Heart - Cardiomyopathies and myocarditis

11 important questions on Heart - Cardiomyopathies and myocarditis

What is Dilated cardiomyopathy (DCM)

progressive cardiac dilation and contractile (systolic) dysfunction, usually with concurrent hypertrophy
characteristic: dilation of the ventricles

What is the fundamental defect in DCM?

ineffective contraction (end-stage: ejection fraction is typically less than 25%)

What is Arrhythmogenic right ventricular cardiomyopathy

autosomal dominant disorder that classically manifests with right-sided heart failure and rhythm disturbances, which can cause sudden cardiac death
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What is Hypertrophic cardiomyopathy (HCM)

myocardial hypertrophy, defective diastolic filling, and ventricular outflow obstruction

Name a characteristic of HCM

Asymmetric thickness of one wall of the heart (mostly septum)

Amyloidosis is a form of Restrictive cardiomyopathy. How is it caused?

The deposition of extracellular proteins with a predilection for forming insoluble β-pleated sheets

What are the consequenses of Amyloidosis

  1. Amyloid within and/or in between cardiomyocytes: inhibition of heart function
  2. Amyloid in blood vessels: can cause myocardial infarction

Endomyocardial fibrosis is a form of Restrictive cardiomyopathy. How is it caused?

dense diffuse fibrosis of the ventricular endocardium and subendocardium, often involving the tricuspid and mitral valves

Besides amyloid deposition in Restrictive cardiomyopathy, there are also other molecules that can be stored in the heart that are pathogenic. Name these molecules and their consequence

Storage of glycogen and iron. This results in less myofibrils and thus a decrease of heart function

Lymphocytic myocarditis is caused by a viral infection. What are possible consequences of lymphocytic myocarditis?

  • arrhythmia
  • vasospasm (=contraction of blood vessels): this can cause myocardial infarction
  • heart failure: probably related interleukins that have a negative effect on contractility
  • *NOTABLY: in LM is cell death of cardiomyocytes limited unless a patient has a fulminant myocarditis: then there is extensive cell death of cardiomyocytes  

In infectious myocarditis the predominant inflammatory cell points to the cause. Name the different cell types with their causative infection agent

lymphocytes: virus
neutrophilic granulocytes: fungi or bacteria
eosinophilic granulocytes: parasites
aggregates of macrophages (and/or Giant cells): tuberculosis

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