Pm_Cardiopulm_Intmed_Cardiomyopathies

Front 1

Name for Dxs that involve the muscle of heart causing cardiac mal-fxn. Not the result of Coronary Art. Dx, HTN, Valvular, CONGENTIAL or Pericardial Dx

Back 1

Cardiomyopathies, 4 Cats:

Front 2

What are the 4 Primary types of Cardiac Myopathies?

With each, give the mechanical problem(s) too.
Use:
D = Dilated
R= Resitrictive
H= HYPERtrophic

Back 2

1. Idiopathic (D.R.H.)
2. Familial (D.H.)
3. Eosinophilic Endomyocardial Dx (R)
4. Endomyocardial Fibrosis (R)

Front 3

What sort of Secondary CardioMyopathies do Infections Cause?
Most common type of infection?

Back 3

Infections generally cause Dilated Myopathies
--Usually Viral

Front 4

List a few secondary causes of Cardiomyopathies

Back 4

-Connective Tissue Dx
-Infiltrative--(Fibrils w/ Amyloidosis; sarcoidosis w/ non-caseating granules})
-Storage Dxs
-Muscular Dystrophies
-Friedrichs Ataxia
-Peri-Partum (up to 6mos post)
-Alcohol
-----don't need to know all these

Front 5

1. Dilated CMyoPs
How/Which Ventricle affected?
Effect on:
-Systolic Fxn?
-EF? CO?
Arrhythmogenic?
Embologenic?

Back 5

Dilated:
Enlargement: Left &/or Right Ventricle
-Sys Fxn is impaired, will ahve Congested HF
--Low EF, Low CO
---Sitting ducks for Arhytmias and Clots

Front 6

What Primary CMyoP involves the Septum more than the Walls?
-w/ or w/o Intraventricular Systolic P gradient
-LV Cavity usually Not dilated

Back 6

This is Hypertrophic CMyoP
--May have ventricular mm. hypertrophy, but Condition is due to Disproportionately HYPERtrophic Septum blockingin outflow

Front 7

What CMyoP involves Scaring or Myocardial Fibrosis?
-Problem with Filling/Diastole of Left or Right Ventricle

Back 7

This is a Restrictive CMyoP
--Relaxing Problem.
--Decrease in Distensibilty/Compliance with INCREASe in Stiffness

Front 8

Genetically Prone Teens/Young Adults die from this during Exercise due to Ventricular Arrhythmias due to What Type of CMyoP

Back 8

-Hypertrophic CMyoP
--Huge septum
----causes problems with outflow to aorta

--can occur much later in life too

Front 9

Familial Problem
-Right Ventricle literally Replaced with Fibrile/Fatty Tissue in RV.

Die of Arrhytmogenic Death?
Called

Back 9

Arrthymogenic Right Ventricular Cardiomyopathy

Front 10

Women 50-60 with CMyoP due to Adrenergic Surge of catecholamines

Back 10

Takosubo CMyoP
--Broken Heart Syndrome

--Have Normal Coronary Arts, but present with Angina and abnormal ECG.

Front 11

In a Dilated Type CMyoP, are the ventricles Hypertrophied?

Back 11

Can Be. may be normal.
Will have barral shaped LV

Front 12

What is a SAM?
With which type of CMyoP do you see it

Back 12

SAM= Systolic Anterior Motion o Mitral leaflet (Anterior).
---Seen with Hypertrophic Type.
--Exacerbates this Obstructive CMyoP.
-----Not all H's have this, but many will

Front 13

With Systolic Dyxn, as Dilated Cardiomyopathies Mostly are (can be diastolic, unusual though), there is Low EF/CO. What is Increased in Dilated CMyoPs

Back 13

Left Vent. End Diastolic Pressure
LVEDP is Increased in Dilated

--more often in Middle aged men, Blacks

Front 14

What sort of Leaks are associated with Dilated CMyoP?
Lung Sounds?
How will pulse pressure be?

Back 14

Mitral Regurge
(tricuspid too, can be RV dysfxn)
-Will auscultate crackles/ peripheral edema/hepatomegalia
--Narrow pulse pressure

Front 15

Describe the following Presures with Dilated CMyoP
LVEDP?
PCWP (wedge)
LA Pres?
L & R sided Filling P?
CO?

Back 15

All UP

CO is down

Front 16

What are Non-Pharma Tx for Dilated CMyoP
--Pharma?

Back 16

Non-Pharmacologic
--No alcohol. Isotonic Exercise. Na and Fluid Restriction--particullary with weight gain/edema
--Dig/Inotrophics
-BetasBlockers
-Alpha Rec Blockers/Vasodilators
-ACEI or ARB
-Aldosterone Antags
-Diuretics if crackles/wet/edematous
-NO (N+H) to decrease (venodilators) Preload

Front 17

Which Beta blockers are especially usefull given studies on Class I, II and III
(not on Class IV/Decompensates)

Back 17

Carvedilol, Metoprolol

Front 18

What is 1yr Survival Rate for Transplant with CMyoP.

