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Echo > CM > Flashcards

CM Flashcards

1
Q

What is cardio myopathy?

A

Cardiomyopathy is a disease of the heart muscle that makes it harder for your heart to pump blood to the rest of your body.

*It can lead to heart failure

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2
Q

The main types of CM includes:

A

dilated

hypertrophic

restrictive

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3
Q

Treatment for CM

A

medications

surgically implanted devices

(for severe case) heart transplant

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4
Q

Dilated Cardiomyopathy

Dilated CMO (aka: congestive CMO) is the most common CMO.

T or F ?

A

T

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5
Q

Dilated Cardiomyopathy

Dilated CMO is known for……..

A

multi-chamber enlargement

decreased systolic & diastolic function

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6
Q

Dilated Cardiomyopathy

etiology/causes

A
  • idiopathic
  • hereditary (up to ⅓)
  • valvular disease
  • IHD/CAD/MI
  • long standing systemic HTN
  • arrhythmia
  • infectious in particular viral infections that cause heart inflammation
  • metabolic *thiamine deficiency
  • some chemotherapy agents
  • toxins i.e. lead, mercury, arsenic, cobalt
  • drugs i.e cocaine, amphetamines etc
  • alcohol abuse
  • endocrine disease i.e thyroid disease, diabetes
  • immune system disorder i.e Lupus
  • neuromuscular disorder i.e muscular dystrophy
  • peri-partum (during pregnancy) or post-partum complications

*Thiamine/vitamin B1, is a water-soluble vitamin found naturally in some foods, added to foods, and sold as a supplement. Thiamin plays a vital role in the growth and function of various cells.

*A thiamin deficiency can result in several health problems including confusion, seizures, shortness of breath, brain disease, coma, and more

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7
Q

Dilated Cardiomyopathy

signs & symptoms

A
  • HF & associated findings: dyspnea, orthopnea, pulmonary edema
  • decreased CO & associated findings: tachycardia, fatigue, weakness, hypotension
  • dysrhythmia & associated findings: palpitation, syncope, sudden death
  • regurgitant valves & associated findings: murmurs, hemoptysis, decreased CO
  • ischemia & associated findings: CP
  • thrombus (usually located in LA/LV) & associated findings: systemic embolization, neurological events
  • pericardial effusion (usually small)
  • infective endocarditis (rare)
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8
Q

Dilated Cardiomyopathy

treatment

A
  • no treatment if asymptomatic
  • manage underlying issues
  • treat/control signs & symptoms
  • prevent CMO from progressing
  • reduce risk of sudden death
  • lifestyle adjustments
  • medical therapy
  • implantable cardiovascular defibrillator (ICD)
  • transplant
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9
Q

Dilated Cardiomyopathy

Sometimes dilated CMO comes and goes on its own.

T or F ?

A

T

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10
Q

Dilated Cardiomyopathy

2D echo findings:

A
  • gross dilatation of the heart
  • thin walls
  • spontaneous echo contrast (smoke)
  • possible thrombus primarily at the apex *Interrogate apex!
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11
Q

Dilated Cardiomyopathy

To identify thrombus…

A
  • use various windows to interrogate apex
  • focus on the apex with higher resolution
  • use different depth; it helps distinguish between artifacts and thrombus
  • utilize the CFD; color travels around it if there’s a mass
  • document the thrombus in at least two views
  • TEE may be indicated
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12
Q

Dilated Cardiomyopathy

M-mode findings

A
  • thin walls with decreased function
  • increased chamber size
  • increased EPSS (MV E point to septal separation) > 0.7cm due to LV dilatation and decreased septal function
  • decreased D-E excursion
  • decreased MV excursion *double diamond shape
  • B notch on MV due to increased LVEDP ≥15mmHg

*image

M-mode findings in dilated cardiomyopathy.The mitral M-mode shows increased mitral E-point septal separation (EPSS) and a “B-bump” (left). The aortic M-mode shows decreased aortic root motion with early closure of the aortic valve (right).

