lesson 1 cardiomyopathies

WHAT IS CARDIOMYOPATHY? Affects what ages’s of population? is there a cure for CM? what dysfuncyion is assocatied with CM? All CM will lead to what?

Disease of the heart muscle, specifically of the myocardium

Affects any age population

There is no cure but good treatments options available

Both systolic and diastolic dysfunction can be affected

All CM can lead to HF

what is the etiology (the cause of the disease) for cardiomopathy? and what its origin?

Idiopathic (without an identitiy, we dont know what caused it and not a result of another disease)

(there can be both genetic and nongetic origin)

Nongenetic Origin ( can from bio effections, automine disease, expsoure to toxins, pregrancy’s?) - secoundary causes

Familial Transmission, name 3

Autosomal Dominant

Autosomal Recessive

Sex linked

familial transmission:

DEFINE AUTOSOMAL DOMINANT

An individual will only need to get an ………… …………from …………parent to ……….the ………..

what is the % chance of the child getting the ………

An individual will only need to get an abnormal gene from ONE parent to inherit the disease.

(50% chance of the child getting the gene)

familial transmission:

Define AUTOSOMAL RECESSIVE TRAIT

an individual born to who what? Has a what chance of getting the malfunctioning genes from ___ _______ and developing the disease?

An individual has a ___in ____ chance of inheriting one abnormal gene

An individual born to parents who BOTH carry an autosomal recessive disease

change (mutation) has a 1 in 4 chance of getting the malfunctioning genes from both

parents and developing the disease

An individual has a 2 in 4 chance of inheriting one abnormal gene

• 1 out of 4 children (25%)
  → can inherit BOTH abnormal genes
  → and have the disease

• 2 out of 4 children (50%)
  → inherit only ONE abnormal gene
  → they are just carriers
  → they usually do NOT have the disease

• 1 out of 4 children (25%)
  → inherit NO abnormal genes
  → completely normal

Describe the SEX LINKED : X-LINKED (DOMINANT) for the father?

The Father: (X+)(Y)

The father with the X+ will always give the disease to the daughters and never to the son

Describe the SEX LINKED : X-LINKED (DOMINANT) for the mother?

The Mother: (X+)(X)

The mother with the X+ will have a 50/50 chance for all children

• 50% chance she passes the normal X

• 50% chance she passes the abnormal X+

That means:

EACH child has a 50% chance of getting the abnormal gene.

BOTH sons and daughters each have a 50% chance of inheriting the abnormal X from the mother.

each pregnancy has a 50% probability.

what are the 4 WORLD HEALTH ORGANIZATION: WHO 1996 CLASSIFICATION?

and name another 2

and please name for the 4 what AMERICAN HEART ASSOCIATION: AHA’S CLASSIFICATION OF

CARDIOMYOPATHY:

1. Dilated Cardiomyopathy

this is a primary cardiomyopathies (predominantly involving the heart) MIXED

1. Hypertrophic cardiomyopathy

this is a primary cardiomyopathies (predominantly involving the heart) GENETIC

1. Restrictive cardiomyopathy

this is a primary cardiomyopathies (predominantly involving the heart) MIXED (non-hyperthrophied and non - dilated)

1. Arrhythmogenic Right Ventricular Cardiomyopathy (ARVD/C)

this is a primary cardiomyopathies (predominantly involving the heart) GENETIC

5. Unclassified cardiomyopathy

6. Specific cardiomyopathy

Unclassified cardiomyopathy… name 4

• Fibroelastosis •

Noncompacted myocardium (LVNC) •

Systolic dysfunction with minimal dilatation •

Mitochondrial involvement

Specific cardiomyopathy….. name 10

Ischemic cardiomyopathy •

Valvular Cardiomyopathy •

Hypertensive cardiomyopathy •

Inflammatory/Infective Cardiomyopathy •

Metabolic Cardiomyopathy •

General system disease •

Muscular dystrophies •

Neuromuscular disorders •

Toxic •

Peripartal

AMERICAN HEART ASSOCIATION: AHA’S CLASSIFICATION OF

CARDIOMYOPATHY, how does it classifiy DCM?

Primary cardiomyopathies , Mixed (can be both genetic and acquired)

what are the HEMODYNAMICS IN CARDIOMYOPATHY?

