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Contribution of mitral valve leaflet length and septal wall thickness to outflow tract obstruction in patients with hypertrophic cardiomyopathy

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Abstract

We sought to examine whether elongation of the mitral valve leaflets in patients with hypertrophic cardiomyopathy (HCM) is synergistic to septal wall thickness (SWT) in the development of left ventricular outflow tract obstruction (LVOTO). HCM is a common genetic cardiac disease characterized by asymmetric septal hypertrophy and predisposition towards LVOTO. It has been reported that elongation of the mitral valve leaflets may be a primary phenotypic feature and contribute to LVOTO. However, the relative contribution of this finding versus SWT has not been studied. 152 patients (76 with HCM and 76 non-diseased age, race and BSA-matched controls) and 18 young, healthy volunteers were studied. SWT and the anterior mitral valve leaflet length (AMVLL) were measured using cine MRI. The combined contribution of these variables (SWT × AMVLL) was described as the Septal Anterior Leaflet Product (SALP). Peak LVOT pressure gradient was determined by Doppler interrogation and defined as “obstructive” if ≥ 30 mmHg. Patients with HCM were confirmed to have increased AMVLL compared with controls and volunteers (p < 0.01). Among HCM patients, both SWT and SALP were significantly higher in patients with LVOTO (N = 17) versus without. SALP showed modest improvement in predictive accuracy for LVOTO (AUC = 0.81) among the HCM population versus SWT alone (AUC = 0.77). However, in isolated patients this variable identified patients with LVOTO despite modest SWT. Elongation of the AMVLL is a primary phenotypic feature of HCM. While incremental contributions to LVOTO appear modest at a population level, specific patients may have dominant contribution to LVOTO. The combined marker of SALP allows for maintained identification of such patients despite modest increases in SWT.

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Abbreviations

HCM:

Hypertrophic Cardiomyopathy

LVOTO:

Left Ventricular Outflow Tract Obstruction

SWT:

Septal Wall Thickness

AMVLL:

Anterior Mitral Valve Leaflet Length

PMVLL:

Posterior Mitral Valve Leaflet Length

CMRI:

Cardiac Magnetic Resonance Imaging

BSA:

Body Surface Area

LVEF:

Left Ventricular Ejection Fraction

LGE:

Late Gadolinium Enhancement

LV:

Left Ventricle

EDV:

End Diastolic Volume

ESV:

End Systolic Volume

SALP:

Septal-wall Anterior Leaflet Product

HE:

Hyper-enhancement

ROC:

Receiver Operating Characteristic

NYHA:

New York Heart Association

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Acknowledgements

The authors gratefully acknowledge the staff of the CMCR and Stephenson Cardiac Imaging Centre for their contributions and support. In particular we wish to thank Dr Guanmin Chen for his assistance in data analysis and Linda Marziali for her administrative support. The study was funded in part by the Ontario Research Fund (ORF) Imaging in Cardiovascular Therapeutics grant, the Canada Foundation for Innovation (CFI) and the Program for Experimental Medicine (POEM) at Western University.

Funding

The study was funded in part by the Ontario Research Fund (ORF) Imaging in Cardiovascular Therapeutics grant, the Calgary Health Trust. Dr White is supported by an Early Investigator Award from the Heart and Stroke Foundation of Canada, receives research grant contributions from Circle Cardiovascular, Inc and is a shareholder of Cohesic Inc.

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Authors and Affiliations

  1. Department of Medicine, London Health Sciences Centre, London, ON, Canada

    Kareem Morant, David McCarty & James A. White

  2. Stephenson Cardiac Imaging Centre at Libin Cardiovascular Institute of Alberta, University of Calgary, Suite 0700 – Special Services Building, Foothills Medical Centre, 1403 – 29th Street NW, Calgary, AB, T2N 2T9, Canada

    Yoko Mikami, Louis Kolman, Bobby Heydari, Carmen Lydell, Andrew Howarth & James A. White

  3. Imaging Research Laboratory- Robarts Research Institute, Western University, London, ON, Canada

    Immaculate Nevis, John Stirrat, David Scholl & Martin Rajchl

  4. Department of Cardiac Sciences at Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, AB, Canada

    Yoko Mikami, Peter Giannoccaro, Louis Kolman, Bobby Heydari, Carmen Lydell, Andrew Howarth, Andrew Grant & James A. White

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  1. Kareem Morant
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  2. Yoko Mikami
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  3. Immaculate Nevis
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  14. James A. White
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Correspondence to James A. White.

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Morant, K., Mikami, Y., Nevis, I. et al. Contribution of mitral valve leaflet length and septal wall thickness to outflow tract obstruction in patients with hypertrophic cardiomyopathy. Int J Cardiovasc Imaging 33, 1201–1211 (2017). https://doi.org/10.1007/s10554-017-1103-5

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  • DOI: https://doi.org/10.1007/s10554-017-1103-5

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