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MHD 1 > L46 Cardiomyopathy > Flashcards

L46 Cardiomyopathy Flashcards

1
Q

What is a cardiomyopathy?

A

Primary abnormality of the myocardium in which the dysfunction is not attributable to pressure or volume overload.

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2
Q

What are the three types of cardiomyopathy?

A
  1. Dilated
  2. Hypertrophic
  3. Restrictive
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3
Q

What is the most common type of cardiomyopathy?

A

Dilated

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4
Q

What is caused by dilated cardiomyopathy?

A

Contractile (systolic) dysfunction; four chamber dilation, heart is often 2-3x normal weight

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5
Q

Describe the gross changes to the heart in dilated cardiomyopathy.

A

Lots of dilation, some hypertrophy

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6
Q

What are the non-genetic causes of dilated cardiomyopathy?

A
  1. Myocarditis
  2. Peripartum
  3. Toxic (alcohol)
  4. Idiopathic
  5. Iron overload
  6. Stress-provoked
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7
Q

___% of dilated cardiomyopathy is genetic.

A

25-50

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8
Q

Broadly, describe the genetic causes of dilated cardiomyopathy.

A

Various proteins are mutated leading to defects in force generation/transmission and/or myocyte signaling.

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9
Q

What is the phenotype of dilated cardiomyopathy?

A

Hypertrophy, dilation, fibrosis, intracardiac thrombi

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10
Q

What are the clinical outcomes of both dilated and hypertrophic cardiomyopathy?

A

Heart failure, sudden death, atrial fibrillation, stroke

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11
Q

Discuss the hypertrophic sarcomere changes that occur in response to volume overload.

A

New sarcomeres are added in series (increases myocyte length and cavity volume)

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12
Q

Discuss the hypertrophic sarcomere changes that occur in response to pressure overload.

A

New sarcomeres are added in parallel, increasing myocyte thickness, decreases cavity volume

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13
Q

What happens as a result of dilated cardiomyopathy?

A

Progressive systolic CHF –> heart failure symptoms, arrhythmias, and mural thrombi with embolic complications

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14
Q

How is dilated cardiomyopathy treated?

A

Medical therapy and heart transplantation

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15
Q

What causes hypertrophic cardiomyopathy (HOCM)?

A

Marked left ventricular myocardial hypertrophy

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16
Q

Describe the gross changes to the heart in hypertrophic cardiomyopathy.

A

The septum hypertrophies more than the free wall, creating a banana-shaped left ventricular cavity; this causes abnormal diastolic filling.

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17
Q

What can happen as a result of hypertrophic cardiomyopathy?

A

LV outflow obstruction (septal hypertrophy, anterior mitral valve leaflet contacts ventricular septum)

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18
Q

Describe the histology of hypertrophic cardiomyopathy.

A

Hypertrophied myocytes, disorganized myocytes, interstitial fibrosis

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19
Q

What are the primary causes of hypertrophic cardiomyopathy?

A

Genetic (autosomal dominant)

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20
Q

What happens when there are mutations in genes that encode sarcomeres (regarding hypertrophic cardiomyopathy)?

A

Increased myofilament function and myocyte hypercontractility

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21
Q

What are the most frequent mutations seen in hypertrophic cardiomyopathy?

A

Beta-myosin heavy chain (most common), also myosin binding protein C and troponin T

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22
Q

What are the clinical manifestations of hypertrophic cardiomyopathy?

A

Diastolic heart failure, exertional dyspnea, harsh systolic ejection murmur, anginal pain, intractable heart failure, arrhythmias

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23
Q

What is the classic story related to hypertrophic cardiomyopathy?

A

Sudden death in young athletes

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24
Q

How is hypertrophic cardiomyopathy treated?

A

Medical therapy that enhances ventricular relaxation, surgical excision of muscle

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25
Q

What causes restrictive cardiomyopathy?

A

Primary decrease in ventricular compliance leading to impeded LV filling during diastole (systolic function is preserved)

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26
Q

Describe the gross changes to the heart in restrictive cardiomyopathy.

A

The left atrium is dilated with a firm stiff myocardium, limiting the expansion ability; the cavity itself is normal

27
Q

What are some of the causes of restrictive cardiomyopathy?

A
  1. Idiopathic
  2. Radiation fibrosis
  3. Amyloidosis
  4. Sarcoidosis
  5. Inborn errors of metabolism
  6. Endocardial fibroelastosis (thickening of endocardium)
  7. Loeffler endomyocarditis (thickening of endocardium + eosinophilic infiltrate)
  8. Iron overload
28
Q

What are amyloids?

A

Misfolded proteins deposited in extracellular spaces that cause tissue damage

29
Q

What are common features of amyloid deposits?

A

Beta-pleated sheet configuration, congo red staining in tissue, apple-green under polarized light

30
Q

What is one example of a common amyloid?

