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Published on: June 14, 2016
Left ventricular anatomy in obstructive hypertrophic cardiomyopathy: beyond basal septal hypertrophy
Uxio Hermida1, David Stojanovski1, Betty Raman2
1School of Biomedical Engineering and Imaging Sciences, King's College London, 5th Floor Becket House, Lambeth Palace Road, London SE1 7EU, UK.
Insights
Obstructive hypertrophic cardiomyopathy (oHCM) involves specific left ventricular (LV) shapes, including lengthening and apical dilatation. Understanding this LV anatomy can help redefine management strategies for LVOT obstruction.
Area of Science:
- Cardiology
- Medical Imaging
- Biomedical Engineering
Background:
- Obstructive hypertrophic cardiomyopathy (oHCM) is defined by dynamic left ventricular outflow tract (LVOT) obstruction.
- The precise role of left ventricular (LV) shape in oHCM pathophysiology remains unclear.
- This study investigates the specific LV end-diastolic morphology associated with oHCM.
Purpose of the Study:
- To identify the characteristic LV end-diastolic morphology in patients with oHCM.
- To quantify LV shape variations using a statistical shape model.
- To explore the relationship between LV morphology, LVOT obstruction, and genotype.
Main Methods:
- Analysis of cardiovascular magnetic resonance images from 2398 HCM patients.
- Construction of 3D LV models and application of principal component analysis.
- Development of linear discriminant axes to quantify LV morphology (Z-scores).
Main Results:
- oHCM is characterized by basal septal hypertrophy, LV lengthening, apical dilatation, and LVOT inward remodeling.
- Distinct LV morphological differences were observed between obstructive cases at rest and during stress.
- Genotype-negative cases exhibited a trend towards more obstructive phenotypes.
Conclusions:
- The LV anatomy in oHCM involves a complex remodeling pattern beyond basal septal hypertrophy.
- Variations in LV morphology at rest versus stress suggest diverse mechanisms for LVOT obstruction.
- Quantifiable Z-scores for LV anatomy may aid in redefining oHCM management strategies.
Aims:
Obstructive hypertrophic cardiomyopathy (oHCM) is characterized by dynamic obstruction of the left ventricular (LV) outflow tract (LVOT). Although this may be mediated by interplay between the hypertrophied septal wall, systolic anterior motion of the mitral valve, and papillary muscle abnormalities, the mechanistic role of LV shape is still not fully understood. This study sought to identify the LV end-diastolic morphology underpinning oHCM.
Methods And Results:
Cardiovascular magnetic resonance images from 2398 HCM individuals were obtained as part of the NHLBI HCM Registry. Three-dimensional LV models were constructed and used, together with a principal component analysis, to build a statistical shape model capturing shape variations. A set of linear discriminant axes were built to define and quantify (Z-scores) the characteristic LV morphology associated with LVOT obstruction (LVOTO) under different physiological conditions and the relationship between LV phenotype and genotype. The LV remodelling pattern in oHCM consisted not only of basal septal hypertrophy but a combination with LV lengthening, apical dilatation, and LVOT inward remodelling. Salient differences were observed between obstructive cases at rest and stress. Genotype negative cases showed a tendency towards more obstructive phenotypes both at rest and stress.
Conclusions:
LV anatomy underpinning oHCM consists of basal septal hypertrophy, apical dilatation, LV lengthening, and LVOT inward remodelling. Differences between oHCM cases at rest and stress, as well as the relationship between LV phenotype and genotype, suggest different mechanisms for LVOTO. Proposed Z-scores render an opportunity of redefining management strategies based on the relationship between LV anatomy and LVOTO.
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