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PTE me > Cardiomyopathies > Flashcards

Cardiomyopathies Flashcards

1
Q

What are the three major types of cardiomyopathies?

A
  1. Dilated
  2. Restrictive
  3. Infiltrative (Restrictive Infiltrative)
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2
Q

What % of Dilated cardiomyopathies have diminished systolic dysfunction?

A

100%

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3
Q

What leaflet motion is present in the mitral valve for dilated cardiomyopathy?

A

Carpentier Class 3b = Restricted leaflet motion in systole

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4
Q

What is a Type I Carpentier Mitral Valve Motion?

A

Normal Motion of Mitral Valve Leaflets

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5
Q

What is a Type II Carpentier Mitral Valve Motion?

A

Increased mobility

Flail leaflet

Ruptured cord

Billowing

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6
Q

What is a Type IIIa Carpentier Mitral Valve Motion?

A

Restricted motion during sysole and diastole

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7
Q

What is the inheritance pattern for hypertrophic cardiomyopathy?

(Specific 3 things)

A

1. Autosomal Dominant

2. Variable Penetrance - Penetrance refers to the proportion of people with a particular genetic change (such as a mutation in a specific gene) who exhibit signs and symptoms of a genetic disorder

3. Variable expressivity - In genetics, expressivity is the degree to which a phenotype is expressed by individuals having a particular genotype

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8
Q

What arrythmogenic complications can arise from hypertrophic cardiomyopathy patients?

A

RBBB

(Will have pacemakers in place that depolarize right heart before left heart)

Septum depolarizes before lataral wall

This will decrease the outflow tract obstruction

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9
Q

What is the most common substance in infiltrative cardiomyopathy?

A

Beta Amyloid in Amyloidosis

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10
Q

What % of patients with infiltrative cardiomyopathy have diastolic dysfunction?

A

100%

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11
Q

What is the most common cardiomyopathy?

A

Dilated Cardiomyopathy

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12
Q

What are the major etiologies of dilated cardiomyopathy?

A
  1. Idiopathic
  2. Peripartum
  3. Infections
  4. Genetic (Muscular Dystrophy)
  5. Toxins
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13
Q

What infections can cause a dilated cardiomyopathy?

A

Post Viral (Coxsackie B virus, Chagas Disease)

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14
Q

What toxins can cause dilated cardiomyopathy?

A

Alcohol

Doxorubicin

Cobalt

Snake Bites

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15
Q

What is the mortality rate of dilated cardiomyopathy for 2 years?

A

50%

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16
Q

What is the mortality rate of dilated cardiomyopathy for 5 years?

A

75%

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17
Q

What percent of dilated cardiomyopathy improve?

A

25%

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18
Q

What end diastolic diameter of dilated cardiomyopathy predicts a higher mortality?

A

> 4 cm / m2

19
Q

What pathology is seen with Type IIIa leaflet motion?

A

Rheumatic Heart Disease

20
Q

Outside of Dilated cardiomyopathy, when will we see Type IIIb motion of the mitral valve?

A

Ischemic mitral insufficiency

More of a ventricular (papillary muscle) problem not a leaflet problem

21
Q

What emptying velocity using pulse wave doppler on the left atrial appendage when is there likely to be no clot?

A

>40 cm/sec = Risk of low

>55 cm/sec = 100% negative predictive value

22
Q

When is there a 100% Negative predictive value of thrombus in the left atrial appendage?

A

55-60 cm/sec

23
Q

What wall is typically spared of hypertrophy in all subtypes of Hypertrophic cardiomyopathy?

A

Basal Inferolataral wall

AKA
Basal Posterior wall

24
Q

Comment on the systolic function of Hypertrophic cardiomyopatahy.

A

Normal

25
Q

Comment on the diastolic function of Hypertrophic cardiomyopatahy.

A

Grossly abnormal

26
Q

What percentage of hypertrophic cardiomyopathy patients have obstructive physiology in the LVOT?

A

25%

27
Q

What is seen here?

A

Y sign for LVOT obstruction

  1. Anterior motion of Mitral Valve
  2. Posterior direction of mitral insufficiency
28
Q

What is seen here on CWD?

A

Late peaking dagger shaped CWD profile

Early in systole = No flow acceleration

Late in systole = Late peaking dagger shape

29
Q

If you want to use M-mode to assess HOCM, what do you do?

A

Put cursor across Aortic Valve in Long Axis

Select M Mode

30
Q

What is seen here in M-mode across aortic valve?

A
  1. During systole, valve abruptly opens
  2. Valve stays open

Resembles a “box”

31
Q

What is seen here?

A
  1. Aortic valve leaflets open, but close pre-maturely due to LVOT proximally

*Coarse fluttering of the aortic valve leaftlets with premature closure*

  1. Proximal obstruction pushing the leaflet open
32
Q

What are the two major etiologies of Restrictive infiltrative cardiomyopathy?

A
  1. Primary
  2. Secondary
33
Q

What are the three major primary etiologies of Restrictive infiltrative cardiomyopathy?

A
  1. Loeffler’s Hypereosinophilic Endocarditits
  2. Endomyocardial Fibrosis
  3. Idiopathic
34
Q

What are the six major secondary etiologies of Restrictive infiltrative cardiomyopathy?

A
  1. Amyloidosis
  2. Sarcoidosis
  3. Glycogen Storage Diseases
  4. Hemochromatosis
  5. Drugs (Antrhocyclines, Ergotamines, Methysergide, Serotonin)
  6. Radiation
35
Q

Of the primary and secondary causes of Restrictive infiltrative cardiomyopathy, which is the most common?

A

Amyloidosis

36
Q

What is the major pathophysiologic cause of amyloidosis causing Restrictive infiltrative cardiomyopathy?

A

infiltration of Beta Amyloid

37
Q

How does the myocardium appear in amyloidosis?

A

Granular, Speckled or Starry Skied

38
Q

What is the systolic and diastolic function of Restrictive infiltrative cardiomyopathy?

A

Diastolic dysfunction always

Systolic dysfunction

39
Q

How do you distinguish between restrictive infiltrative cardiomyopathy and constrictive pericarditis?

A

RICM:

Restrictive transmitral inflow velocitites (E > A)

Reduced tissue doppler velocities

Constrictive Pericarditis:

Restrictive transmitral inflow velocitites (E > A)

Normal Tissue doppler velocities (even elevated sometimes)

40
Q

Quantitatively, what is the EM in RICM vs. Constrictive Pericarditis?

A

EM < 8 cm/sec in RICM

EM >8 cm/sec in constrictive pericarditis

41
Q

What determines prognosis in Restrictive infiltrative cardiomyopathy?

A

Wall Thickness

Diastolic Function
Systolic Function

42
Q

Once symptoms occur in RICM, what is survival?

A

2-3 year survival <50%

43
Q

What do amyloidosis patients require before heart transplantation?

A

Bone marrow biopsy to get rid of amyloid cells

44
Q

How do you distinguish an unroofed septal perforator vs. VSD

A

Look at timing of Flow LV to RV

Flow during systole with VSD
Flow during diastole with Unroofed septal perforator

PTE me (33 decks)

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