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Cardiology > Cardiomyopathy - HCM, DCM, RCM, ARVC > Flashcards

Cardiomyopathy - HCM, DCM, RCM, ARVC Flashcards

1
Q

What is cardiomyopathy and what are the different types? [5]

A
  1. disease of the myocardium that affects the mechanical or electrical function of the heart
  2. hypertrophic cardiomyopathy (HCM)
  3. dilated cardiomyopathy (DCM)
  4. restricted cardiomyopathy (RCM)
  5. arrhythmogenic right ventricular cardiomyopathy (ARVC)
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2
Q

What are the risk factors of cardiomyopathy? [5]

A
  1. family history of cardiomyopathy
  2. high blood pressure
  3. obesity
  4. diabetes
  5. previous MI
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3
Q

What is hypertrophic cardiomyopathy? [2]

A
  1. ventricular hypertrophy/thickening of the myocardium

2. sarcomere pathology

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4
Q

What is the epidemiology of hypertrophic cardiomyopathy? [3]

A
  1. quite common (prevalence 0.2%)
  2. leading cause of sudden cardiac death in the young
  3. autosomal dominant inheritance; ask about family history of sudden death
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5
Q

What is the pathophysiology of hypertrophic cardiomyopathy? [3]

A
  1. caused by sarcomeric mutations in beta myosin, alpha tropomyosin and troponin T
  2. hypertrophic non-compliant ventricles impair diastolic filling, resulting in decreased stroke volume and thus cardiac output
  3. there is also myofibrillar disarray of cardiac myocytes, which affects cardiac conduction
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6
Q

What is the clinical presentation of hypertrophic cardiomyopathy? [5]

A
  1. sudden death may be first manifestation of HCM
  2. chest pain, angina, dyspnoea, palpitations, syncope
  3. cardiac arrhythmia
  4. ejection systole murmur
  5. jerky carotid pulse
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7
Q

How can hypertrophic cardiomyopathy be diagnosed? [3]

A
  1. ECG - left ventricular hypertrophy, progressive T wave inversion, deep Q waves
  2. echocardiogram - ventricular hypertrophy with small left ventricle cavity
  3. genetic analysis - ask about family history of sudden death
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8
Q

What is the treatment of hypertrophic cardiomyopathy? [3]

A
  1. beta blockers - e.g. atenolol
  2. calcium channel blockers - e.g. verapamil
  3. amiodarone
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9
Q

What is dilated cardiomyopathy? [2]

A
  1. enlarged heart with dilated ventricles that contract poorly and cannot pump blood effectively
  2. cytoskeletal pathology
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10
Q

What is the epidemiology of dilated cardiomyopathy? [3]

A
  1. most common cardiomyopathy
  2. associations - alcohol, increased BP, thyrotoxicosis, congenital
  3. autosomal dominant inheritance
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11
Q

What is the pathophysiology of dilated cardiomyopathy? [2]

A
  1. dilation of left ventricle / right ventricle / all four chambers of the heart
  2. poorly generated contractile force leading to progressive dilation of the heart
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12
Q

What is the clinical presentation of dilated cardiomyopathy? [7]

A
  1. shortness of breath
  2. fatigue, dyspnoea
  3. pulmonary oedema
  4. arrhythmias
  5. heart failure as ventricles can’t contract properly
  6. increased pulse but decreased blood pressure
  7. increased jugular venous pressure
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13
Q

How can dilated cardiomyopathy be diagnosed? [3]

A
  1. chest x-ray - cardiomegaly and pulmonary oedema
  2. ECG - tachycardia, arrhythmia and non-specific T wave changes
  3. echocardiogram - globally dilated hypokinetic heart with low ejection fraction
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14
Q

What is the treatment for dilated cardiomyopathy? [3]

A
  1. beta blockers - e.g. atenolol, bisoprolol
  2. ACE inhibitors - e.g. ramipril, lisinopril
  3. thiazide diuretics - e.g. bendroflumethiazide
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15
Q

What is restrictive cardiomyopathy? [1]

A
  1. walls of the heart are rigid and thus the heart is restricted from stretching and filling with blood properly during diastole
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16
Q

What is the aetiology of restrictive cardiomyopathy? [4]

A
  1. idiopathic
  2. amyloidosis
  3. sarcoidosis
  4. endomyocardial fibrosis
17
Q

What is the pathophysiology of restrictive cardiomyopathy? [2]

A
  1. normal/decreased volume of both ventricles with bi-atrial enlargement and impaired diastolic ventricular filling
  2. poor dilation of the heart restricts its ability to receive blood and pump it to the rest of the body
18
Q

What is the clinical presentation of restrictive cardiomyopathy? [6]

A
  1. dyspnoea and fatigue
  2. increased jugular venous pressure
  3. Kussmaul’s sign - rise in jugular venous pressure and increased neck vein distension during inspiration
  4. pulsus paradoxus - large decrease in stroke volume and systolic blood pressure during inspiration
  5. hepatomegaly
  6. oedema and ascites
19
Q

How can restrictive cardiomyopathy be diagnosed? [5]

A
  1. chest x-ray
  2. echocardiogram
  3. ECG
  4. cardiac catheterisation
  5. CT/MRI to distinguish from constrictive pericarditis
20
Q

What is the treatment of restrictive cardiomyopathy? [2]

A
  1. treat the underlying cause of RCM

2. consider cardiac transplantation

21
Q

What is arrhythmogenic right ventricular cardiomyopathy (ARVC)? [2]

A
  1. affects the right ventricle of the heart and causes arrhythmias
  2. desmosome / cell adhesion pathology
22
Q

What is the aetiology of ARVC? [3]

A
  1. progressive genetic cardiomyopathy characterised by right ventricular myocardium replaced with fibro-fatty material
  2. cause is idiopathic
  3. usually autosomal dominant with incomplete penetrance
23
Q

What is the pathophysiology of ARVC? [2]

A
  1. mutation in genes encoding for desmosomes leading to inefficient cell adhesion, resulting in cell separation
  2. right ventricular myocardium replaced with fibro-fatty material
24
Q

What is the clinical presentation of ARVC? [4]

A
  1. cardiac cells held together less well, thus there are conduction issues
  2. arrhythmia is the most common feature
  3. palpitations and syncope during exercise
  4. Naxos disease - palmoplantar keratoderma, woolly hair, fibrofatty heart
25
Q

How can ARVC be diagnosed? [3]

A
  1. ECG - epsilon waves present, broad QRS complex in V1 to V3, with T wave inversion
  2. echocardiogram - enlarged hypokinetic right ventricle with thin RV wall
  3. genetic testing - autosomal dominant trait with reduced penetrance, desmosome mutations
26
Q

What is the treatment for ARVC? [3]

A
  1. beta blockers - e.g. atenolol
  2. amiodarone
  3. cardiac transplant indicated occasionally

Cardiology (5 decks)

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