With regular (non-transplant) Tx of Dilated CMyoP, what % die in 1 yr

Back 18

90% 1yr

50% die 1year due to Arrhythmias (emboli etc)

Front 19

Alcohol causes what type of Cardiomyopathy?

Back 19

Dilated
--Less than 25% survive 3 yrs due to Alcy/ Holiday Heart Syndrom

Front 20

Global Hypokinesis is associated with what CMyoP?

Back 20

Dilated CardioMyops
--remember MRegurg,
causes
-->>Left Atrial enlargement

Front 21

In a Dilated Type CMyoP, are the ventricles Hypertrophied?

Back 21

Can Be. may be normal.
Will have barral shaped LV

Front 22

What is a SAM?
With which type of CMyoP do you see it

Back 22

SAM= Systolic Anterior Motion o Mitral leaflet (Anterior).
---Seen with Hypertrophic Type.
--Exacerbates this Obstructive CMyoP.
-----Not all H's have this, but many will

Front 23

With Systolic Dyxn, as Dilated Cardiomyopathies Mostly are (can be diastolic, unusual though), there is Low EF/CO. What is Increased in Dilated CMyoPs

Back 23

Left Vent. End Diastolic Pressure
LVEDP is Increased in Dilated

--more often in Middle aged men, Blacks

Front 24

What sort of Leaks are associated with Dilated CMyoP?
Lung Sounds?
How will pulse pressure be?

Back 24

Mitral Regurge
(tricuspid too, can be RV dysfxn)
-Will auscultate crackles/ peripheral edema/hepatomegalia
--Narrow pulse pressure

Front 25

Describe the following Presures with Dilated CMyoP
LVEDP?
PCWP (wedge)
LA Pres?
L & R sided Filling P?
CO?

Back 25

All UP

CO is down

Front 26

What are Non-Pharma Tx for Dilated CMyoP
--Pharma?

Back 26

Non-Pharmacologic
--No alcohol. Isotonic Exercise. Na and Fluid Restriction--particullary with weight gain/edema
--Dig/Inotrophics
-BetasBlockers
-Alpha Rec Blockers/Vasodilators
-ACEI or ARB
-Aldosterone Antags
-Diuretics if crackles/wet/edematous
-NO (N+H) to decrease (venodilators) Preload

Front 27

Which Beta blockers are especially usefull given studies on Class I, II and III
(not on Class IV/Decompensates)

Back 27

Carvedilol, Metoprolol

Front 28

What is 1yr Survival Rate for Transplant with CMyoP.

With regular (non-transplant) Tx of Dilated CMyoP, what % die in 1 yr

Back 28

90% 1yr

50% die 1year due to Arrhythmias (emboli etc)

Front 29

Alcohol causes what type of Cardiomyopathy?

Back 29

Dilated
--Less than 25% survive 3 yrs due to Alcy/ Holiday Heart Syndrom

Front 30

Global Hypokinesis is associated with what CMyoP?

Back 30

Dilated CardioMyops
--remember MRegurg,
causes
-->>Left Atrial enlargement

Front 31

Multiparous, Afro-Americans, older than 30yrs are at risk for what if pregnant

Back 31

Peripartum Cardiomyopathy

--also if eclampsia

Front 32

Medicines that may cause Cardiomyopathies.

Back 32

--Anthracycline derivatives--Doxorubicin (adriamyocin)= (D)
-5 FluoroUracil
-Lithium
-Coke

Front 33

A defect of myocyte Proteins without obvious cause (ie, HTN or Aortic Stenosis) may be indicative of what?

Back 33

Hypertrophic CMyoP
-LV Hypertrophy perhaps too
-SUBAortic Stenois--many names for same thing, too big a septum below Outflow tract of LV

Front 34

With Disorganized Cardiac Muscle Cells, think what?

Back 34

Hypertrophic CMyoP
--LVOT due to Septum

----60-80& have this Subaortic Stenosis as a consequence of SAM of AnteriorMV

Front 35

How does degree of Obstruction with (H) CMyoP progress

Back 35

LVOT Obstruction INCREASES with
-Inc. Contractility
-Decreased Preload & Afterload.
--INC LVED

--Think, as this is counterintuitive

Front 36

A glycogen storage Dx (LAMP in Lec) affecting the heart may be what?
What type of CardioMyop

Back 36

Danon's Disease
-X-linked and causes
Hypertrophic CMyoP

Front 37

Syncope, Dyspnea and Chest Pain are common Sx for which 2 Dx? One of which is CMyoP

Back 37

Aortic Stenosis &
--Hypertrophic CMyoP

Front 38

What are Physical Sigs of LVOT?
--Heart Sounds? Murmur?

