Inflammation across the spectrum of hypertrophic cardiac phenotypes

Area of Science:

• Cardiovascular Medicine
• Genetics
• Immunology

Background:

• Hypertrophic cardiomyopathy (HCM) is a diverse group of diseases characterized by left ventricular hypertrophy.
• Phenocopies, including Fabry disease (FD) and cardiac amyloidosis (CA), share HCM's phenotype but have different underlying causes.
• The role of inflammation in cardiomyopathies is increasingly recognized as a significant factor in disease progression.

Purpose of the Study:

• To review the current understanding of inflammation's role in HCM.
• To examine the prevalence, clinical significance, and therapeutic potential of inflammation in HCM and its phenocopies (FD and CA).
• To explore how inflammation contributes to cardiomyocyte dysfunction and disease severity.

Main Methods:

• Literature review synthesizing current evidence on inflammation in HCM, FD, and CA.
• Analysis of studies investigating the pathophysiological mechanisms linking inflammation to cardiac hypertrophy and dysfunction.
• Evaluation of research on the clinical impact and therapeutic targets related to inflammation in these cardiomyopathies.

Main Results:

• Inflammation is implicated in the pathogenesis of HCM and its phenocopies, contributing to cardiomyocyte hypertrophy, extracellular matrix deposition, and microvascular dysfunction.
• Systemic inflammation may influence disease severity, clinical outcomes, and the progression to heart failure in patients with these conditions.
• Evidence suggests inflammation is a common pathway across different forms of hypertrophic cardiomyopathy.

Conclusions:

• Inflammation is a key pathophysiological mediator in hypertrophic cardiomyopathy and its phenocopies.
• Targeting inflammatory pathways may offer novel therapeutic strategies for managing HCM, FD, and CA.
• Further research is needed to fully elucidate the complex role of inflammation in these cardiac diseases.