Cardiomyopathy Flashcards

1
Q

what is ischemic cardiomyopathy

A

HF following severe CAD, not a true cardiomyopathy

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2
Q

describe the dysfn in DCM

A

cardiac dilation and systolic dysfn, usually w/ hypertrophy and HF

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3
Q

some causes of DCM (6)

A

genetic, viral myocarditis, hyperthyroidism, EtOH, doxorubicin, peripartum

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4
Q

pathophys of genetic DCM

A

mostly autosomal dominant, usually cytoskeleton protein abnormalities

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5
Q

pathology of DCM

A

enlargment of all chambers, poor contractile fn expsoses to mural thrombosis and embolism

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6
Q

histo of DCM

A

myocyte hypertrophy, interstitial fibrosis, wavy fibers

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7
Q

characteristics of hypertrophic cardiomyopathy (HCM)

A

massive hypertrophy, abnormal diastolic filling (stiffened, less compliant), sometimes intermittent outflow obstruction

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8
Q

etiology of HCM

A

genetic usually AD w/ variable penetrance and expression

prognosis depends on mutation

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9
Q

patho of HCM

A

severe hypertrophy usually in LV or septum, massive cardiomegaly, systolic outflow obstruction, no dilation but LA could dilate

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10
Q

histo of HCM

A

severely hypertrophied myocytes, in disarray w/ abnormal branches and excess fibrosis

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11
Q

describe infiltrative cardiomyopathy

A

decrease in ventricular compliance resulting in impaired diastolic filling, most common caused by amyloidosis, hemochromatosis, sarcoidosis, fibrolatstosis

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12
Q

describe the amyloid subtype of ICM

A

congo red positive (green amyloid), myocyte atrophy and subendocardial deposits

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13
Q

hemochromatosis subtype of ICM

A

accumulation of iron leads to cardiomegaly w/ excess myocyte hemosiderin and interstitial fibrosis

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14
Q

what is endocardial fibroelastosis

A

happens to kids under 2, fibroelastic tissue in endocardium either focal or diffuse, assoc w/ AV obstrtuction

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15
Q

what viruses are involved w/ myocarditis and thus DCM

A

coxsackievirus B, parvovirus B19, echovirus, HIV

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16
Q

clinical manifestations of DCM

A

same as w/ left and right HF, arrythmias which can cause sudden death

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17
Q

Dx of DCM

A

xray: cardiomegaly
EKG: shows LVH
Echo: can see dilated chambers and reduced EF
cath/stress test: to rule out CAD

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18
Q

Tx of DCM

A

underlying causes- tachy, EtOH, hyperthyroid)

HF tx (ACEi, Beta blockers, aldosterone antagonists, resynch therapy

ICD for arrhythmia prevention

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19
Q

Dx criteria for HCM

A

LV thickness >15 mm
fam history or genetic mutation
exclude things like HTN

20
Q

HCM during systole

A

usually normal contractility until late, lower chamber volume w/ outflow obstruction

21
Q

cause of HCM outflow obstruction

A

acceleration of flow through narrowed track brings anterior leaflet near the septum, Systolic anterior motion of the leaflet obstructs

limits CO, can lead to MR

22
Q

changes in HCM outflow obstruction

A

anything that reduces ventricular volume will worsen the obstruction

23
Q

Sx of HCM

A

dyspnea, angina, palpitations, sudden death

24
Q

HCM and HF

A

decreased compliance leads to less filling and HF, dyspnea from increased atrial and pulmonary pressures- pulmonary edema

25
HCM and angina
increased demand from increased muscle mass less supply from compressed coronary microvasculature
26
HCM and syncope
can be from arrhythmias or insufficient CO (outflow obstruction)
27
sudden cardiac death and HCM
from arrythmias (fibrosis, disarrayed myocytes) increased risk w/ fam history, syncope history, wall thickness >300 prevented w/ ICD
28
physical exam of HCM
S4 gallop, outflow murmur w/ outflow obstruction, could have MR also for same reason
29
differentiating AS and HCM murmurs
AS is fixed obstruction, HCM is dynamic increase in preload (squatting) increases AS murmur decrease in preload (valsalva) increases HCM
30
HCM on EKG
LVH and T wave inversions
31
gold standard for HCM dx
echo
32
use of MRI w/ HCM
can tell how much fibrosis, this predicts severity and arrhythmia risk
33
medical tx of HCM
mainly targeting angina- reduce demand w/ beta blockers (first line) and CCBs
34
prevention of SCD
limit exercise, no sports ICD with multiple risk factors
35
procedural tx for HCM
myectomy- gold standard (remove myocardium) and alcohol septal ablation (controlled MI)
36
primary and late manifestation of ICM
primary is increase in diastolic ventricular pressure, causes venous congestion and Right HF sx later is a decrease in CO
37
echo for ICM
marked hypertrophy w/ very large atria
38
EKG of ICM
low voltage (despite echo hypertrophy) and pseudoinfarct Q waves
39
clinical manifestations of amyloid ICM
severe diastolic dysfn, heart block/arrhythmias, postural hypertension, stroke
40
tx for amyloid ICM
bone marrow transplant if AL, heart transplant if hereditary standard HF drugs not effective
41
define sarcoidosis
granulomatous disorder w/ multiple organs (typically lymph nodes and lungs) w/ unknown etiology
42
clinical manifestations of sarcoid ICM
asymptomatic, conduction system disease, ventricular arrythmias, systolic HF
43
Tx for sarcoid ICM
glucocorticoids, HF therapies, pacemaker/ICD, transplant
44
cause of arrhythmogenic RV cardiomyopathy
fibrofatty infiltration of RV causing ventricular arrhythmias
45
clinical manifestations of ARVD
arrhythmias and SCD
46
Tx of ARVD
no athletics, ICD when high risk