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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 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,...

Cardiomyopathy V: Interprofessional Care

Cardiomyopathy 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...

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 VI: Nursing Management

Cardiomyopathy VI: Nursing Management

Assessment: Nursing management of patients with cardiomyopathy begins with a thorough assessment of the patient's history, including a family history of cardiomyopathy or sudden cardiac death, personal history of heart disease, hypertension, diabetes, and any alcohol consumption or drug use.During the physical examination, assess vital signs, look for signs of heart failure (such as edema, jugular venous distention, and cyanosis), auscultate for abnormal heart sounds (like murmurs and gallops),...

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Updated: May 13, 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

Cardiomyopathies in children.

Young Mi Hong¹

¹Department of Pediatrics, Ewha Womans University School of Medicine, Seoul, Korea.

Korean Journal of Pediatrics

|March 14, 2013

Summary

Cardiomyopathy (CMP) is a cardiac muscle disease with dilated or hypertrophic types, often with genetic links. Genetic testing is crucial for diagnosing CMP, guiding treatment, and identifying new therapeutic targets.

Area of Science:

Cardiology
Genetics
Pediatrics

Background:

Cardiomyopathy (CMP) is a heterogeneous cardiac muscle disease with primary and secondary forms.
Primary CMP lacks extrinsic causes and can be influenced by genetics and ethnicity.
Existing classification systems for primary CMP vary among major cardiology organizations.

Purpose of the Study:

To review the epidemiology, pathophysiology, diagnosis, and treatment of cardiomyopathy.
To provide pediatricians with insights for early identification and management of idiopathic CMP in children.
To highlight the role of genetic testing in CMP diagnosis, care, and prognosis.

Main Methods:

Literature review focusing on cardiomyopathy epidemiology, pathophysiology, diagnosis, and treatment.

Keywords:

Cardiomyopaties Child Genes

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Analysis of genetic factors and their impact on CMP incidence and classification.

Examination of diagnostic and therapeutic strategies for idiopathic CMP in pediatric populations.

Main Results:

CMP presents as dilated or hypertrophic types, with primary CMP often linked to genetic factors and family history.
Genetic testing is vital for confirming CMP diagnosis, personalizing patient care, and potentially improving prognosis.
Identifying genetic causes in families can lead to the discovery of novel therapeutic targets for CMP.

Conclusions:

Early identification and management of pediatric idiopathic CMP are critical.
Genetic testing offers significant benefits for patients and families affected by cardiomyopathy.
Further research into genetic factors may unlock new treatment avenues for this complex disease.