The DOI matches its Crossref record; 14 of the 14 references it cites were found in a registry or an index.
Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease Evidence for a disease specific abnormality of the myocardial interstitium
Abstract
AIMS: Anderson-Fabry Disease (AFD), an X-linked disorder of sphingolipid metabolism, is a cause of idiopathic left ventricular hypertrophy but the mechanism of hypertrophy is poorly understood. Gadolinium enhanced cardiovascular magnetic resonance can detect focal myocardial fibrosis. We hypothesised that hyperenhancement would be present in AFD. METHODS AND RESULTS: Eighteen males (mean 43+/-14 years) and eight female heterozygotes (mean 48+/-12 years) with AFD underwent cine and late gadolinium cardiovascular magnetic resonance. Nine male (50%) had myocardial hyperenhancement ranging from 3.4% to 20.6% (mean 7.7+/-5.7%) of total myocardium; in males, percentage hyperenhancement related to LV mass index (r=0.78, P=0.0002) but not to ejection fraction or left ventricular volumes. Lesser hyperenhancement was also found in four (50%) heterozygous females (mean 4.6%). In 12 (92%) patients with abnormal gadolinium uptake, hyperenhancement occurred in the basal infero-lateral wall where, unlike myocardial infarction, it was not sub-endocardial. In two male patients with severe LVH (left ventricular hypertrophy) and systolic impairment there was additional hyperenhancement in other myocardial segments. CONCLUSIONS: These observations suggests that myocardial fibrosis occurs in AFD and may contribute to the hypertrophy and the natural history of the disease.
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Cites
American Heart Association Writing Group on Myocardial Segmentation and Registration for Cardiac Imaging:, Cerqueira, M. D., Weissman, N. J., Dilsizian, V., Jacobs, A. K., Kaul, S., Laskey, W. K., Pennell, D. J., Rumberger, J. A., Ryan, T., et al. (2002) · Circulation · doi:10.1161/hc0402.102975 · Cited by 6,990
Safety and Efficacy of Recombinant Human α-Galactosidase A Replacement Therapy in Fabry's Disease
Eng, C. M., Guffon, N., Wilcox, W. R., Germain, D. P., Lee, P., Waldek, S., Caplan, L., Linthorst, G. E., & Desnick, R. J. (2001) · New England Journal of Medicine · doi:10.1056/nejm200107053450102 · Cited by 1,559
Images in Cardiovascular Medicine
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An Improved MR Imaging Technique for the Visualization of Myocardial Infarction
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McCrohon, J. A., Moon, J. C. C., Prasad, S. K., McKenna, W. J., Lorenz, C. H., Coats, A. J. S., & Pennell, D. J. (2003) · Circulation · doi:10.1161/01.cir.0000078641.19365.4c · Cited by 1,154
Self-citationMoon, J. C. C., McKenna, W. J., McCrohon, J. A., Elliott, P. M., Smith, G. C., & Pennell, D. J. (2003) · Journal of the American College of Cardiology · doi:10.1016/s0735-1097(03)00189-x · Cited by 766
Self-citationKim, R. J., Chen, E.-L., Lima, J. A. C., & Judd, R. M. (1996) · Circulation · doi:10.1161/01.cir.94.12.3318 · Cited by 662
Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy
Choudhury, L., Mahrholdt, H., Wagner, A., Choi, K. M., Elliott, M. D., Klocke, F. J., Bonow, R. O., Judd, R. M., & Kim, R. J. (2002) · Journal of the American College of Cardiology · doi:10.1016/s0735-1097(02)02602-5 · Cited by 627
α-Galactosidase A deficiency. Fabry disease
Desnick, R. J. (2001) · Cited by 605
Cited by
2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy
Authors/Task Force members, Elliott, P. M., Anastasakis, A., Borger, M. A., Borggrefe, M., Cecchi, F., Charron, P., Hagege, A. A., Lafont, A., Limongelli, G., et al. (2014) · European Heart Journal · doi:10.1093/eurheartj/ehu284 · Cited by 4,332
Self-citation2023 ESC guidelines for the management of cardiomyopathies
Arbelo, E., Protonotarios, A., Gimeno, J. R., Arbustini, E., Barriales-Villa, R., Basso, C., Bezzina, C. R., Biagini, E., Blom, N. A., de Boer, R. A., et al. (2023) · European Heart Journal · doi:10.1093/eurheartj/ehad194 · Cited by 2,844
Self-citationGersh, B. J., Maron, B. J., Bonow, R. O., Dearani, J. A., Fifer, M. A., Link, M. S., Naidu, S. S., Nishimura, R. A., Ommen, S. R., Rakowski, H., et al. (2011) · Circulation · doi:10.1161/cir.0b013e318223e2bd · Cited by 2,104
2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy
Gersh, B. J., Maron, B. J., Bonow, R. O., Dearani, J. A., Fifer, M. A., Link, M. S., Naidu, S. S., Nishimura, R. A., Ommen, S. R., Rakowski, H., et al. (2011) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2011.06.011 · Cited by 1,276
Delayed enhancement cardiovascular magnetic resonance assessment of non-ischaemic cardiomyopathies
Mahrholdt, H., Wagner, A., Judd, R. M., Sechtem, U., & Kim, R. J. (2005) · European Heart Journal · doi:10.1093/eurheartj/ehi258 · Cited by 914
Metadata
- Authors
- Moon, J.
- Venue
- European Heart Journal
- Publisher
- Oxford University Press (OUP)
- Published
- 2003-12
- Volume
- 24
- Issue
- 23
- Pages
- 2151-2155
- ISSN
- 0195-668X (ISSN-L)
- OpenAlex
- W2158457377
- Cited by
- 466
- References
- 14
- FWCI
- 7.42
- Type
- journal article
- Language
- en
- Open access
- closed
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