Myocardial fibrosis in congenital heart disease

Blanca Gordon1, Víctor González-Fernández1, Laura Dos-Subirà1

  • 1Integrated Adult Congenital Heart Disease Unit, Vall d'Hebron University Hospital-Santa Creu i Sant Pau University Hospital, Barcelona, Spain.

Frontiers in Pediatrics
|December 5, 2022
PubMed

Insights

Myocardial fibrosis, excessive collagen in the heart, impairs function in cardiovascular diseases. This review covers its mechanisms, consequences, and assessment in congenital heart disease.

Area of Science:

  • Cardiovascular Pathology
  • Cardiac Physiology

Background:

  • Myocardial fibrosis, characterized by excessive collagen deposition, is a key feature in various cardiovascular diseases, including congenital heart anomalies.
  • Interstitial fibrosis significantly contributes to myocardial dysfunction by disrupting cardiac architecture and altering interstitial properties.

Purpose of the Study:

  • To review the mechanisms and consequences of myocardial fibrosis in heart failure and arrhythmias.
  • To evaluate current imaging and biomarker strategies for assessing myocardial fibrosis.
  • To synthesize evidence on myocardial fibrosis in congenital heart diseases for research and treatment implications.

Main Methods:

  • Literature review of existing research on myocardial fibrosis.
  • Analysis of studies focusing on mechanisms, consequences, and assessment methods.
  • Synthesis of data related to myocardial fibrosis in congenital heart disease subsets.

Main Results:

  • Myocardial fibrosis is a significant contributor to myocardial dysfunction and adverse cardiac remodeling.
  • Current imaging techniques and biomarkers show promise but require further validation for fibrosis assessment.
  • Evidence highlights the presence and impact of myocardial fibrosis across diverse congenital heart disease types.

Conclusions:

  • Understanding myocardial fibrosis mechanisms and consequences is crucial for managing cardiovascular diseases.
  • Improved diagnostic tools are needed for accurate myocardial fibrosis detection and quantification.
  • Targeting myocardial fibrosis presents a potential therapeutic strategy for improving outcomes in congenital heart disease.

Related Concept Videos

Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

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...
29
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...
31
Myocarditis I: Introduction01:21

Myocarditis I: Introduction

Myocarditis is inflammation of the myocardium, which is the muscular layer of the heart.EtiologyMyocarditis has a diverse etiology, including a wide range of infectious and non-infectious causes:Infectious CausesViral: Common viruses include Coxsackie A and B, adenovirus, parvovirus B19, enteroviruses, and influenza A.Bacterial: Examples include infections caused by Streptococcus, Staphylococcus, and Mycoplasma species.Rickettsial: Infections like Rocky Mountain spotted fever can result in...
20
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
36
Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

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...
25
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy 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...
18