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Cardiology-Exam 2 > Cardiomyopathy > Flashcards

Cardiomyopathy Flashcards

1
Q

“heart muscle disease”; heart disease from PRIMARY myocardial abnormality; excludes CAD, valve disease, HTN and nutritional disorders

A

Cardiomyopathies

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2
Q

Three subtypes of cardiomyopathies

A

Dilated (most common)

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3
Q

Most common cardiomyopathy; progressive cardiac dilation, ineffective contraction (cytoskeletal protein abnormalities) and eccentric hypertrophy of all chambers; autosomal dominant; can be caused by viral myocarditis, alcoholism, toxins or pregnancy

A

Dilated cardiomyopathy

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4
Q

Cardiomyopathy that can result from pregnancy (late pregnancy or first postpartum months) (poorly understood mechanism)

A

Dilated cardiomyopathy

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5
Q

Predominant mode of inheritance for dilated cardiomyopathy

A

Autosomal dominant

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6
Q

Key Protein mutations in dilated cardiomyopathy

A

Dystrophin
Desmin
Mitochondrial proteins
Troponin/Tropomyosin

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7
Q

Poor contractile function (like in dilated cardiomyopathy) predisposes one to…

A

mural thrombosis and embolism

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8
Q

Microscopic features of dilated cardiomyopathy

A

Rather non-specific (hypertrophy, fibrosis, wavy fibers, and sometimes inflammatory cells)

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9
Q

_________ __________ contributes to dilated cardiomyopathy

A

Myocyte lengthening

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10
Q

Microscopic differences between pathologic and physiologic hypertrophy

A

Pathologic: Fibrosis and +/- cardiac dysfunction (concentric hypertrophy)
Physiologic: No fibrosis or dysfunction

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11
Q

hypertrophic growth of the heart without overall enlargement; addition of sarcomeres to myocytes in parallel thickens walls and its volume is diminished

A

Concentric hypertrophy

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12
Q

hypertrophic growth of the heart with overall enlargement; addition of sarcomeres in series, leads to a large, dilated ventricle with relative wall thinning

A

Eccentric hypertrophy

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13
Q

Dilated cardiomyopathy can lead to a ________ shaped heart, as opposed to a cone shaped one

A

globe/round

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14
Q

Stain that can help detect collagen (fibrosis); dyes them blue

A

Trichrome stain

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15
Q

viral causes of myocarditis (4 total)

A

Coxsackievirus B
Parvo B19
Echovirus
HIV

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16
Q

Potential symptoms of heart failure (6 total)

A 
Jugular vein distention
Rales/crackles
S3 or S4
Orthopnea
Hepatomegaly
Peripheral Edema
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17
Q

Clinical manifestations of dilated cardiomyopathy

A 
Heart failure (DJV, rales, edema, etc.)
Arrhythmias (like sudden cardiac death)
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18
Q

Diagnosing dilated cardiomyopathy

A 
Chest X-ray (enlargement?)
EKG with LVH
Echo (for dilation)
Ejection fraction <40%
Exclude CAD
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19
Q

Key diagnostic criteria for dilated cardiomyopathy

A

Ejection fraction <40%

Exclude coronary artery disease (stress test or cath)

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20
Q

Treatment for dilated cardiomyopathy

A

Treat underlying cause (tach, alcohol, hyperthyroidism)
Similar to HF (ACEi, B-blockers, aldoesterone antagonist)
ICD for sudden cardiac death
Anti-arrhythmics as needed

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21
Q

also known as asymmetric septal hypertrophy; massive myocardial hypertrophy, abnormal diastolic filling (stiff) and possible outflow obstruction; autosomal dominant with variable penetrance/expression; abnormalities in troponins, tropomyosin and B-myosin heavy chain

A

Hypertrophic Cardiomyopathy

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22
Q

Hypertrophic cardiomyopathy mostly affects what parts of the heart

A

Left Ventricle

Interventricular septum

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23
Q

_________ thickening in hypertrophic cardiomyopathy can lead to outflow obstruction due to the anterior mitral valve

A

IVS

24
Q

______ ______ may dilate secondary to impaired LV diastolic filling (hypertrophic cardiomyopathy)

A

Left atrium

25
Q

Microscopic structures for hypertrophic cardiomyopathy

A

Hypertrophied myofibers
Haphazard arrangement and branching
Interstitial fibrosis

26
Q

Diagnostic criteria for hypertrophic cardiomyopathy

A

LV wall >15mm
Family history of mutation
Exclude other causes (HTN)

27
Q

Hypertrophic cardiomyopathy is more common in (men/women) and (AA/Caucasians)

A

Men; AA

28
Q

Hypertrophic cardiomyopathy is a diastolic issue due to…

A

stiff and progressive decrease in chamber volume (until tract obstruction, then systolic issue also)

29
Q

Most common symptom of hypertrophic cardiomyopathy

A

Dyspnea (due to elevated filling pressures and decreased stroke volume)

30
Q

Diastolic dysfunction (such as HCM) due to stiff ventricle can cause higher left ventricular pressure, leading to increased ______ ______ _____ and pulmonary edema

A

left atrial pressure

31
Q

Why might hypertrophic cardiomyopathy cause angina?