Back 38

Carotid Pulse with Bifid (double pulse) quality
--Sustained LV impulse
---Pardoxic S2 Split -- P2, then A2 (usual split is A2 then P2)
--Loud Systolic Murmur

Front 39

How might you exacerbate Sx of Hypertrophic CMyoP
--for diagnosis

Back 39

-Anything that Increases Contractility
--Excercise, Digitalis

Front 40

Which CMyoP has 50% positive familial element

Back 40

Hypertrophic cardiomyopathy
---Remember
if LVOT Obstruction Present--Degree of Obstruction INCREASES as you Decrease PreLoad/Afterload
--or-- Increase Contractility

Front 41

What is Another way to exacerbate Hypertrophic CMyop?

Back 41

Maneuvers that Dec. Ventricular Volume (remem, dec Preload/Afterload--> Inc. Obst)
--Diuretics, Sudden Standing
--(explanation--with dec volume to heart, the LV cavity is less distended, this exacerabtes the obstructive nature of a hypertrophic heart--ie, obstructive cavity just got narrow.

Hint 41

Thus, maneuvers that DEC ventricular Volume Exacerbate Hypertrophic CMyoP. Similarly, Maneuvers that INC. CONTRactility (exercise/Dig) will also Exac it.

Front 42

What are 3 observable effects of Exacerbating (H) CMyoP due to increased Contractility OR Dec Ventricular Volume?

Back 42

Will increase Gradient
will increase Obstruction
will Increase Murmur

Front 43

What Maneuvers relieve Sx of (H) MyoP?

Back 43

ANYthing that INC LV Cavity Size:
Raised BP
Squating (inc Pre/AferLoad
Expand Blood Volume

Front 44

How might Septum appear (in quality) on an Echo with (H) CMYOP

Back 44

Ground glass

Front 45

What are Cardiac Cath Findings for (H) CMyoP for:
LVDP?
Gradient b/w LV & SubAortic?
-Systolic Fxn?

Back 45

LVDP Increased
Large Gradient b/w
Vigorous Sys Fxn

Front 46

What drugs do you NOT use with Hypertrophic CMyoP

Back 46

--NOT Strenuous Phys Activity
Stay away from
Dig/ Inotrophic Stims

Front 47

What drugs do you use with Hypertrophic CMyoP

Back 47

Drugs that Dec. Contractility & Dec Degree of Obst:
-beta blocks
-Amiodarone for arrhythms
-CCBlocerks --with caution
---pacemakers/ shaving of septum in surgery

Front 48

litotes

Back 48

n.
understatement for emphasis.
To say, "He little realizes," when we mean that he does not realize at all, is an example of the kind of understatement we call litotes.

Front 49

This CMyoP is a Relaxing Problem. Problem of R or L Ventricle NOT filling as walls are effed up.
--is this Diastolic or Systolic

Back 49

Restrictive Cardiomyopathy
--A Diastolic Dys
due to Stiffened/Non-Compliant vents

Front 50

What is Cause of Restrictive CMyoP

Back 50

--Unknown.
Myocardial Fibrosis, Hypertrophy, Infiltrations

Front 51

What are the 2 Major Cats of (R) CMyoP?

Back 51

1. Idiopathic - fibrosis of Myocard

2. EMF - Endomyocardial Fibrosis
i. end stage of eosinophilic syndromes
ii. thrombus inLV and Obiteration of Ventricular cavity by fibrous tissue.

Front 52

What are the 2 forms of EMF?

Who are more affected by this

Back 52

1. Active Inflammatory Type
2. Chronic Type -- Tropics

children, young folks more affected with EMFs

Front 53

In this Secondary (R) CMyoP there is:
-Diastolic & Systolic Dysfxn
-Orthostatic HTN (dec Perfusion to head/faint)
-Arrhythmias
-SPECKLED Myocardium on Echo

Back 53

Amyloidosis as 2ndary cause of (R)

Front 54

This Secondary Cause of (R) CMyoP arises from:
--Multiple transfusion Pts
-Hemoglobinopathy
-Familial: D.M., cirrhosis,

Back 54

Hemochromatosis as 2nd Cardiomyopathy of Restrictive Nature

Front 55

This Secondary Cause of (R) CMyoP results in:
Endocardial Fibrosis w/ Stenosis/Regurg of Tricuspid/Pulmonic Valves

Back 55

Carcinoid Synd.

dont' worry about
Other Causes of (R):
Sarcoid
Radiation

Front 56

Loeffler's Endocardititis is aka?

Back 56

Eosinophilic EndoMyocardial Dx
--Develop Large Mural thrombi that cause Fibrosis
--Thus can cause Resistrictive CMyoP

Front 57

Jugular Venous P. RISES with inspiration/ Vein Gets Bigger=

Back 57

Kussmaul's Sign

Normal is when Neck Veins DECREASE with Inhalation

Front 58

In Hypertrophic CMyoP, the QRS is ?

In Restrictive CMyoP, the QRS is?
----NOte: with Sq Root sign??? think what CMyoP

Back 58

Hyper=Big QRS
Dilated, sometimes

REstricitve = Small QRS
---sq. root sign indicate Restrictive