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Hypercontractile phenotype in hypertrophic cardiomyopathy indicates unfavorable hemodynamics, coronary flow and
Karina Wierzbowska-Drabik1, Eszter Dalma Pálinkás2, Maria Grazia D'Alfonso3
1Department of Internal Diseases and Clinical Pharmacology, Medical University of Lodz, ul. Kniaziewicza 1/5, 91-347 Lodz, Poland.
Insights
The left ventricular hypercontractile phenotype (HP) in hypertrophic cardiomyopathy (HCM) is linked to poorer cardiac function and reduced long-term survival in patients with preserved ejection fraction.
Area of Science:
- Cardiology
- Cardiovascular Physiology
- Echocardiography
Background:
- Hypertrophic cardiomyopathy (HCM) is a complex genetic heart disease.
- The clinical and prognostic significance of the left ventricular (LV) hypercontractile phenotype (HP) in HCM remains unclear.
- Preserved ejection fraction (EF) is common in HCM patients, but outcomes can vary.
Purpose of the Study:
- To assess the clinical and prognostic significance of the LV hypercontractile phenotype (HP) in hypertrophic cardiomyopathy (HCM).
- To investigate the hemodynamic profile and long-term survival associated with HP in HCM patients with preserved EF.
Main Methods:
- 1533 HCM patients with EF ≥ 50% underwent transthoracic echocardiography (TTE).
- LV force was calculated (systolic blood pressure + LVOTG/ESV) and HP defined as the highest quartile (> 7.32 mmHg/mL).
- Survival analysis was performed on 1200 patients with follow-up data.
Main Results:
- HP patients exhibited higher heart rate, lower stroke volume, larger left atrial volume index, and increased coronary flow velocity.
- LV force correlated moderately with LV EF and weakly with LV outflow tract gradient (LVOTG).
- A force > 7.32 mmHg/mL was independently associated with a 1.44-fold increased hazard of all-cause death (HR 1.44, 95% CI 1.00-2.07).
Conclusions:
- The LV hypercontractile phenotype (HP) in HCM is associated with unfavorable systemic, cardiac, and coronary hemodynamic profiles.
- HP in HCM patients with preserved EF is linked to significantly lower long-term survival.
- These findings highlight the prognostic importance of assessing LV force in HCM management.
Aims:
Our aim was to assess the clinical and prognostic significance of the left ventricular (LV) hypercontractile phenotype (HP) in hypertrophic cardiomyopathy (HCM), which until now remains unclear.
Methods And Results:
We enrolled 1533 HCM patients (age 51 ± 15 years, 965 males, 63%) with ejection fraction (EF) ≥ 50%, referred for rest transthoracic echocardiography (TTE) in 27 laboratories from 13 countries. Two-dimensional volumetric TTE assessment included LV outflow tract gradient (LVOTG), LV EF, and LV force (systolic blood pressure + LVOTG/ESV, mmHg/mL). HP was defined as the highest quartile of the force (> 7.32 mmHg/mL). Survival analysis was performed in a subset of 1200 patients with follow-up information. Compared with non-HP patients, HP showed higher heart rate (72 ± 14 vs. 67 ± 14 beats per minute, P < 0.001), lower stroke volume (50 ± 22 vs. 67 ± 22 mL, P < 0.001), larger left atrial volume index (44 ± 16 vs. 40 ± 16 mL/m2, P = 0.011) and higher coronary flow velocity in the mid-distal left anterior descending artery (n = 325, 41 ± 12 vs. 37 ± 14 cm/s, P = 0.021). Force was moderately related to LV EF (r = 0.48, P < 0.001) and weakly to LVOTG (r = 0.36, P < 0.001). During a median follow-up of 87 months (interquartile range 45-143 months), 131 all-cause deaths occurred. At multivariable Cox analysis, a force > 7.32 mmHg (the fourth quartile for analyzed patients) was associated with a hazard ratio of 1.44 (95% Confidence intervals 1.00-2.07) for all-cause death, independently of LVOTG and LV EF.
Conclusion:
HP in HCM is associated with a disadvantageous systemic, cardiac and coronary hemodynamic profile as well as lower survival in the long-term.
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