Cardiomyopathies and sutff

Question 1

What is the ventricular morphology of dilated cardiomyopathy?

Answer 1

Dilated LV with little hypertrophy

Question 2

What is the ventricular morphology of arrhythmogenic RVCM?

Answer 2

dilated RV with sections of missing muscle.

Question 3

What is the ventricular morphology of HCM?

Answer 3

Marked hypertrophy.

Question 4

What is the ventricular morphology of RCM?

Answer 4

fibrotic/infiltrated myocardium

Question 5

What are the symptoms of DCM?

Answer 5

Fatigue, weakness, dyspnea, orthopnea, paroxysmal nocturnal dyspnea

Question 6

What are the symptoms of ARVCM?

Answer 6

R heart failure
Palpatations
Syncope
Sudden death

Question 7

What are the symptoms of HCM?

Answer 7

Exertional dyspnea
Angina
Syncope
A-Fib, V-Fib
CHF
Sudden Death (1.5%)

Question 8

What are the symptoms of RCM?

Answer 8

Dyspnea

Fatigue

Question 9

What presents in the physical exam for DCM?

Answer 9

pulmonary rales
s3
functional regurge
possible RV failure signs

Question 10

What presents in the physical exam for ARVCM?

Answer 10

rhythm disturbances (ventricular arrhythmias)

Question 11

What presents in the physical exam for HCM?

Answer 11

S4

Outflow obstruction: systolic murmur and mitral regurgitation

Question 12

What presents in the physical exam for RCM?

Answer 12

Signs of RV failure
JVD
Hepatomegaly
Peripheral edema

Question 13

What is the pathophysiology of DCM?

Answer 13

imparied systolic contraction

mural thrombi is common

Question 14

What is the pathophysiology of ARVCM?

Answer 14

nothing.

Question 15

What is the pathophysiology of HCM?

Answer 15

impaired diastolic relaxation

LV systolic function vigorous with dynamic obstruction of mitral valve during mid-to-late systole

Question 16

What is the pathophysiology of RCM?

Answer 16

“stiff” LV
impaired diastolic relaxation
normal systolic function

Question 17

What is the cardiac size for DCM?

Answer 17

Dilated/enlarged

Question 18

What is the cardiac size for HCM?

Answer 18

normal or dilated

Question 19

What is the cardiac size for RCM?

Answer 19

usually normal

Question 20

What does the echo show for DCM?

Answer 20

dilated, poorly contractile LV

Question 21

What does the echo show for HCM?

Answer 21

LV hypertrophy more pronounced in septum (acquired) or concentric (genetic)

Systolic anterior movement of MV with mitral regurge

Question 22

What does the echo show for RCM?

Answer 22

Usually normal systolic contraction

Speckled appearance in infiltrative disorders

Question 23

What does the histology show for DCM?

Answer 23

Interstitial fibrosis

replacement fibrosis

Question 24

What does the histology show for ARVCM?

Answer 24

myocyte atrophy

fibrosis

fatty tissue

Question 25

What does the histology show for HCM?

Answer 25

increased diameter of myocytes abnormal arteries myofiber disarray

Question 26

What does the histology show for RCM?

Answer 26

interstitial fibrosis

Question 27

What is the pathogenesis for DCM?

Answer 27

- often in 30s and 40s - genetic, usually autosomal dominant - in kids with DCM, often mitochondrial gene mutations - infectious: coxsackie, adenovirus, parvovirus, HIV - iron overload - child birth

Question 28

What is the pathogenesis for ARVCM?

Answer 28

- autosomal dominant with variable penetrance - defective desmosomes > detachment of myocytes at intercalated discs > myocyte death and fibrosis - disease occurs in bursts

Question 29

What is the pathogenesis for HCM?

Answer 29

- heart thickening usually happens in kids - autosomal dominant mutation of sarcomeric genes (B-myosin heavy chain, myosin binding protein C, Troponin T) * *Troponin T is important**

Question 30

What is the pathogenesis for RCM?

Answer 30

- genetics, involving Troponin I and desmin genes - amyloidosis - endomyocardial fibrosis - Loeffler endomyocarditis: hypereosinophilia due to release of granulocytes

Question 31

What is the treatment for ARVCM?

Answer 31

none (80% dies of arrhythmias, 20% die of CHF)

Question 32

What is the treatment for HCM?

Answer 32

- implanted automatic defibrillator - negative inotropic agents - myocardial reduction with surgery or alcohol

Question 33

What is the treatment for RCM?

Answer 33

Can be treated with platelet derived growth factor