Cardiology: Cardiomyopathy

Front 1

Define Heart failure

Back 1

The heart is unable to supply the needs of tissues or is able to do so only at high filling pressures.

Front 2

4 General categories of things that can malfunction and cause HF

Back 2

Valves
Pericardium
Myocardium
Vessels/Blood

Front 3

CO is a product of

Back 3

HR and SV

Front 4

4 things that influence CO

Back 4

Preload
Afterload (BP)
Wall stress
Contractility

Front 5

In patients with a ___1___ CM, ___2___ function is impaired meaning that contractility is poor and the body responds by increasing BP (afterload) by secreting ___3____. This is ca improve perfusion, but worsens the HF.

Back 5

1) Dilated
2) systolic
3) Catecholamines (NE)

Front 6

1) 3 Hormones the body secretes to increase arterial BP (Afterload) when the heart is failing.
2) In practice, how do we counteract this pharmacologically to reduce the afterload stress on the heart?

Back 6

1)NE
AII
Endothelin

2) ACE inhibitors

Front 7

1) The heart (Laplace's law) compensates for increased ___1___ by increasing wall thickness in order to preserve ___2___.

Back 7

1) radius
2) wall stress

Front 8

Name the three GENERAL mechanisms that the heart uses to maintain CO in failure.

Back 8

Frank-Starling
Neurohumoral system
Cardiac Remodeling

Front 9

Starling compensation for ___1____ heart dysfunction worsens the problems because it increases already pathologically high ___2____. This can cause ___3_____ in the lungs.

Back 9

1) Systolic
2) P and V in the ventricle
3) Pulmonary edema.

Front 10

Neurohumoral response to heart failure:
Name as many mediators that are increased in systemic circulation by HF.

Back 10

NE (low in heart)
Renin
AII
Aldosterone
ADH/AVP
TNF
IL6
IL1
Endothelin
ANP (in heart too)
BNP (in heart too)
CNP (not in hear, just systemically)

Front 11

1) Name 4 effects of NE which is elevated in HF.

2) These things can predispose to what complication?

Back 11

1) Systemic vasoconstriction
Increase HR
Stimulates myocardial contractility
Renin Release
(myocyte death)

2) Arrythmia

Front 12

1) In HF, what is the level of NE in the heart? Why?
2) BEta1 receptors? Why?
3) How does (2) affect response to an inotrope like dobutamine?
4) How can we counteract this effect?

Back 12

1) It is low because of exhaustion due to prolonged adrenergic stimulation

2) Low, due to increased stimulation

3) Blunted, this is not good because we use dobutamine to support HF.

4) Beta-blocker increases the receptor level.

Front 13

HF causes renin release, which leads to AII, which ehances release of __1___ and directly causes release of __2__.
3) __2___ worsens HF by two mechanisms. Name them.

Back 13

1) NE
2) Aldosterone
3) Na+/volume retention
Cardiac myocyte remodeling

Front 14

1) AVP is increased or decreased in HF?

2) besides water retention, how else does it worsen HF?

Back 14

1) increased

2) Vasoconstriction

Front 15

1) Elevation of systemic or myocardial levels of these two compensatory, and adaptive proetins are a good marker for HF and Volume overload.
2) What is their direct effect?
3) what other actors do they supress?

Back 15

1) ANP
BNP

2) natriuresis in in response to stretch

3)AII, ADH/AVP, aldo, renin (all the ones in the renal axis)

Front 16

Cytokines of HF- What do they do in general?

(TNF, IL1B, IL6)

2) What is a specific action of TNF?

Back 16

1) Regulate myocyte growth--> hypertrophy

2) Immediate myocyte dysfunction

Front 17

What are the negative sequelae directly on the heart of myocardial hypertrophy.

Back 17

Mismatch of blood supply (low capp. density)
This can lead to ischemia or necrosis.

Front 18

1) Dilated CM causes ___1__ dysfunction. Extra sound?
2) Hypertrophic CM causes ___2__ dysfunction. extra sound?
3) Restrictive CM causes ___3__ dysfunction

Back 18

1) systolic- S3
2) diastolic- S4
3) Diastolic

Front 19

Ischemia causes diastolic or systolic dysfunction or both? Explain the pathophysiology.

Back 19

both
Diastolic- fibrous scar, diastole is active process
Systolic- myocardium loss

Front 20

Random extra two cardiomyopathies
1) Important in kids
2) Catchall

Back 20

1) Arrythmogenic RV Cardiomyopathy- ARVD
(FIbro-fatty change in RV)

2) Unclassafied

Front 21

Forward failure is caused by LV ______ dysfunction

(forward or backward)

Back 21

Forward

Front 22

Hypertensive CM is a type of ___1___ CM.