A

Dec. Cardiac Output

Arrhythmias (A-fib or V-tach)

32
Q

Why might hypertrophic cardiomyopathy cause syncope?

A

Dec. Cardiac Output

Arrhythmias (A-fib or V-tach)

33
Q

Prevention of SCD

A

ICD

34
Q

Hypertrophic cardiomyopathy can result in what heart sounds and why?

A 
S4 gallop (late diastolic filling on a stiff ventricle)
Crescendo-Decrescendo murmur (outflow obstruction from IVS and/or mitral leaflet)
35
Q

What heart sound is NEVER present in A-fib

A

S4

36
Q

In hypertrophic cardiomyopathy, an outflow murmur due to turbulent flow through the LV outflow tract; best heard at left-lower sternal border (Crescendo-decrescendo or Holosystolic)

A

Crescendo-decrescendo

37
Q

Mitral regurgitation due to systolic anterior motion; best heard at apex (Crescendo-decrescendo or Holosystolic)

A

Holosystolic

38
Q

Differential for a harsh crescendo-decrescendo systolic murmur

A

Aortic stenosis

Hypertrophic cardiomyopathy

39
Q

How to tell between an aortic stenosis and HCM murmur

A

Valsalva and Standing makes HCM murmur worse (dec. preload, chamber gets smaller)
Squatting makes better (inc. preload, chamber gets bigger)

40
Q

Diagnosis of hypertrophic cardiomyopathy

A

EKG with LVH
MRI with thick septum
Genetic testing (not required)

41
Q

A negative test for HCM mutations excludes the diagnosis (True or False)

A

False (not all mutations known)

42
Q

Treatment for HCM

A 
ICD (for potential SCD)
B-blockers (angina)
Ca-channel blockers (angina)
Limit exertion (SCD)
Surgical myectomy
Alcohol septal ablation
43
Q

An ICD should be implanted for primary prevention of SCD in patients with HCM and >1 of what risk factors (5 total)

A 
Family history SCD
History of syncope
V-tach
Wall >30mm
Abnormal BP response to exercise
44
Q

Treatment procedures for hypertrophic cardiomyopathy

A

Surgical myectomy

Alcohol septal ablation

45
Q

least common cardiomyopathy; decrease in ventricular compliance resulting in impaired filling; inc. diastolic pressure and dec. cardiac output; amyloidosis, fibroelastosis, sarcoidosis or hemochromatosis

A

Infiltrative/Restrictive Cardiomyopathy

46
Q

Subtype of infiltrative cardiomyopathy; macroscopic gray-pink deposits; microscopic amorphous, eosinophilic proteinaceous substance; Congo red stain positive (and green polarization)

A

Amyloidosis

47
Q

Subtype of infiltrative cardiomyopathy; excessive accumulation of body iron (abnormal metabolism?); cardiomegaly with inc. myocyte hemosiderin and fibrosis; iron will stain blue on microscopy

A

Hemochromatosis

48
Q

Subtype of infiltrative cardiomyopathy; formation of granulomas; more common in SE and African-Americans

A

Sarcoidosis

49
Q

Uncommon associations with infiltrative cardiomyopathy

A 
Endomyocardial fibrosis (tropical EF and Loeffler endomyocarditis)
Endocardial fibroelastosis
50
Q

Amyloidosis diagnosis

A

Echo (thick walls and dilated atria)
EKG (low voltage and Q waves)
Endomyocardial biopsy

51
Q

Clinical manifestations of amyloidosis

A

Diastolic dysfunction (poor compliance)
Heart block/arrhythmias
Postural hypotension
Stroke (intracardiac thrombus)

52
Q

Treatment for amyloidosis

A

Can’t use HF drugs (limited efficacy)
AL amyloidosis: BM transplant
Hereditary amyloidosis: heart transplant

53
Q

Treatment for sarcoidosis

A

Glucocorticoids
HF therapies
Pacemaker/ICD
Heart Transplant if severe

54
Q

ARVD

A

Arrhythmogenic RV Dysplasia

55
Q

Cardiomyopathy due to fibrofatty infiltration of the right ventricle which frequently results in ventricular arrhythmias; more common in Italy; genetic mutations in desmosomal proteins, like plakophilin

A

Arrhythmogenic RV Cardiomyopathy

56
Q

Treatment for Arrhythmogenic Right Ventricular Cardiomyopathy

A

Limited physical exertion

ICD for SCD

Cardiology-Exam 2 (23 decks)

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