Cardiomyopathy Flashcards

1
Q

How do you define cardiomyopathy?

A

Group of diseases of the myocardium that affect the mechanical or electrical function of the heart
All carry an arrhythmic risk

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2
Q

What are the 4 types of acquired cardiomyopathy?

A
  1. Hypertrophic
  2. Dilated
  3. Restricted
  4. Arrhythmogenic right ventricular
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3
Q

What are the risk factors for cardiomyopathy?

A
  • Family history of cardiomyopathy
  • High blood pressure
  • Obesity
  • Diabetes
  • Previous MI
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4
Q

What is hypertrophic cardiomyopathy?

A
  • Ventricular hypertrophy/thickening of the muscle - leads to a thick wall and a small cavity
  • Autosomal dominant - familial
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5
Q

What is the pathophysiology of hypertrophic cardiomyopathy?

A
  • Caused by sarcomeric protein gene mutations e.g troponin T and B-myosin
  • All in the absence of hypertension and valvular disease
  • The hypertrophic, non-compliant ventricles impair diastolic filling resulting in reduced stroke volume and thus cardiac output
  • Diastolic dysfunction
  • Another issue with thick powerful heart is that there is a disarray of cardiac myocytes so conduction is affected
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6
Q

Where is the hypertrophy most likely to occur?

A

In the interventricular septum

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7
Q

What is the epidemiology of hypertrophic cardiomyopathy?

A

1/500 people have it and it can occur at any age and its the most common cause of sudden cardiac death in the young

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8
Q

How does hypertrophic sometimes present cardiomyopathy present?

A

Sudden death may be the first manifestation (SADS - sudden arrhythmic death syndrome)

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9
Q

What are the signs of hypertrophic cardiomyopathy?

A
  • Cardiac arrhythmia
  • Ejection systolic murmur
  • Jerky carotid pulse
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10
Q

What are the symptoms of hypertrophic cardiomyopathy?

A
  • Chest pain
  • Angina
  • Dyspnea
  • Dizziness
  • Palpitation
  • Syncope
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11
Q

What would the ECG look like in hypertrophic myopathy?

A

Abnormal and shows signs of left ventricular hypertrophy with progressive T wave inversion and deep Q waves

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12
Q

What would confirm a diagnosis of hypertrophic myopathy?

A

Genetic testing as its an autosomal dominant condition

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13
Q

What is the pharmacological treatment for hypertrophic myopathy?

A
  • Amiodarone - Anti-arrythmatic medication, if at high risk of arrhythmia then can place an implantable cardiac defibrillator
  • Calcium channel blocker e.g. Verapamil
  • Beta-blocker e.g. Atenolol
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14
Q

What is dilated cardiomyopathy?

A
  • Dilated left ventricle which contracts poorly/has thin muscle
  • All 4 chambers are dilated
  • Heart failure diagnosis
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15
Q

What is the pathophysiology of dilated cardiomyopathy?

A

All four chambers of the heart become dilated leaving the muscle walls very thin for contraction so the contractions are weak and less blood is pumped out with each contraction.

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16
Q

What can cause dilated cardiomyopathy?

A
  • Idiopathic
  • Genetic mutation
  • Infection
17
Q

What are the signs of dilated cardiomyopathy?

A
  • Arrhythmias
  • Increased jugular venous pressure
  • Heart failure signs - since can’t contract
18
Q

What are the two main risks of dilated cardiomyopathy?

A
  • Can cause sudden death

- Can cause a Thromboembolism

19
Q

What would you see on the ECG in dilated cardiomyopathy?

A
  • Tachycardia
  • Arrhythmia
  • Non-specific T wave changes
20
Q

What would you see on the CXR in dilated cardiomyopathy?

A

Cardiac enlargement

21
Q

What is the treatment for dilated cardiomyopathy?

A

Heart failure and atrial fibrillation treated in conventional way

22
Q

What happens in restrictive cardiomyopathy?

A

The ventricles are stiff and restricted and so when they fill with blood they aren’t compliant and so can’t fill with as much blood so it pumps out less blood to the body.

23
Q

What type of heart failure does restrictive cardiomyopathy cause?

A

Diastolic heart failure (as the problem is during the filling)

24
Q

What can cause restrictive cardiomyopathy?

A
  • Amyloidosis
  • Sarcoidosis (granulomas form in tissues)
  • Endocardial fibroelastosis
  • Loffler endocarditis
  • Hemochromatosis - iron deposits in the heart tissue
  • Radiation
25
Q

What is amyloidosis?

A

Where proteins become misfolded and get deposited in different tissues. When this gets deposited in the cardiac tissue it can become stiff and cause restrictive cardiomyopathy.

26
Q

What do you see on the ECG in restrictive cardiomyopathy?

A

Low amplitude QRS

27
Q

What is the treatment for restrictive cardiomyopathy?

A
  • No specific treatment with poor prognosis
  • Patients die within a year
  • Can consider cardiac transplantation
28
Q

What is arrhythmogenic right ventricular cardiomyopathy?

A

Progressive genetic cardiomyopathy characterised by progressive fatty and fibrous replacement of ventricular myocardium

29
Q

What is the pathophysiology of arrhythmogenic right ventricular cardiomyopathy?

A
  • Desmosome (normally hold cardiac cells together) gene mutation
  • Right ventricle replaced by fat and fibrous tissue
  • Muscle dies and replaced by fat as part of inflammatory process
  • Cardiac cells are held less together thus conduction issues
30
Q

What are the signs of arrhythmogenic right ventricular cardiomyopathy?

A
  • ARRHYTHMIA is most common feature
  • In late stages may be signs of right heart failure
  • Naxos disease (autosomal recessive condition) can cause arrythmic cardiomyopathy - only one that has physical symptoms:
  • Palmoplantar keratoderma, wooly hair
31
Q

What would you see on the ECG in arrhythmogenic right ventricular cardiomyopathy?

A
  • Usually normal but may show T wave inversion

- Abnormal in anterior leads v1-v3

32
Q

What is the gold standard for diagnosis in arrhythmogenic right ventricular cardiomyopathy?

A

Genetic testing

33
Q

What is the pharmacological treatment in arrhythmogenic right ventricular cardiomyopathy?

A
  • Beta-blockers e.g. Atenolol for patients with non-life-threatening arrhythmias
  • Amiodarone for symptomatic arrhythmias