Back 22

Hypertrophic

Front 23

Hypertrophic CM is caused by RV or LV ________ failure.

(forward of backward)

Back 23

backward

Front 24

Rx of choice for a pure diastolic dysfunction (pure hypertrophic)

Back 24

Diuretic

Front 25

Describe the changes in P-V loop in systolic and diastolic dysfunctional HF.

Back 25

1) systolic dysfunction is due to down-right shift of the end systolic P-V relation (straight line at the top). This is reduced conrtactility

2)Diastolic dysfunction is due to the up -left movement of the end-diastolic passive P-V relation. This means increased stiffness or decreased compliance.

Front 26

1)__1___ is the biggest cause of both systolic and diastolic HF.
2) HTN, DM, and AS are a bigger cause of systolic or diastolic dysfunction
3) alcohol are a bigger cause of systolic or diastolic dysfunction

Back 26

1) CAD
2) Diastolic (Hypertophy)
3) Systolic (Dilated)

Front 27

1) Dyspnea, Orthopnea, and PND are symptoms of ___-sided, ___ward HF.

2) RUQ tenderness, Reduced appetite, Edema are symptoms of ___sided, ___ward HF.
3) Weakness and fatigue are symptoms of ___sided, ___ward HF.

4) What are the symptoms of Left sided Backward HF?
5) What are the symptoms of backward Right sided HF?

6) Forward HF?

Back 27

1) Left sided, backward
2) RIght sided backward
3) Left sided forward

4) Tachypnea, Lung crackles
5) JVD, Hepatomegaly, Edema
6) Decreased mentation
Cool extremeities
Pulsus alternans
Cheyne-stokes respirations

Front 28

What is indicated by a loud pulmonary valve closure? What might cause this?

Back 28

Pulmomary HTN
(maybe due to backward left sided HF)

Front 29

Three possible causes of idiopathic DCM?

Back 29

familial
viral myocarditis
Immunological abnormalities

Front 30

DCM natural history
25% die in __1____ year(s)
50% dies in ____2__ year(s)

Back 30

1) 1
2) 5

Front 31

Thigns that predict poor prognosis in DCM?

Back 31

degree of ventricular enlargement
limited exercise capacity
rduced maximal O2 uptake

Front 32

Two causes of DCM that we might be able to fix without heart transplant?

Back 32

alcohol
CAD

Front 33

__1____ should be performed in any patient with suspected HF

2) Once the diagnosis of DCm has been made, what two test should be give to look for CAD?

3) what will be the finding on CXR?

4) ECG findings that may be present,(but needn't be).

Back 33

1) echo
2) stress test or cardiac cath
3) enlarged heart with or without fluid in the lungs


4) poor R wave progression, LBBB

Front 34

1) Two three- letter acronyms that characterize bad HCM?

2) the second one is caused by the ____effect

Back 34

2) ASH- assymetrical septal hypertrophy
SAM- systolic anterior movement of the anterior leaflet of the mitral valve.

2) venturi

Front 35

in HCM with LVOT, the LV pressure is much __1___ than the aortic pressure.
This can be quantified by using ___2___.

Back 35

1) higher
2) Doppler

Front 36

1) SAM caused by HTM can cause ___2___. If this is severe, what symptom may be felt.

3) describe the heart sound that would be heard (in total)

Back 36

1) MR
2) Dyspnea.
3) Late systolic myrmur (MR)
maybe a holosystolic murmur
from the LVOT
Probably an S4

Front 37

How does HCM lead to myocardial ischemia (4 ways)

Back 37

Hypertrophy of coronary vessels leading to imparment in dilation

inadequate capp. density for the muscle.

Increased demand

Compression of coronaries during strong contraction

Front 38

1) Inheritance pattern of HCM
2) most common

Back 38

1) AD
2) Beta myosin heavy chain

Front 39

histology of HCM

Back 39

disorganized muscle bundles leading to a whorled appearance

fibrosis and necrosis

Thickening of coronary arteries

Front 40

1) Most Common symptom of HCM

2) other common ones

Back 40

1) dyspnea
2) angina, fatigue, syncope, palpitations, sudden death

Front 41

Two physical (no tools) findings in HCM?

Back 41

strong apical impulse
Strong carotid pulse

Front 42

1) Describe how the holosystolic ejection murmur from LVOT can change with postion?

