Cardiomyopathy

Question 1

what is ischemic cardiomyopathy

Answer 1

HF following severe CAD, not a true cardiomyopathy

Question 2

describe the dysfn in DCM

Answer 2

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

Question 3

some causes of DCM (6)

Answer 3

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

Question 4

pathophys of genetic DCM

Answer 4

mostly autosomal dominant, usually cytoskeleton protein abnormalities

Question 5

pathology of DCM

Answer 5

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

Question 6

histo of DCM

Answer 6

myocyte hypertrophy, interstitial fibrosis, wavy fibers

Question 7

characteristics of hypertrophic cardiomyopathy (HCM)

Answer 7

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

Question 8

etiology of HCM

Answer 8

genetic usually AD w/ variable penetrance and expression

prognosis depends on mutation

Question 9

patho of HCM

Answer 9

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

Question 10

histo of HCM

Answer 10

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

Question 11

describe infiltrative cardiomyopathy

Answer 11

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

Question 12

describe the amyloid subtype of ICM

Answer 12

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

Question 13

hemochromatosis subtype of ICM

Answer 13

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

Question 14

what is endocardial fibroelastosis

Answer 14

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

Question 15

what viruses are involved w/ myocarditis and thus DCM

Answer 15

coxsackievirus B, parvovirus B19, echovirus, HIV

Question 16

clinical manifestations of DCM

Answer 16

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

Question 17

Dx of DCM

Answer 17

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

Question 18

Tx of DCM

Answer 18

underlying causes- tachy, EtOH, hyperthyroid)

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

ICD for arrhythmia prevention

Question 19

Dx criteria for HCM

Answer 19

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

Question 20

HCM during systole

Answer 20

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

Question 21

cause of HCM outflow obstruction

Answer 21

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

Question 22

changes in HCM outflow obstruction

Answer 22

anything that reduces ventricular volume will worsen the obstruction

Question 23

Sx of HCM

Answer 23

dyspnea, angina, palpitations, sudden death

Question 24

HCM and HF

Answer 24

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

Question 25

HCM and angina

Answer 25

increased demand from increased muscle mass less supply from compressed coronary microvasculature

Question 26

HCM and syncope

Answer 26

can be from arrhythmias or insufficient CO (outflow obstruction)

Question 27

sudden cardiac death and HCM

Answer 27

from arrythmias (fibrosis, disarrayed myocytes) increased risk w/ fam history, syncope history, wall thickness >300 prevented w/ ICD

Question 28

physical exam of HCM

Answer 28

S4 gallop, outflow murmur w/ outflow obstruction, could have MR also for same reason

Question 29

differentiating AS and HCM murmurs

Answer 29

AS is fixed obstruction, HCM is dynamic increase in preload (squatting) increases AS murmur decrease in preload (valsalva) increases HCM

Question 30

HCM on EKG

Answer 30

LVH and T wave inversions

Question 31

gold standard for HCM dx

Answer 31

echo

Question 32

use of MRI w/ HCM

Answer 32

can tell how much fibrosis, this predicts severity and arrhythmia risk

Question 33

medical tx of HCM

Answer 33

mainly targeting angina- reduce demand w/ beta blockers (first line) and CCBs

Question 34

prevention of SCD

Answer 34

limit exercise, no sports ICD with multiple risk factors

Question 35

procedural tx for HCM

Answer 35

myectomy- gold standard (remove myocardium) and alcohol septal ablation (controlled MI)

Question 36

primary and late manifestation of ICM

Answer 36

primary is increase in diastolic ventricular pressure, causes venous congestion and Right HF sx later is a decrease in CO

Question 37

echo for ICM

Answer 37

marked hypertrophy w/ very large atria

Question 38

EKG of ICM

Answer 38

low voltage (despite echo hypertrophy) and pseudoinfarct Q waves

Question 39

clinical manifestations of amyloid ICM

Answer 39

severe diastolic dysfn, heart block/arrhythmias, postural hypertension, stroke

Question 40

tx for amyloid ICM

Answer 40

bone marrow transplant if AL, heart transplant if hereditary standard HF drugs not effective

Question 41

define sarcoidosis

Answer 41

granulomatous disorder w/ multiple organs (typically lymph nodes and lungs) w/ unknown etiology

Question 42

clinical manifestations of sarcoid ICM

Answer 42

asymptomatic, conduction system disease, ventricular arrythmias, systolic HF

Question 43

Tx for sarcoid ICM

Answer 43

glucocorticoids, HF therapies, pacemaker/ICD, transplant

Question 44

cause of arrhythmogenic RV cardiomyopathy

Answer 44

fibrofatty infiltration of RV causing ventricular arrhythmias

Question 45

clinical manifestations of ARVD

Answer 45

arrhythmias and SCD

Question 46

Tx of ARVD

Answer 46

no athletics, ICD when high risk