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Related Concept Videos

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...
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...
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...
Cardiomyopathy II: Dilated Cardiomyopathy01:30

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,...
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...
Cardiomyopathy V: Interprofessional Care01:29

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...

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Isolated left ventricular noncompaction syndrome.

Christopher Stanton1, Charles Bruce, Heidi Connolly

  • 1Mayo Clinic, Rochester, Minnesota.

The American Journal of Cardiology
|October 6, 2009
PubMed
Summary

Isolated left ventricular noncompaction (ILVNC) outcomes are similar to dilated cardiomyopathy. Deaths in ILVNC patients occurred only with reduced ejection fraction, suggesting targeted implantable cardioverter-defibrillator (ICD) use.

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Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Isolated left ventricular noncompaction (ILVNC) is a rare congenital cardiomyopathy.
  • Its clinical characteristics, outcomes, and therapies are not well-defined.

Purpose of the Study:

  • To analyze clinical characteristics and outcomes of ILVNC patients.
  • To compare ILVNC outcomes to community and dilated cardiomyopathy (DC) controls.
  • To examine implantable cardioverter-defibrillator (ICD) therapies in ILVNC.

Main Methods:

  • Retrospective analysis of 30 ILVNC patients diagnosed between 2001-2006.
  • Comparison of mortality, stroke, and atrial fibrillation (AF) with control groups.
  • Evaluation of ICD therapies and adverse events.

Main Results:

  • No significant mortality difference between ILVNC, DC, and community controls.
  • Deaths in ILVNC patients occurred only with decreased left ventricular ejection fraction.
  • New-onset AF diagnosed in 2 ILVNC patients; 2 experienced inappropriate ICD therapies.

Conclusions:

  • Mortality in ILVNC is comparable to DC.
  • ICD therapy may be beneficial for ILVNC patients with reduced ejection fraction.
  • Atrial fibrillation can lead to inappropriate ICD discharges in ILVNC.