- Meeting abstract
- Open Access
227 Early detection of systolic and diastolic dysfunction in asymptomatic hypertrophic cardiomyopathy mutation carriers using cardiac magnetic resonance imaging
© Germans et al; licensee BioMed Central Ltd. 2008
- Published: 22 October 2008
- Cardiac Magnetic Resonance
- Diastolic Dysfunction
- Hypertrophic Cardiomyopathy
- Cardiac Magnetic Resonance Imaging
- Left Ventricular Wall Thickness
Hypertrophic Cardiomyopathy (HCM) is characterized by asymmetric hypertrophy of the left ventricle (LV). While over 400 HCM mutations have been identified, increasing number of HCM mutation carriers without hypertrophy have been detected. No data on regional functional and structural abnormalities in HCM mutation carriers before the development of hypertrophy are yet available. With cardiac magnetic resonance imaging (CMR), both regional function and structure can accurately be evaluated.
28 HCM mutation carriers from 9 different families and 28 age- and gender matched controls, of whom 15 were genotype negative family members of HCM mutation carriers, underwent CMR. The imaging protocol included full coverage of the LV and left atrium with cine imaging. Subsequently, three short axis slices at basal, mid and apical level were obtained with myocardial tissue tagging (temporal resolution 14 ms, tag persistence >1000 ms). Finally, delayed contrast enhancement imaging was performed. From these datasets, global LV dimensions and left atrial volumes, end-diastolic wall thickness, septal-to-lateral-wall-thickness ratio (SL-ratio), wall thickening, peak systolic circumferential strain (peak SCS), peak systolic circumferential strain rate (peak SCSR), peak diastolic circumferential strain rate (peak DCSR) were determined.
Functional abnormalities, characterized by reduced systolic and diastolic strain and strain rate are present in HCM mutation carriers without manifest hypertrophy. In addition, these patients exhibit structural abnormalities including septal to lateral asymmetry in left ventricular wall thickness and the presence of crypts. These structural abnormalities and decreased regional peak DCSR may thus be recognized as important, early identification of asymptomatic HCM mutation carriers.
This article is published under license to BioMed Central Ltd.