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Related Concept Videos

Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

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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...
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Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

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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,...
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Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

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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...
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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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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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Cardiomyopathy VI: Nursing Management01:29

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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 6, 2026

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Left ventricular noncompaction cardiomyopathy: updated review.

Dioma U Udeoji1, Kiran J Philip, Ryan P Morrissey

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Therapeutic Advances in Cardiovascular Disease
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Left ventricular noncompaction (LVNC) is a rare congenital heart defect. Early diagnosis and symptom-based management are crucial for improving outcomes in patients with this genetically diverse condition.

Keywords:
cardiomyopathygeneticsnoncompaction

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Area of Science:

  • Cardiology
  • Genetics
  • Pediatric Cardiology

Background:

  • Left ventricular noncompaction (LVNC) is a rare congenital cardiomyopathy.
  • It results from incomplete myocardial compaction during fetal development.
  • LVNC exhibits genetic heterogeneity and varied clinical presentations.

Purpose of the Study:

  • To review the pathogenesis, clinical features, and management of LVNC.
  • To discuss diagnostic approaches and prognosis.
  • To highlight the importance of accurate diagnosis and long-term care.

Main Methods:

  • Literature review of medical publications from 1984 to 2013.
  • Analysis of studies on LVNC pathogenesis, clinical manifestations, and treatment.
  • Synthesis of information on diagnostic modalities and genetic patterns.

Main Results:

  • LVNC presents with diverse genetic patterns (X-linked, autosomal-dominant, mitochondrial).
  • Clinical manifestations range from asymptomatic cases to severe heart failure, arrhythmias, and sudden cardiac death.
  • Improved diagnostic technologies have increased identification and improved prognosis.

Conclusions:

  • LVNC is a primary genetic cardiomyopathy with significant variability.
  • Accurate diagnosis is essential due to potential co-occurring anomalies and the need for family screening.
  • Symptom-based management is key, with symptomatic patients facing a poorer prognosis.