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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

24
Q

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

A

Left atrium

25
Microscopic structures for hypertrophic cardiomyopathy
Hypertrophied myofibers Haphazard arrangement and branching Interstitial fibrosis
26
Diagnostic criteria for hypertrophic cardiomyopathy
LV wall >15mm Family history of mutation Exclude other causes (HTN)
27
Hypertrophic cardiomyopathy is more common in (men/women) and (AA/Caucasians)
Men; AA
28
Hypertrophic cardiomyopathy is a diastolic issue due to...
stiff and progressive decrease in chamber volume (until tract obstruction, then systolic issue also)
29
Most common symptom of hypertrophic cardiomyopathy
Dyspnea (due to elevated filling pressures and decreased stroke volume)
30
Diastolic dysfunction (such as HCM) due to stiff ventricle can cause higher left ventricular pressure, leading to increased ______ ______ _____ and pulmonary edema
left atrial pressure
31
Why might hypertrophic cardiomyopathy cause angina?
Dec. Cardiac Output | Arrhythmias (A-fib or V-tach)
32
Why might hypertrophic cardiomyopathy cause syncope?
Dec. Cardiac Output | Arrhythmias (A-fib or V-tach)
33
Prevention of SCD
ICD
34
Hypertrophic cardiomyopathy can result in what heart sounds and why?
``` S4 gallop (late diastolic filling on a stiff ventricle) Crescendo-Decrescendo murmur (outflow obstruction from IVS and/or mitral leaflet) ```
35
What heart sound is NEVER present in A-fib
S4
36
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)
Crescendo-decrescendo
37
Mitral regurgitation due to systolic anterior motion; best heard at apex (Crescendo-decrescendo or Holosystolic)
Holosystolic
38
Differential for a harsh crescendo-decrescendo systolic murmur
Aortic stenosis | Hypertrophic cardiomyopathy
39
How to tell between an aortic stenosis and HCM murmur
Valsalva and Standing makes HCM murmur worse (dec. preload, chamber gets smaller) Squatting makes better (inc. preload, chamber gets bigger)
40
Diagnosis of hypertrophic cardiomyopathy
EKG with LVH MRI with thick septum Genetic testing (not required)
41
A negative test for HCM mutations excludes the diagnosis (True or False)
False (not all mutations known)
42
Treatment for HCM
``` ICD (for potential SCD) B-blockers (angina) Ca-channel blockers (angina) Limit exertion (SCD) Surgical myectomy Alcohol septal ablation ```
43
An ICD should be implanted for primary prevention of SCD in patients with HCM and >1 of what risk factors (5 total)
``` Family history SCD History of syncope V-tach Wall >30mm Abnormal BP response to exercise ```
44
Treatment procedures for hypertrophic cardiomyopathy
Surgical myectomy | Alcohol septal ablation
45
least common cardiomyopathy; decrease in ventricular compliance resulting in impaired filling; inc. diastolic pressure and dec. cardiac output; amyloidosis, fibroelastosis, sarcoidosis or hemochromatosis
Infiltrative/Restrictive Cardiomyopathy
46
Subtype of infiltrative cardiomyopathy; macroscopic gray-pink deposits; microscopic amorphous, eosinophilic proteinaceous substance; Congo red stain positive (and green polarization)
Amyloidosis
47
Subtype of infiltrative cardiomyopathy; excessive accumulation of body iron (abnormal metabolism?); cardiomegaly with inc. myocyte hemosiderin and fibrosis; iron will stain blue on microscopy
Hemochromatosis
48
Subtype of infiltrative cardiomyopathy; formation of granulomas; more common in SE and African-Americans
Sarcoidosis
49
Uncommon associations with infiltrative cardiomyopathy
``` Endomyocardial fibrosis (tropical EF and Loeffler endomyocarditis) Endocardial fibroelastosis ```
50
Amyloidosis diagnosis
Echo (thick walls and dilated atria) EKG (low voltage and Q waves) Endomyocardial biopsy
51
Clinical manifestations of amyloidosis
Diastolic dysfunction (poor compliance) Heart block/arrhythmias Postural hypotension Stroke (intracardiac thrombus)
52
Treatment for amyloidosis
Can't use HF drugs (limited efficacy) AL amyloidosis: BM transplant Hereditary amyloidosis: heart transplant
53
Treatment for sarcoidosis
Glucocorticoids HF therapies Pacemaker/ICD Heart Transplant if severe
54
ARVD
Arrhythmogenic RV Dysplasia
55
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
Arrhythmogenic RV Cardiomyopathy
56
Treatment for Arrhythmogenic Right Ventricular Cardiomyopathy
Limited physical exertion | ICD for SCD

Cardiology-Exam 2 (23 decks)

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