Back to Search
Start Over
Late gadolinium enhanced cardiovascular magnetic resonance of lamin A/C gene mutation related dilated cardiomyopathy.
- Source :
-
Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance [J Cardiovasc Magn Reson] 2011 Jun 20; Vol. 13, pp. 30. Date of Electronic Publication: 2011 Jun 20. - Publication Year :
- 2011
-
Abstract
- Background: The purpose of this study was to identify early features of lamin A/C gene mutation related dilated cardiomyopathy (DCM) with cardiovascular magnetic resonance (CMR). We characterise myocardial and functional findings in carriers of lamin A/C mutation to facilitate the recognition of these patients using this method. We also investigated the connection between myocardial fibrosis and conduction abnormalities.<br />Methods: Seventeen lamin A/C mutation carriers underwent CMR. Late gadolinium enhancement (LGE) and cine images were performed to evaluate myocardial fibrosis, regional wall motion, longitudinal myocardial function, global function and volumetry of both ventricles. The location, pattern and extent of enhancement in the left ventricle (LV) myocardium were visually estimated.<br />Results: Patients had LV myocardial fibrosis in 88% of cases. Segmental wall motion abnormalities correlated strongly with the degree of enhancement. Myocardial enhancement was associated with conduction abnormalities. Sixty-nine percent of our asymptomatic or mildly symptomatic patients showed mild ventricular dilatation, systolic failure or both in global ventricular analysis. Decreased longitudinal systolic LV function was observed in 53% of patients.<br />Conclusions: Cardiac conduction abnormalities, mildly dilated LV and depressed systolic dysfunction are common in DCM caused by a lamin A/C gene mutation. However, other cardiac diseases may produce similar symptoms. CMR is an accurate tool to determine the typical cardiac involvement in lamin A/C cardiomyopathy and may help to initiate early treatment in this malignant familiar form of DCM.
- Subjects :
- Adolescent
Adult
Cardiomyopathy, Dilated physiopathology
Chi-Square Distribution
Electrocardiography
Female
Fibrosis
Finland
Genetic Predisposition to Disease
Humans
Hypertrophy, Left Ventricular diagnosis
Hypertrophy, Left Ventricular genetics
Male
Middle Aged
Myocardium pathology
Phenotype
Predictive Value of Tests
Severity of Illness Index
Ventricular Dysfunction, Left diagnosis
Ventricular Dysfunction, Left genetics
Ventricular Function, Left
Ventricular Function, Right
Cardiomyopathy, Dilated diagnosis
Cardiomyopathy, Dilated genetics
Contrast Media
Lamin Type A genetics
Magnetic Resonance Imaging, Cine
Meglumine
Mutation
Organometallic Compounds
Subjects
Details
- Language :
- English
- ISSN :
- 1532-429X
- Volume :
- 13
- Database :
- MEDLINE
- Journal :
- Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
- Publication Type :
- Academic Journal
- Accession number :
- 21689390
- Full Text :
- https://doi.org/10.1186/1532-429X-13-30