2069 Assessment of genetic dilated cardiomyopathy in LMNA-mutation carriers by cardiac MRI
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BioMed Central
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Meeting abstract
2069 Assessment of genetic dilated cardiomyopathy in LMNA-mutation carriers by cardiac MRI Kristel M Antila*1, Juha R Koikkalainen2, Jyrki MP Lötjönen2, Tiina Heliö3, Sari M Kivistö4 and Kirsi Lauerma4 Address: 1Helsinki University, Helsinki, Finland, 2VTT Technical Research Centre of Finland, Tampere, Finland, 3Department of Cardiology, Helsinki University Central Hospital, Helsinki, Finland and 4Department of Radiology, Helsinki Medical Imiging Center, Helsinki, Finland * Corresponding author
from 11th Annual SCMR Scientific Sessions Los Angeles, CA, USA. 1–3 February 2008 Published: 22 October 2008 Journal of Cardiovascular Magnetic Resonance 2008, 10(Suppl 1):A338
doi:10.1186/1532-429X-10-S1-A338
Abstracts of the 11th Annual SCMR Scientific Sessions - 2008
Meeting abstracts – A single PDF containing all abstracts in this Supplement is available here. http://www.biomedcentral.com/content/pdf/1532-429X-10-S1-info.pdfThis abstract is available from: http://jcmr-online.com/content/10/S1/A338 © 2008 Antila et al; licensee BioMed Central Ltd.
Introduction
Results
DCM is characterized by enlargement and impaired contraction of left or both ventricles. With an estimated prevalence of 36/100 000 in adults in the United States, DCM is a significant cause of morbidity and mortality. One third to one half of the causes are familial and the most important risk gene for cardiomyopathy is lamin A/C gene. Thus, we wanted to look for other methods than conventional echocardiography, to recognize this type of serious cardiomyopathy early. We wanted to characterize more precisely the cardiac findings of the Finnish LMNA patients using magnetic resonance imiging (MRI)
Purpose
All carriers were in NYHA class I and had sinus rhythm. 6/ 12 carriers presented with first degree atrioventricular block. 5/12 LMNA-carriers were asymptomatic, palpitation being the most common symptom. The carriers had higher BSA corrected LVEDV (93 +/- 13 vs 80 +/- 20, p = 0.005), LVESV(36 +/- 7 vs 24 +/- 7, p = 0.003) and lower EF(61 +/- 6 vs 70 +/- 3, p = 0.0003). The carriers had decreased regional wall motion at basal inferior, anterior, and septal segments, and decreased thickening of apicoseptal segment. 7/12 carriers showed midwall late enhancement that represents myocardial fibrosis. Inferior segments were most commonly involved; fibrosis was also observed in anterior, septal and lateral walls.
To characterize the cardiac functional parameters and myocardial late enhancement pattern in LMNA-carriers.
Conclusion
Methods 12 LMNA-carriers (representing 9 families) who were heterozygotes for one lamin A/C mutation (Ser143Pro, T1085deletion, Ala132Pro, Arg190Tpr) that may cause dilated cardiomyopathy and 14 healthy controls were studied with a 1.5 T MR imager. The volumes, wall thickness and motion of both ventricles were assessed from cine images. The LMNA-carriers were evaluated with late enhancement imaging.
Due to the severity of the disease and the possibility of early interventi
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