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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

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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...
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...
Heart Failure II: Pathophysiology01:29

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Systolic Heart Failure and Compensatory MechanismsSystolic heart failure (also termed HFrEF, Heart Failure with Reduced Ejection Fraction) is the most prevalent type of heart filure. It results in a decreased volume of blood being pumped from the ventricle. The aortic arch and carotid sinuses have baroreceptors that detect reduced blood pressure, triggering the sympathetic nervous system (SNS) to release epinephrine and norepinephrine. Initially, this response aims to boost heart rate and...
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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,...
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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...

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Right ventricular endomyocardial fibrosis.

Gouranga Santra1, Pradip Kumar Sinha, Sibaji Phaujdar

  • 1Dept. of Medicine, Medical College, 88 College Street, Kolkata, PIN-700073.

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Endomyocardial fibrosis, a restrictive cardiomyopathy, can affect the right ventricle. This case report highlights unusual features and its occurrence outside typical equatorial regions, emphasizing the need for more research.

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Area of Science:

  • Cardiology
  • Pathology

Background:

  • Endomyocardial fibrosis (EMF) is a restrictive cardiomyopathy characterized by subendocardial thickening.
  • It commonly affects biventricular or left ventricles, leading to diastolic dysfunction.
  • Isolated right ventricular involvement is rare, with most Indian cases reported from Kerala.

Observation:

  • This report details a rare case of isolated right ventricular EMF from West Bengal.
  • Key features included massive right atrial enlargement, tricuspid valve disorganization, and pericardial effusion.
  • Notably, the patient had a normal eosinophil count and the condition occurred outside the equatorial belt.

Findings:

  • X-ray findings mimicked pericardial effusion, masking the underlying EMF.
  • The case presents unique clinical and radiological characteristics.
  • Sporadic occurrence outside the equatorial belt challenges previous epidemiological understanding.

Implications:

  • This case underscores the diverse presentations of endomyocardial fibrosis.
  • It highlights the importance of considering EMF even with atypical geographical distribution.
  • Further research is crucial to understand the pathophysiology and epidemiology of this neglected condition.