAL HEART DISEASE

Impaired myocardial blood flow and coronary flow reserve of the anatomical right systemic ventricle in patients with congenitally corrected transposition of the great arteries

M Hauser¹, F M Bengel², A Hager¹, A Kuehn¹, S G Nekolla², H Kaemmerer¹, M Schwaiger² and J Hess¹

¹ Deutsches Herzzentrum Munich, Technical University, Department of Paediatric Cardiology, Munich, Germany
² Nuklearmedizinische Klinik, Technical University, Munich

Correspondence to:
Correspondence to:
Dr Michael Hauser, Deutsches Herzzentrum Munich, Technical University, Department of Paediatric Cardiology, Lazarettstrasse 36, 80636 Munich, Germany;
hauser{at}dhm.mhn.de

Objectives: To investigate myocardial blood flow of the morphological right systemic ventricle in unoperated patients with congenitally corrected transposition of the great arteries (CCTGA) by positron emission tomography (PET).

Design: Prospective cross sectional clinical study.

Setting: Tertiary referral centre for paediatric cardiology.

Patients: 15 patients with CCTGA were investigated by PET with nitrogen-13 ammonia at rest and during adenosine vasodilatation. A subgroup of seven patients had isolated CCTGA (group A, mean (SD) age 30.3 (11.9) years) and the remaining eight patients had complex CCTGA associated with subpulmonary stenosis; four of this second group also had ventricular septal defect (group B, mean (SD) age 30.6 (16.4) years). Eleven healthy adults (mean (SD) age 26.2 (5.1) years) served as the control group.

Results: Resting myocardial blood flow was not different between both groups of patients with CCTGA and the controls. Hyperaemic blood flows were significantly lower in both groups of CCTGA than in the control group (mean (SD) 195 (21) ml/100 g/min in group A, 201 (27) ml/100 g/min in group B, 309 (74) ml/100 g/min in the control group; p < 0.001). Thus, coronary flow reserve was significantly lower in both groups of CCTGA than in the control group (mean (SD) 2.5 (0.28) in group A, 2.6 (0.48) in group B, and 4.0 (0.73) in the control group; p < 0.001).

Conclusion: Blood flow measurements suggest that coronary reserve is decreased in the absence of ischaemic symptoms in patients with CCTGA. The global impairment of stress flow dynamics may indicate altered global vasoreactivity, and quantitative changes in microcirculation suggest that their role in the pathogenesis of systemic right ventricular dysfunction is important.

Keywords: congenitally corrected transposition; myocardial blood flow; systemic right ventricle; positron emission tomography

Abbreviations: CCTGA, congenitally corrected transposition of the great arteries; CFR, coronary flow reserve; MBF, myocardial blood flow; NYHA, New York Heart Association; O₂max, maximum oxygen consumption

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Winter, M M, Bouma, B J, Groenink, M, Konings, T C, Tijssen, J G P, van Veldhuisen, D J, Mulder, B J M (2009). Latest insights in therapeutic options for systemic right ventricular failure: a comparison with left ventricular failure. Heart 95: 960-963 [Abstract] [Full Text]  
• Quinn, D. W., McGuirk, S. P., Metha, C., Nightingale, P., de Giovanni, J. V., Dhillon, R., Miller, P., Stumper, O., Wright, J. G., Barron, D. J., Brawn, W. J. (2008). The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction.. J. Thorac. Cardiovasc. Surg. 135: 1137-1144.e2 [Abstract] [Full Text]  
• Warnes, C. A. (2006). Transposition of the Great Arteries. Circulation 114: 2699-2709 [Abstract] [Full Text]  
• Fratz, S, Hauser, M, Bengel, F M, Hager, A, Kaemmerer, H, Schwaiger, M, Hess, J, Stern, H C (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: 1673-1677 [Abstract] [Full Text]  
• Davlouros, P A, Niwa, K, Webb, G, Gatzoulis, M A (2006). The right ventricle in congenital heart disease. Heart 92: i27-i38 [Abstract] [Full Text]  
• Tops, L. F., Roest, A. A. W., Lamb, H. J., Vliegen, H. W., Helbing, W. A., van Der Wall, E. E., de Roos, A. (2005). Intraatrial Repair of Transposition of the Great Arteries: Use of MR Imaging after Exercise to Evaluate Regional Systemic Right Ventricular Function. Radiology 237: 861-867 [Abstract] [Full Text]  
• Salehian, O., Schwerzmann, M., Merchant, N., Webb, G. D., Siu, S. C., Therrien, J. (2004). Assessment of Systemic Right Ventricular Function in Patients With Transposition of the Great Arteries Using the Myocardial Performance Index: Comparison With Cardiac Magnetic Resonance Imaging. Circulation 110: 3229-3233 [Abstract] [Full Text]  
• Derrick, G., Deanfield, J. E. (2004). Decline in ventricular function and clinical condition after Mustard repair. Eur Heart J 25: 1863-1864 [Full Text]  
• Graham, T. P. Jr (2004). The year in congenital heart disease. J Am Coll Cardiol 43: 2132-2141 [Full Text]