Hypersynchrony in sarcomeric hypertrophic cardiomyopathy: description and mechanistic approach using multimodal
Patricia Réant1,2,3,4,5, Guillaume Bonnet1,2,3,4,5, Frédérique Dubé1,2,6
1Cardiology Department, Bordeaux University Hospital, Bordeaux, France.
Insights
Hypertrophic cardiomyopathy (HCM) alters left ventricular (LV) contraction, with many patients losing the normal apex-to-base delay. This hypersynchrony is linked to distinct electrical activation patterns and smaller LV dimensions, offering insights into HCM mechanics.
Area of Science:
- Cardiology
- Cardiac Electrophysiology
- Cardiovascular Imaging
Background:
- Hypertrophic cardiomyopathy (HCM) is characterized by abnormal heart muscle thickening.
- Little is known about the interplay between mechanical contraction and electrical activation in HCM.
- Understanding these sequences may help predict response to pacing therapies in obstructive HCM.
Purpose of the Study:
- To compare left ventricular (LV) mechanical and electrical activation sequences in HCM patients versus healthy controls.
- To investigate potential differences between obstructive and non-obstructive HCM.
- To explore associations between contraction patterns and cardiac imaging parameters.
Main Methods:
- Prospective study of 40 HCM patients (20 obstructive, 20 non-obstructive) and 20 controls.
- Assessed LV mechanical activation using echocardiography and cardiac MRI.
- Evaluated electrical activation with 3D electrocardiographic mapping (ECM).
Main Results:
- Healthy controls exhibited a physiological apex-to-base delay (ABD) in contraction.
- 18 HCM patients (45%) showed a loss of ABD (hypersynchrony), more than controls (5%).
- Hypersynchronous HCM patients had smaller LV volumes, lower native T1 values, and a distinct ECM pattern.
Conclusions:
- LV contraction sequence is altered in HCM, with loss of physiological ABD observed in a significant subset.
- This altered mechanical sequence correlates with specific electrical activation patterns and smaller LV dimensions.
- Further research is needed to clarify if these patterns reflect an electrical substrate or cardiac geometry.
Background:
Little is known about left ventricular (LV) sequences of contraction and electrical activation in hypertrophic cardiomyopathy (HCM). A better understanding of the underlying relation between mechanical and electrical activation may allow the identification of predictive response criteria to right ventricular DDD pacing in obstructive patients.
Objective:
To describe LV mechanical and electrical activation sequences in HCM patients compared to controls.
Materials And Methods:
We prospectively studied, in 40 HCM patients (20 obstructive and 20 non-obstructive) and 20 healthy controls: (1) mechanical activation using echocardiography at rest and cardiac magnetic resonance imaging, (2) electrical activation using 3-dimensional electrocardiographic mapping (ECM).
Results:
In echocardiography, healthy controls had a physiological apex-to-base delay (ABD) during contraction (23.8 ± 16.2 ms). Among the 40 HCM patients, 18 HCM patients presented a loss of this ABD (<10 ms, defining hypersynchrony) more frequently than controls (45% vs. 5%, p = 0.017). These patients had a lower LV end-diastolic volume (71.4 ± 9.7 ml/m2 vs. 82.4 ± 14.8 ml/m2, p = 0.01), lower native T1 values (988 ± 32 ms vs. 1,028 ± 39 ms, p = 0.001) and tended to have lower LV mass (80.7 ± 23.7 g/m2 vs. 94.5 ± 25.3 g/m2, p = 0.08) compared with HCM patients that had a physiological contraction sequence. There was no significant relation between ABD and LV outflow tract obstruction. While HCM patients with a physiological contraction sequence presented an ECM close to those encountered in controls, patients with a loss of ABD presented a particular pattern of ECM with the first potential more frequently occurring in the postero-basal region.
Conclusion:
The LV contraction sequence can be modified in HCM patients, with a loss of the physiological ABD, and is associated with smaller LV dimensions and a particular pattern of ECM. Further research is needed to determine whether this pattern is related to an electrical substrate or is the consequence of the hypertrophied heart's specific geometry.
Clinical Trial Registration:
ClinicalTrial.gov: NCT02559726.
Related Concept Videos
Heart Failure II: Pathophysiology
Cardiomyopathy I: Introduction and Classification
Cardiomyopathy II: Dilated Cardiomyopathy
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Cardiomyopathy IV: Restrictive Cardiomyopathy
Cardiomyopathy V: Interprofessional Care


