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.
Abstract

Related Concept Videos

Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
45
Heart Failure II: Pathophysiology01:29

Heart Failure II: Pathophysiology

Systolic Heart Failure and Compensatory MechanismsSystolic heart failure (also termed HFrEF, Heart Failure with Reduced Ejection Fraction) is the most prevalent type of heart filure. It results in a decreased volume of blood being pumped from the ventricle. The aortic arch and carotid sinuses have baroreceptors that detect reduced blood pressure, triggering the sympathetic nervous system (SNS) to release epinephrine and norepinephrine. Initially, this response aims to boost heart rate and...
37
Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
32
Imbalances in Cardiac Output01:26

Imbalances in Cardiac Output

The heart's primary function is to pump blood throughout the body, maintaining a balance between blood sent out (cardiac output) and blood returning (venous return). If this balance is disrupted, it can result in congestive heart failure (CHF), a severe condition where the heart becomes an inefficient pump, leading to inadequate blood circulation.
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send...
1.5K
Pathophysiology of Heart Failure01:17

Pathophysiology of Heart Failure

Heart failure (HF) is a progressive syndrome involving ventricles that leads to inadequate cardiac output. It can be classified based on location and output or ejection fraction. Ejection fraction (EF) is an essential measurement in the diagnosis and surveillance of HF. Reduced EF corresponds to systolic heart failure (HFrEF). However, HF with preserved ejection fraction (HFpEF) is becoming increasingly prevalent. Also known as diastolic HF, this form of HF is related to aging. The...
1.8K
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
21