The study of forces involved in the circulation of blood •

Systolic • Diastolic

explain the HEMODYNAMICS: SYSTOLIC FUNCTION

• Contraction of the heart muscle •

Systole: The time the heart contracts

explain the HEMODYNAMICS: DIASTOLIC FUNCTION

Relaxation of the heart muscle •

Diastole: The period of relaxation of the heart muscle

explain preload

the degree that …………………….prior to

The degree that myocardial fiber is stretched prior to contraction

Preload, the ventricular wall tension at what cardiac cycle? And what is it Approximated by?

The ventricular wall tension at the end of diastole •

Approximated by the end-diastolic volume (EDV) or end-diastolic pressure (EDP)

This sentence is talking about preload. Your slide says preload is:

“the degree that myocardial fiber is stretched prior to contraction” and it is “approximated by the end-diastolic volume (EDV) or end-diastolic pressure (EDP).”

Here’s the simple version:

What is preload?

Preload basically means:

How full the ventricle is before it squeezes

or

How stretched the heart muscle is before contraction

---

Think of a Balloon

The more water you put into a balloon:

• the more it stretches

The ventricle works similarly:

• more blood filling it
  = more stretch
  = higher preload

---

What is End-Diastolic Volume (EDV)?

Diastole = filling phase of the heart.

So:

• EDV = the amount of blood in the ventricle RIGHT BEFORE it contracts.

Bigger EDV:

• more blood in ventricle

• more stretch

• more preload

---

What is End-Diastolic Pressure (EDP)?

As the ventricle fills with blood:

• pressure inside rises

So:

• EDP = pressure in the ventricle at the END of filling.

Higher EDP usually means:

• the ventricle is fuller

• or stiffer

• and preload is higher

---

What does “approximated by” mean?

It means:

We cannot directly measure preload easily.

So doctors/sonographers use:

• EDV
  or

• EDP

to ESTIMATE preload.

---

Super Simple Memory Trick Preload =

“How much blood is in the ventricle before squeeze”

EDV =

“How much blood volume is there?”

EDP =

“How much pressure is there from filling?”

explain afterload

Resistance the ventricle must overcome to empty its contents

Afterload, Ventricular wall stress (pressure) that develops during what cardiac cycle?

Ventricular wall stress (pressure) that develops during systolic ejection

define Stroke Volume and the equation

Stroke Volume – the volume of blood pumped by the left ventricle of the heart in one contraction

• SV= EDV – ESV

define cardiac output and the equation

Cardiac Output- the volume of blood that is pumped by the heart each minute • CO = SV x HR

define contactility

Force generated by the myocardium when muscle fibers shorten

When contractility increases what happens with cardiac output?

When contractility increases, cardiac output increases

When contractility decreases, what happens with cardiac output?

When contractility decreases, cardiac output decreases

define HEART FAILURE and the affects it causes to the heart?

The inability of the heart to meet the metabolic demands of the body

Via systolic or diastolic dysfunction, and a decrease in CO

can you explain the steps for the Cardiac Performance?

what are the 4 PRIMARY CARDIOMYOPATHIES?

Dilated Cardiomyopathy

Hypertrophic Cardiomyopathy

Restrictive Cardiomyopathy

Arrhythmogenic Right Ventricular Cardiomyopathy

what is DILATED CARDIOMYOPATHY? what does it cause?

Dilation of the LV or RV

Causes systolic dysfunction and dilated ventricle

HYPERTROPHIC CARDIOMYOPATHY, what is it? (hint its disproportionate/abnormal….)

what dysfunction is this?

can be what or non?

what is the etiology of this? name 2

Disproportionate/abnormal hypertrophy of the LV and RV •

Diastolic Dysfunction •

Can be obstructive or non obstructive •

Idiopathic hypertrophic subaortic stenosis via muscular subaortic stenosis

RESTRICITVE CARDIOMYOPATHY, define what this is ?

what does it restricts ?

what dysfunction is this?

Endomyocardial scarring affecting the LV and or the RV •

Restricts ventricular diastolic filling •

Marked diastolic dysfunction

(walls become stiff - not thick, have a hard time relaxig and filling ) (grade 3 diastolic dysfinction with restrictve CM)

(common to have a big atria on both the right and left side)

ARRHYTHMOGENIC (ARVD/C) CARDIOMYOPATHY, define what this is & resulting in what two things and what dysfunction?

Fibrofatty replacement of RV tissue resulting in wall thinning and aneurysm formation

RV dilatation and dysfunction

what is ECHO’S ROLE IN CARDIOMYOPATHY? Name 5

1. Identify in patient’s history •

2. History and signs and symptoms •

3. Family history •

4. Identify on echo •

5. Identify what needs to be focused on in a complete echocardiogram for certain cardiomyopathy

what does MOGES stand for?