Related Experiment Video
Updated: Jul 3, 2026
10:33
Assessment of Right Ventricular Structure and Function in Mouse Model of Pulmonary Artery Constriction by Transthoracic Echocardiography
Published on: February 3, 2014
27.7K
Systemic Vascular Resistance and Myocardial Work Analysis in Hypertrophic Cardiomyopathy and Transthyretin Cardiac
Cesare de Gregorio1, Giancarlo Trimarchi1, Denise Cristiana Faro2
1Department of Clinical and Experimental Medicine, University Hospital of Messina, 98125 Messina, Italy.
Journal of Clinical Medicine
|March 28, 2024
Summary
Systemic vascular resistance (SVR) and global myocardial work index (GWI) were impaired in many patients with hypertrophic cardiomyopathy (HCM) and transthyretin cardiac amyloidosis (ATTR), despite preserved ejection fraction.
Area of Science:
- Cardiology
- Cardiovascular Physiology
Background:
- Systemic vascular resistance (SVR) and global myocardial work index (GWI) are key indicators of cardiovascular function.
- Their impact in hypertrophic cardiomyopathy (HCM) and transthyretin cardiac amyloidosis (ATTR) with preserved ejection fraction warrants further investigation.
Purpose of the Study:
- To investigate the pathophysiological role of SVR and GWI in patients with HCM and ATTR presenting with heart failure and preserved ejection fraction (HFpEF).
- To assess the correlation between these hemodynamic parameters and clinical characteristics in these distinct hypertrophic phenotypes.
Main Methods:
- Sixty outpatients (30 HCM, 30 ATTR) with HFpEF were evaluated for SVR and GWI.
- Patients were classified into four functional groups based on established cut-off values for SVR (1440 dyne/s/cm⁻⁵) and GWI (1576 mm Hg%).
Main Results:
- 85% of patients exhibited high SVR, with 70% of HCM and 100% of ATTR patients affected.
- Impaired SVR and GWI were observed in 43% of HCM patients and 93% of ATTR patients.
- ATTR patients were older and in worse NYHA functional class compared to HCM patients.
Conclusions:
- A significant proportion of patients with HCM and ATTR, even with preserved LVEF, demonstrate impaired SVR and GWI.
- The proposed classification may enhance understanding of the pathophysiology and clinical presentation of these hypertrophic cardiomyopathies.
Keywords:
cardiac amyloidosisheart failure with preserved left ventricular ejection fractionhypertrophic cardiomyopathystrain echocardiographysystemic vascular resistanceventricular arterial couplingMore Related Videos
Related Concept Videos
01:29Pathophysiology of Cardiac Performance
Typical heart performance is influenced by heart rate, rhythm, myocardial contraction, and metabolism or blood flow. The cardiac muscle exhibits distinct electrophysiological features, including pacemaker activity and calcium channel control, which play a vital role in the heart's response to various drugs. The autonomic nervous system, comprising the sympathetic and parasympathetic branches, regulates heart rate. Sympathetic activation increases heart rate, while parasympathetic activation...
01:26Imbalances 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 blood...
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 blood...
01:29Heart 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...
01:29Cardiomyopathy 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...
01:29Cardiomyopathy IV: Restrictive Cardiomyopathy
Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
01:29Cardiomyopathy 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...