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13
Q

Hypertrophic Cardiomyopathy (HCMO)

What is HCMO?

A

hypertrophic, hyperdynamic, non-dilated LV that is frequently (but not necessarily) associated with LVOTO (LVOT obstruction)

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14
Q

Hypertrophic Cardiomyopathy (HCMO)

etiology

A
  • idiopathic
  • genetic/gene mutations cause the heart muscle to grow abnormally thick
  • microscopic disorganization of the myocardial fibers
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15
Q

Hypertrophic Cardiomyopathy (HCMO)

It is recommended that the relatives of HCMO patients undergo an echo and/or genetic testing to rule out family link.

T or F ?

A

T

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16
Q

Hypertrophic Cardiomyopathy (HCMO)

_____ is used on mitral annulus to detect the mutation through diastolic dysfunction

A

TDI

*hypertrophy may not present until adolescence or later

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17
Q

Hypertrophic Cardiomyopathy (HCMO)

Types of HCMO:

A
  • hypertrophic obstructive cardiomyopathy (HOCM)
  • provocable HOCM
  • non-obstructive HCMO
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18
Q

Hypertrophic Cardiomyopathy (HCMO)

What is Hypertrophic Obstructive Cardiomyopathy (HOCM)?

A

LVH (LV hypertrophy) that is asymmetric, concentric, or midventricular with a LVOTO

*WHAT IS LVH?

Left ventricular hypertrophy is a form of cardiac remodeling that causes the heart wall muscle to thicken, which leads to an increase in LV mass (LVM). There are different sub-categories that fall into the diagnosis of LVH, which includes concentric, eccentric and concentric remodeling.

There are key factors that play a major role in determining the presence of LVH:

  • sex
  • age
  • body size
  • BP
  • HR
  • medications
  • diabetes
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19
Q

Hypertrophic Cardiomyopathy (HCMO)

Types of Hypertrophic Obstructive Cardiomyopathy (HOCM):

A
  • idiopathic hypertrophic subaortic stenosis (IHSS) with asymmetric septal hypertrophy (ASH) + systolic anterior motion (SAM) of the MV leaflets and/or chordae tendineae
  • HOCM with concentric LVH + SAM
  • HOCM with midventricular LVH: asymmetric left ventricular hypertrophy and by a pressure gradient between basal and apical sites in the left ventricle.
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20
Q

Hypertrophic Cardiomyopathy (HCMO)

What is provocable HOCM?

A

LVH with a LVOTO, but only when provoked with exercise, coughing, drugs, Valsalva maneuver other

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21
Q

Hypertrophic Cardiomyopathy (HCMO)

What is non-obstructive HCMO?

A

LVH that is apical or other, without LVOTO

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22
Q

Hypertrophic Cardiomyopathy (HCMO)

LVH severity scale

normal values for men & women?

A
  • men: 6-10 mm
  • women: 6-9 mm
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23
Q

Hypertrophic Cardiomyopathy (HCMO)

LVH severity scale

mild values for men & women?

A

men: 11-13 mm
women: 10-12 mm

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24
Q

Hypertrophic Cardiomyopathy (HCMO)

LVH severity scale

severe values for men & women?

A

men: ≥17mm
women: ≥16mm

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25
Q

Hypertrophic Cardiomyopathy (HCMO)

signs & symptoms

A
  • dyspnea on exertion (DOE)
  • orthopnea (SOB while laying down)
  • paroxysmal nocturnal dyspnea (intermittent SOB at night)
  • CP especially with exercise or exertion
  • dysrhythmias/palpitations
  • fatigue
  • syncope
  • pulmonary edema
  • patients with non-obst HCMO may be asymptomatic
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26
Q

Hypertrophic Cardiomyopathy (HCMO)

complications

A
  • infective endocarditis
  • embolus *likely due to LA embolus
  • HF
  • sudden death especially with exertion
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27
Q

Hypertrophic Cardiomyopathy (HCMO)

Although rare, HCMO is the leading cause of heart-related sudden death in those under the age of 30.

