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Related Concept Videos

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 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 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,...
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...
Mitral Valve Prolapse I: Introduction01:27

Mitral Valve Prolapse I: Introduction

IntroductionThe mitral valve, one of the heart's four valves, regulates blood flow. These valves have flaps that open and close to direct blood properly through the heart and body. During each heartbeat, the flaps open for blood to pass through and seal shut to prevent backflow. Specifically, the mitral valve opens to allow blood flow from the heart's upper left chamber to the lower left chamber. It then closes securely as the lower left chamber contracts to pump blood to the body, preventing...
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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Mechanical Control of Relaxation Using Intact Cardiac Trabeculae
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Acquired left ventricular hypertrabeculation/noncompaction in myotonic dystrophy type 1.

J Finsterer, C Stöllberger, R Wegmann

    International Journal of Cardiology
    |August 12, 2008
    PubMed
    Summary
    This summary is machine-generated.

    Acquired left ventricular hypertrabeculation/noncompaction (LVHT) was observed in a patient with myotonic dystrophy type 1 (MD1). This case highlights a previously undescribed cardiac manifestation in MD1 patients.

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    Area of Science:

    • Cardiology
    • Genetics
    • Neuromuscular Disorders

    Background:

    • Congenital left ventricular hypertrabeculation/noncompaction (LVHT) is frequently associated with neuromuscular disorders (NMDs).
    • Acquired LVHT is rare and has not been previously reported in myotonic dystrophy type 1 (MD1).

    Observation:

    • A 48-year-old female with MD1 (CTG-repeat expansion 700-800) presented with LVHT in the apex and mid-ventricular septum.
    • Previous echocardiograms showed no evidence of LVHT, suggesting an acquired condition.
    • The patient had a normal cardiac history and examination, but ECG revealed non-specific ST-abnormalities and supraventricular ectopic beats.

    Findings:

    • Echocardiography revealed LVHT in the apex and mid-ventricular septum in the MD1 patient.
    • Ambulatory ECG monitoring indicated episodic sinus bradycardia, ventricular ectopic beats, and sleep apnea syndrome.
    • The LVHT was deemed acquired due to its absence in prior cardiac assessments.

    Implications:

    • This case represents the first description of acquired LVHT associated with MD1.
    • It expands the spectrum of cardiac manifestations in MD1.
    • Further research may explore the mechanisms and prevalence of acquired LVHT in NMDs.