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Cardiomyopathy I: Introduction and Classification

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...

Cardiomyopathy III: Hypertrophic Cardiomyopathy

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...

Cardiomyopathy IV: Restrictive Cardiomyopathy

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...

Cardiomyopathy II: Dilated Cardiomyopathy

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,...

Myocarditis I: Introduction

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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...

Myocarditis II: Clinical Features and Diagnostic Tests

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Related Experiment Video

Updated: May 25, 2026

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

Microfibrillar cardiomyopathy: a rare case.

Narender Kumar¹, Ruma Ray

¹Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

Indian Journal of Pathology & Microbiology

|January 12, 2012

Summary

Microfibrillar cardiomyopathy, a rare cause of restrictive cardiomyopathy, presents uniquely. Pathologists should consider this diagnosis in amyloidosis cases negative for Congo red staining.

Area of Science:

Cardiology
Pathology
Electron Microscopy

Background:

Restrictive cardiomyopathy (RCM) is a group of diseases that restrict the expansion of the ventricles.
Microfibrillar cardiomyopathy is an exceedingly rare cause of RCM.

Observation:

A male patient presented with shortness of breath and pedal edema, clinically diagnosed with RCM.
Endomyocardial biopsy revealed subendocardial and interstitial hyaline eosinophilic material.
This material did not stain with Congo red but electron microscopy showed it was composed of microfibrils.

Findings:

The deposited material in this RCM case was identified as microfibrils.
The etiology of this microfibrillar deposition remains unknown.
The case highlights a distinct subtype of RCM characterized by microfibrillar accumulation.

Related Experiment Videos

Last Updated: May 25, 2026

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

Implications:

Microfibrillar cardiomyopathy should be considered in the differential diagnosis of RCM, especially when amyloidosis is suspected but Congo red staining is negative.
This finding expands the understanding of cardiomyopathies and their underlying molecular pathologies.
Further research is needed to elucidate the etiology and pathogenesis of microfibrillar cardiomyopathy.