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Related Concept Videos

Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

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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...
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Mitral Regurgitation I: Introduction01:20

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Mitral regurgitation is characterized by the backward circulation of blood from the left ventricle to the left atrium during systole, a phase of the cardiac cycle when the heart contracts and pumps blood out of the chambers. This abnormal flow occurs primarily due to the dysfunction of the mitral valve or its supporting structures, which include the mitral leaflets, chordae tendineae, annulus, and papillary muscles.Etiology and Mechanisms:Primary Mitral Regurgitation: This type arises from...
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Heart Failure II: Pathophysiology01:29

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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...
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Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

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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...
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Mitral Stenosis I: Introduction01:22

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Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
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Right ventricular strain in Fabry disease: Prognostic implications.

Maria Chiara Meucci1, Rosa Lillo2, Federica Mango3

  • 1Department of Cardiovascular Science, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.

International Journal of Cardiology
|December 31, 2022
PubMed
Summary

Right ventricular free wall strain (RV-FWS) is not an independent predictor of cardiovascular events in Anderson-Fabry disease (FD). Prognosis in FD is primarily determined by left ventricular cardiomyopathy severity, not RV-FWS.

Keywords:
CardiomyopathyFabry diseaseRight ventricleSpeckle-tracking echocardiographyStrain

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Area of Science:

  • Cardiology
  • Genetics
  • Medical Imaging

Background:

  • Anderson-Fabry disease (FD) commonly involves left ventricular (LV) hypertrophy, but right ventricular (RV) dysfunction also occurs.
  • Previous studies have not established a clear link between conventional RV performance metrics and outcomes in FD patients.
  • The prognostic value of RV free wall strain (RV-FWS) in FD remains uncertain.

Purpose of the Study:

  • To investigate the association between RV free wall strain (RV-FWS) and cardiovascular event occurrence in a cohort of Anderson-Fabry disease (FD) patients.
  • To determine if RV-FWS can serve as an independent predictor of adverse cardiovascular outcomes in FD.

Main Methods:

  • Retrospective analysis of 56 FD patients.
  • Assessment of RV free wall strain (RV-FWS) using 2D speckle tracking analysis.
  • Defined study endpoint as cardiovascular mortality, severe heart failure, new atrial fibrillation, or major arrhythmias.

Main Results:

  • Reduced RV-FWS (<23%) was observed in 45% of patients.
  • During a median follow-up of 47 months, 29% of patients experienced a study endpoint.
  • While RV-FWS showed association on univariable analysis, it lost significance after adjusting for LV global longitudinal strain or indexed left atrial volume.

Conclusions:

  • RV free wall strain (RV-FWS) is not independently associated with cardiovascular event risk in Anderson-Fabry disease (FD).
  • The findings suggest that the prognosis in FD is predominantly influenced by the severity of left ventricular cardiomyopathy.