T or F ?

A

T

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28
Q

Hypertrophic Cardiomyopathy (HCMO)

testing/diagnosis

A
  • genetic testing
  • EKG
  • echo *gold standard
  • stress echo *determine if LVOT is provocable with stress
  • cardiac MRI *provide very precise measurements of myocardial wall thickness
  • ambulatory EKG monitor i.e. Holter monitor, Zio Patch) - detect arrhythmias
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29
Q

Hypertrophic Cardiomyopathy (HCMO)

Echo is the gold standard to diagnose and assess HCMO.

T or F ?

A

T

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30
Q

Hypertrophic Cardiomyopathy (HCMO)

treatment

A
  • medical therapy: beta blockers, calcium channel blockers, antiarrythmics, anticoagulants etc)
  • surgical options for significant LVOTO such as:
    • surgical septal myectomy: surgical removal of a portion of the thickened septum
    • alcohol septal ablation: cath lab procedure where a catheter inserted into the septal coronary artery. Once lined up with LVOT, 1-2mL of 100% ethyl alcohol is injected into the artery causing controlled damage to the myocardium. In the time, the myocardial thickness decreases, and this decreases the LVOTO
  • ICD (implantable cardioverter defibrillator) is for those who are at the high risk for sudden death
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31
Q

Hypertrophic Cardiomyopathy (HCMO)

__________ is a potential complication of alcohol septal ablation

A

heart block

*Heart block occurs when the electrical signals from the top chambers of your heart don’t conduct properly to the bottom chambers of your heart. There are three degrees of heart block. First degree heart block may cause minimal problems, however third degree heart block can be life-threatening.

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32
Q

Hypertrophic Cardiomyopathy (HCMO)

Which type of HOCM (hypertrophic obstructive CM) is this?

  • small LV cavity with hyperdynamic wall motion which often causes cavity obliteration (eradicate/erase) and compounds the LVOT
  • myocardial brightening due to fiber disarray
  • MR is probable
  • LAE (LA enlargement) due to MR and/or DD
A
  • HOCM with IHSS (idiopathic hypertrophic subaortic stenosis)
  • HOCM with concentric LVH
  • HOCM with midventricular LVH
  • HOCM with apical/other LVH
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33
Q

Hypertrophic Cardiomyopathy (HCMO)

Which type of HOCM (hypertrophic obstructive CM) is this?

  • SAM of the MV apparatus: the closed MV leaflet and/or chordae tendineae are pulled anteriorly into the narrowed, turbulent LVOT = leading to LVOTO
  • MV thickening and septal scarring result from the MV leaflets striking the IVS
  • MVP and/or MAC (mitral annular calcification) are potential findings
A

HOCM with IHSS

HOCM with concentric LVH

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34
Q

Hypertrophic Cardiomyopathy (HCMO)

What is ASH?

A

asymmetric septal hypertrophy

*Normally IVS/LVPW ratio is 1/1 or 1

ASH is classified by a IVS/LVPW ratio > or = 1.3/1.0 or 1.3

ex: if the IVS = 1.8 and LVPW = 1.1, the IVS/LVPW ratio = 1.8/1.1 = 1.6 (ASH)

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35
Q

Hypertrophic Cardiomyopathy (HCMO)

Which type of HOCM (hypertrophic obstructive CM) is this?

  • HOCM with ASH + SAM +associate findings with LVOT
A

HOCM with IHSS

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36
Q

Hypertrophic Cardiomyopathy (HCMO)

Which type of HOCM is this?

  • small, hyperdynamic LV
    myocardial brightening
  • MR
  • LAE
  • LVOTO
  • SAM
  • MV thickening/scarring
  • MVP
  • possible MAC
A

HOCM with concentric LVH + SAM + other findings associated with LVOTO

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37
Q

Hypertrophic Cardiomyopathy (HCMO)

Which type of HOCM is this?

