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Updated: Oct 4, 2025

Echocardiographic Assessment Using Subxiphoid-Only Examination for Hypotensive Patients
Published on: April 18, 2025
Hypotension in hereditary cardiomyopathy
Johny Al-Khoury1, Danielle Jacques1, Ghassan Bkaily2
1Department of Immunology and Cell Biology, Faculty of Medicine, University of Sherbrooke, Sherbrooke, Quebec, J1H 5N4, Canada.
Insights
Hypotension, or low blood pressure, is linked to hereditary cardiomyopathy (HCM). Blocking the NHE1 protein partially reversed this hypotension, suggesting it as a potential biomarker for HCM progression to heart failure.
Area of Science:
- Cardiology
- Genetics
- Physiology
Background:
- Hypertension is a known risk factor for heart failure (HF).
- The role of hypotension in hereditary cardiomyopathy (HCM) onset and progression to HF remains unclear.
- HCM can lead to heart failure and premature death.
Purpose of the Study:
- To investigate blood pressure changes during different stages of HCM.
- To determine if hypotension contributes to HCM progression.
- To explore the role of the sodium-hydrogen exchanger 1 (NHE1) in HCM-associated hypotension.
Main Methods:
- Utilized the UM-X7.1 hamster model of HCM (HCMH).
- Monitored mean arterial pressure (MAP) across HCM phases: asymptomatic, necrosis, hypertrophy, and heart failure.
- Administered the NHE1 inhibitor EMD 87580 (rimeporide) to assess its effect on MAP in HCMH and normal hamsters (NH).
Main Results:
- No significant MAP change in asymptomatic HCMH compared to NH.
- Progressive MAP decrease observed in HCMH, reaching lowest levels during heart failure.
- MAP reduction during necrosis phase correlated with increased NHE1 levels.
- EMD 87580 treatment reversed hypotension in HCMH but did not affect NH MAP.
- NHE1 inhibition partially mitigated hypotension, indicating other contributing mechanisms.
Conclusions:
- HCM development is associated with hypotension.
- Decreased blood pressure may serve as a biomarker for HCM progression to heart failure.
- NHE1 plays a role in HCM-related hypotension, but other pathways are also involved.
Abstract:
It is well accepted that hypertension may lead to the development of heart failure (HF). However, little is known about the development of hypotension that may contribute to the onset of hereditary cardiomyopathy (HCM), thus promoting heart failure and early death. The purpose of this study is to verify whether a decrease in blood pressure takes place during different phases of HCM (asymptomatic, necrosis, hypertrophy, and heart failure). Using the well-known animal model, the UM-X7.1 hamster strain of HCM (HCMH), our results showed the absence of a change in mean arterial pressure (MAP) during the asymptomatic phase preceding the development of necrosis in HCMHs when compared to age-matched normal hamster (NH). However, there was a progressive decrease in MAP that reached its lowest level during the heart failure phase. The MAP during the development of the necrosis phase of HCM was accompanied by a significant increase in the level of the sodium-hydrogen exchanger, NHE1. Treatments with the potent NHE1 inhibitor, EMD 87580 (rimeporide), did not affect MAP of NH. However, treatments with EMD 87580 during the three phases of the development of HCM significantly reversed the hypotension associated with HCM.Our results showed that the development of HCM is associated with hypotension. These results suggest that a decrease in blood pressure could be a biomarker signal for HCM leading to HF and early death. Since the blockade of NHE1 significantly but partially prevented the reduction in MAP, this suggests that other mechanisms can contribute to the development of hypotension in HCM.
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