2) Where is it best heard?

Back 42

1) Increases with valsalva, standing (anything that makes ventricle smaller)

Decreases with squatting or raising the legs (anything that increases ventricular size)

2) between apex and Left sternal border

Front 43

HCM findings on
1) ECG
2) Echo
3) Doppler

Back 43

1) increased voltage, ST and T-wave changes, maybe Q-waves
2) LV thickness maybe with ASK
3) Gradient of P across the aortic valve (low on aorta side)

Front 44

1) Prognosis of HCM in general population (% death yearly)
2) Predictors of poor prognosis

Back 44

1) 1% death yearly
2) abnormal BP response to exercise, syncope, marked hypertrophy

Front 45

Management of HCM
1) to relieve pulmonary congestion
2) to cause decreased contractility
3) to caused increased diastolic filling
4) to depress LV contractility

Back 45

1) Diuretics to reduce pulmonary congestion
2) Beta blockers to relieve LVOT bly blocking contractility
3) Calcium channel blockers (Verapamil specifically) to improve diastolic filling except in people with HF
4) Disopyramide (1-A) to depress LV contractility.

Front 46

5 invasive options for HCM

Back 46

Dual chamber pacer
ICD
Alcohol septal ablation
Myomyectomy
MVR (to relieve SAM)

Front 47

Important causes of Restrictive CM

Back 47

Amyloid
Sarcoid
Gauchers
Fatty infiltration
Hemochromatosis
Fabry
Glycogen storage diseases
Carcinoid
Radiation
Familial
Scleroderma
Diabetic CM

Front 48

1) Initial presentation of RCM
2) After progression, presentation

Back 48

1) dyspnea on exertion, weakness
2) Ascites, Hepatomegaly, edema (anasarca)

Front 49

Findings in RCM:
1) Echo
2) doppler
3) ECG

Back 49

1) normal systolic function
2) diastolic dysfunction
3) Diffusely diminished voltage

Front 50

1) Characteristic sign of RCM and pericardial constriction on a time-pressure tracing.

2) explain why this sign happens

Back 50

1) Square root sign
2) early diastolic filling abruptly halts as the volume in the ventricles quickly reaches its limits imposed by the constrcited pericardium.

Front 51

Three diagnostic tests we use to search for an etiology for RCM

Back 51

fat pad aspirate for amyloid
iron studies fo hemochromatosis
Endomyocardial biopsy

Front 52

Tx of RCM
1) in general
2) if amyloid
3) if hemochromatosis

Back 52

1) diuretics
2) DNA alklating agents
3) Desferroximine and phlebotomy

Front 53

Important causes of constrictive pericarditis

Back 53

idiopathic
infection
post surgical
trauma
radiation
neoplastic
uremia
Connective tissue disorders
Procainamide
hydralazine

Front 54

Constrictive pericarditis presents like ____ failure even though they have not failed

Back 54

ventricular

Front 55

Diagnostic sign seen on exam of constrictive pericarditis (proably in RCM too)

Back 55

Kussmauls sign (inspiratory increase in JVD)

Front 56

Constrictive pericarditis CXR appearance

Back 56

can sometimes see calcification of pericardium in lateral view

Front 57

Findings in constrictive pericarditis
1) Echo
2) Ct or MRI
3) management?

Back 57

1) normal systolic function, dilated atria. cannot see pericardium
2) thick pericardium on either
3) Diuretics or Pericardial stripping

Front 58

Given a sign, tell whether is is present in RCM, constrictive pericarditis, or both. (some answers are "maybe present")

1) S3
2) pericardial knock
3) Kussmaul's sign
4) HF
5) Thick ventricles
6) Valvular Regurg
7)Atrial enlargement
8) Atrial pressures
9) Ventricular pressure (Square root)
10) Pulmonary pressure (numerical)
11)Pericardial thickening (CT or MRI)
12) Biopsy

Back 58

1) absent in PC
Present in RCM
2) Present in PC
absent in RCM
3) present in PC
may be present in RCM
4) present in both
5) absent in PC
may be present in RCM
6) absent in PC
Present in RCM
7) present in both
8) elevated in both
9) square root in both
10) 35-40 mmHg in PC
>50 mmHg in RCM
11) thick in CP
normal in RCM
12) normal in CP
infiltrativ process in RCM

Front 59

5 steps in evaluating a pt. with HF

Back 59

H & P
ECG
CXR
Echo
CT or MRI if indicated