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Published on: August 8, 2022
Comparison of Nonclassic and Classic Phenotype of Hypertrophic Cardiomyopathy Focused on Prognostic Cardiac Magnetic
Magdalena Stachera1, Paweł Przybyło2, Katarzyna Sznajder1
1Clinical Department of Diagnostic Imaging, Institute of Medical Sciences, University of Opole, 45-052 Opole, Poland.
Insights
Nonclassic hypertrophic cardiomyopathy (HCM) patients show distinct cardiac magnetic resonance (CMR) findings compared to classic HCM. These differences in advanced HCM markers may aid future risk stratification and understanding of disease progression.
Area of Science:
- Cardiology
- Medical Imaging
- Biomedical Engineering
Background:
- Nonclassic phenotypes (NCP) represent advanced hypertrophic cardiomyopathy (HCM) and are challenging to diagnose and manage.
- NCP are often underrepresented in research, necessitating further investigation into their unique characteristics.
Purpose of the Study:
- To compare cardiac magnetic resonance (CMR) parameters between nonclassic phenotypes (NCP) and classic phenotypes (CP) of HCM.
- To identify established and potential prognostic indicators in NCP versus CP using standard and non-standard CMR variables.
Main Methods:
- Retrospective analysis of CMR studies from 88 patients diagnosed with HCM between 2011 and 2019.
- Postprocessing of CMR data to obtain standard and non-standard parameters, focusing on left ventricular and atrial function, and tissue characterization.
Main Results:
- NCP exhibited higher late gadolinium enhancement extent (%LGE) and left ventricular mass index (LVMI), lower left atrium emptying fraction (LAEF), greater minimal left atrial volume (LAV min), and reduced myocardial contraction fraction (MCF) and left ventricular global function index (LVGFI) compared to CP.
- No left ventricular outflow tract obstruction (LVOTO) was found in NCP. Significant differences in left ventricular outflow tract (LVOT) and aortic valve (Ao) dimensions were observed.
- HCM risk score and left ventricular maximal thickness (LVMT) were similar between NCP and CP groups.
Conclusions:
- Significant differences in non-standard CMR parameters distinguish NCP from CP in hypertrophic cardiomyopathy.
- These findings may inform future research on disease staging and risk stratification in advanced HCM phenotypes.
Abstract:
Patients with nonclassic phenotypes (NCP)—more advanced stages of hypertrophic cardiomyopathy (HCM)—constitute an intriguing and heterogeneous group that is difficult to diagnose, risk-stratify, and treat, and often neglected in research projects. We aimed to compare cardiac magnetic resonance (CMR) parameters in NCP versus classic phenotypes (CP) of HCM with special emphasis given to the parameters of established and potential prognostic importance, including numerous variables not used in everyday clinical practice. The CMR studies of 88 patients performed from 2011 to 2019 were postprocessed according to the study protocol to obtain standard and non-standard parameters. In NCP, the late gadolinium enhancement extent expressed as percent of left ventricular mass (%LGE) and left ventricular mass index (LVMI) were higher, left atrium emptying fraction (LAEF) was lower, minimal left atrial volume (LAV min) was greater, and myocardial contraction fraction (MCF) and left ventricular global function index (LVGFI) were lower than in CP (p < 0.001 for all). In contrast, HCM risk score and left ventricular maximal thickness (LVMT) were similar in NCP and CP patients. No left ventricular outflow tract obstruction (LVOTO) was observed in the NCP group. Left ventricular outflow tract diameter (LVOT), aortic valve diameter (Ao), and LVOT/Ao ratio were significantly higher and anterior mitral leaflet (AML)/LVOT ratio was lower in the NCP compared to the CP group. In conclusion, significant differences in nonstandard CMR parameters were noted between the nonclassic and classic HCM phenotypes that may contribute to future studies on disease stages and risk stratification in HCM.
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