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The systolic paradox in hypertrophic cardiomyopathy
Trine F Haland1,2, Nina E Hasselberg1,2, Vibeke Marie Almaas1,2
1Department of Cardiology, Institute for Surgical Research and Center for Cardiological Innovation, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Insights
Hypertrophic cardiomyopathy (HCM) patients with left ventricular hypertrophy (LVH+) showed smaller cardiac volumes and preserved ejection fraction (EF) despite reduced global longitudinal strain (GLS). Patients without LVH (LVH-) had reduced volumes and subtle GLS changes, indicating a continuum of disease.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- Left ventricular hypertrophy (LVH) is a common feature of HCM.
- The relationship between cardiac volumes, systolic function, and LVH in HCM requires further elucidation.
Purpose of the Study:
- To investigate cardiac volumes and systolic function in HCM patients with (HCM LVH+) and without (HCM LVH-) left ventricular hypertrophy.
- To compare these parameters with healthy individuals.
- To explore the correlation between cardiac structure and function.
Main Methods:
- Echocardiography was used to assess End-Diastolic Volume Index (EDVI), End-Systolic Volume Index (ESVI), and ejection fraction (EF).
- Speckle tracking echocardiography measured Left Ventricular (LV) global longitudinal strain (GLS).
- 180 HCM LVH+, 100 HCM LVH-, and 80 healthy individuals were included.
Main Results:
- HCM LVH+ and HCM LVH- patients had significantly smaller EDVI and ESVI compared to healthy individuals.
- Ejection fraction (EF) was similar across all groups, despite significantly worse GLS in HCM LVH+ and HCM LVH- patients compared to controls.
- Maximal wall thickness (MWT) in HCM LVH+ correlated with worse GLS but not EF.
Conclusions:
- Smaller cardiac volumes in HCM LVH+ may preserve EF despite impaired GLS, which is linked to MWT.
- HCM LVH- patients exhibit reduced volumes and subtle GLS changes, suggesting a continuum of volumetric and systolic alterations preceding significant hypertrophy.
Objective:
We explored cardiac volumes and the effects on systolic function in hypertrophic cardiomyopathy (HCM) patients with left ventricular hypertrophy (HCM LVH+) and genotype-positive patients without left ventricular hypertrophy (HCM LVH-).
Methods:
We included 180 HCM LVH+, 100 HCM LVH- patients and 80 healthy individuals. End-Diastolic Volume Index (EDVI), End-Systolic Volume Index (ESVI) and ejection fraction (EF) were assessed by echocardiography. Left ventricular (LV) global longitudinal strain (GLS) was measured by speckle tracking echocardiography.
Results:
EDVI and ESVI were significantly smaller in HCM LVH+ compared with HCM LVH- patients (41±14 mL/m2 vs 49±13 mL/m2 and 16±7 mL/m2 vs 19±6 mL/m2, respectively, both p<0.001) and in healthy individuals (41±14 mL/m2 vs 57±14 mL/m2 and 16±7 mL/m2 vs 23±9 mL/m2, respectively, both p<0.001). HCM LVH- patients had significantly lower EDVI and ESVI compared with healthy individuals (49±13 mL/m2 vs 57±14 mL/m2 and 19±6 mL/m2 vs 23±9 mL/m2, both p<0.001). EF was similar (61%±7% vs 60%±8% vs 61%±6%, p=0.43) in the HCM LVH+, HCM LVH- and healthy individuals, despite significantly worse GLS in the HCM LVH+ (-16.4%±3.7% vs -21.3%±2.4% vs -22.3%±3.7%, p<0.001). GLS was worse in the HCM LVH- compared with healthy individuals in pairwise comparison (p=0.001). Decrease in ESVI was closely related to EF in HCM LVH+ and HCM LVH- (R=0.45, p<0.001 and R=0.43, p<0.001) as expected, but there was no relationship with GLS (R=0.02, p=0.77 and R=0.11, p=0.31). Increased maximal wall thickness (MWT) correlated significantly with worse GLS (R=0.58, p<0.001), but not with EF (R=0.018, p=0.30) in the HCM LVH+ patients.
Conclusion:
HCM LVH+ had smaller cardiac volumes that could explain the preserved EF, despite worse GLS that was closely related to MWT. HCM LVH- had reduced cardiac volumes and subtle changes in GLS compared with healthy individuals, indicating a continuum of both volumetric and systolic changes present before increased MWT.
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