A

Transthyretin

31
Q

What causes the signs and symptoms of restrictive cardiomyopathy?

A

Reduced myocardial compliance and SV

32
Q

What is myocarditis?

A

Inflammatory process targeting the myocardium, resulting in myocyte injur

33
Q

What are the infectious causes of myocarditis?

A
  1. Viruses
  2. Bacteria
  3. Parasites
34
Q

What is the most common cause of myocarditis?

A

Enteroviruses

35
Q

What is a common type of enterovirus that causes myocarditis?

A

Coxsakie A and B

36
Q

What are 2 other viruses that can cause myocarditis?

A

Cytomegalovirus and HIV

37
Q

What is the pathogenesis of myocarditis caused by viruses?

A

Directly cause cell death or immune response directed against virus results in myocyte injury

38
Q

What are 2 bacteria that cause myocarditis?

A

Diptheria and B. burgdorferi (Lyme disease)

39
Q

What are 3 parasites that cause myocarditis?

A
  1. Trypanasoma cruzi
  2. Trichinosis
  3. Toxoplasmosis
40
Q

What is caused by Trypanasoma cruzi and where is it found?

A

Chaga’s disease; endemic in South America

41
Q

What are some non-infectious causes of myocarditis?

A
  1. Immune-mediated (hypersensitivity reactions, RF, SLE)

2. Giant cell myocarditis

42
Q

Describe the histological findings of myocarditis that is lymphocytic, hypersensitivity, giant cell, and Chaga’s.

A

Lymphocytic: lots of lymphocytes
Hypersensitivity: + eosinophils
Giant cell: giant cells
Chagas: parasite

43
Q

What are the clinical manifestations of myocarditis?

A
  1. Can be asymptomatic, non-specific symptoms
  2. Acute CHF
  3. Arrhythmias
  4. Progression to dilated cardiomyopathy
44
Q

What are the two layers of the pericardium?

A
  1. Visceral (touches endocardium)

2. Parietal

45
Q

What types of fluid can be seen in a pericardial effusion?

A

Clear yellow serous fluid, blood, pus

46
Q

What is the classic finding of a pericardial effusion on CXR?

A

Globular enlargement

47
Q

What can happen with rapidly developing or large pericardial effusions?

A

Atria and vena cavae are compressed, followed by the ventricles; this restricts cardiac filling. It can end in cardiac tamponade.

48
Q

What is pericarditis?

A

Inflammation of the pericardium, usually secondary to a cardiac, thoracic, or systemic process

49
Q

What can cause pericarditis?

A
  1. Infections (any type of microorganism)
  2. Immune-mediated (RF, SLE, hypersensitivity, uremia, post-myocardial infarction/Dressler syndrome)
  3. Neoplasia
  4. Trauma
  5. Radiation
50
Q

What is Dressler syndrome?

A

Complication of an MI that causes an autoimmune phenomenon

51
Q

What are the 4 types of pericarditis?

A
  1. Fibrinous
  2. Suppurative (Fibrinopurulent)
  3. Hemorrhagic
  4. Caseous
52
Q

Describe the appearance of fibrinous pericarditis.

A

Pericardial surface is irregular and shaggy

53
Q

What can cause fibrinous pericarditis?

A

Acute viral pericarditis, uremia, and acute RF

54
Q

What is a physical exam finding of fibrinous pericarditis?

A

Pericardial friction rub

55
Q

What causes suppurative pericarditis?

A

Acute bacterial infection (can be extension or seeding)

56
Q

Describe the appearance of suppurative pericarditis.

A

Pus, ya’ll

57
Q

What can cause hemorrhagic pericarditis?

A

TB, malignancy

58
Q

What can cause caseous pericarditis?

A

TB

59
Q

How does pericarditis present (labs and symptoms)?

A
  1. Asymptomatic
  2. Chest pain (gets worse lying down)
  3. Systemic complaints
  4. Friction rub
  5. EKG changes (diffuse ST elevation)
60
Q

Describe the healing seen in pericarditis.

A

Focal plaque-like thickening and mild adhesions, can lead to constrictive pericarditis in which the pericardial space is obliterated, the heart is surrounded by a dense scare, and diastolic expansion is limited (reduced CO)

61
Q

How is constrictive pericarditis treated?

A

Pericardiectomy

62
Q

When is cardiac transplantation indicated?

A

Intractable heart failure (dilated and ischemic cardiomyopathy)

63
Q

What are complications of cardiac transplantation?

A
  1. Acute or chronic rejection
  2. Infection
  3. Post-transplant lymphoma (Epstein-Barr virus)
  4. Allograft arteriopathy (fibromuscular intimal hyperplasia)
64
Q

What part of the heart is biopsied?

A

Septum

MHD 1 (58 decks)

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