  • small, hyperdynamic LV
  • myocardial brightening
  • MR
  • LAE
  • LVOTO
A

HOCM with midventricular LVH

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38
Q

Hypertrophic Cardiomyopathy (HCMO)

Which type of HOCM is this?

  • small, hyperdynamic LV
  • myocardial brightening
  • MR
  • LAE
A

Non-obst HCMO with apical/other hypertrophy without LVOTO

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39
Q

Hypertrophic Cardiomyopathy (HCMO)

Which type of HOCM is this?

  • small, hyperdynamic LV
  • myocardial brightening
  • MR
  • LAE
  • LVOTO
  • SAM
  • MV thickening/scarring
  • MVP
  • possible MAC
A

HOCM with IHSS

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40
Q

Hypertrophic Cardiomyopathy (HCMO)

M-Mode findings:

identify 1 - 5

A
  1. ASH (asymmetric septal hypertrophy) or another form of hypertrophy such as concentric, midventricular, or apical
  2. SAM of the MV and/or chordae tendineae
  3. LVOTO created by the small hyperdynamic LV cavity, ASH & SAM
  4. MV E-point to septal contact
  5. small LV with hyperkinetic motion
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41
Q

Hypertrophic Cardiomyopathy (HCMO)

M-Mode findings:

identify 6 - 7

A
  1. AOV notching (partial mid-systolic closure) of the AOV due to a sudden decrease in cardiac output
  2. LAE resulting from MR and/or DD due to a non-compliant LV
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42
Q

Hypertrophic Cardiomyopathy (HCMO)

Explain HCMO Doppler assessment steps:

A
  1. Rule out LVOTO
  • optimize Doppler angle
  • Acquire LVOT Doppler sample via PW: start sample gate in the midventricular region and move slowly move toward the AV
  • If LVOTO present, velocity will increase >2m/s
  1. Acquire the peak velocity
  • Switch to CW to acquire the highest velocity *LVOTO is a late peaking (dagger shape) systolic jet
  • planimeter the LVOT waveform for a mean PG
  1. When both LVOT and MR are present; important to differentiate between the two jets
  • angulation is vital! stay as close as 90 degree to the flow
  • The LVOTO waveform is a late peaking systolic jet with distinct sound
  • The MR waveform is wider than the LVOTO waveform because it encompasses the IVCT and IVRT
  • usually the MR peak velocity > LVOTO peak velocity
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43
Q

Hypertrophic Cardiomyopathy (HCMO)

The LVOTO is a late peaking _______ shape systolic jet that sounds like ________

A

dagger

a sponge being wrung out

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44
Q

Hypertrophic Cardiomyopathy (HCMO)

HCMO Doppler assessment steps continued:

Perform ______ or administer medications while acquiring LVOT Doppler samples if a provocable HOCM is suspected.

A

Valsalva maneuver

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45
Q

Restrictive/Infiltrative Cardiomyopathy

What is R/I CMO?

A

Infiltration of the myocardium that results in stiff, rigid ventricular walls that impedes diastolic filling and cause bilateral enlargement; typically results in heart failure

Increased resistance to ventricular filling due to increased myocardial stiffness (decreased ventricular compliance) associated with elevated ventricular diastolic pressure, increased atrial pressure, global systolic function may be preserved.

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46
Q

Restrictive/Infiltrative Cardiomyopathy

What is the difference between R/I CMO and constrictive pericarditis?

A

constrictive pericarditis usually surrounds the entire heart; whereas, R/I CMO primarily affects the ventricles

*note: restrict means to limit or put boundaries on something, constrict primarily means to make something narrower particularly through the use of a squeezing action, it is sometimes used figuratively to mean to limit something.

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47
Q

Restrictive/Infiltrative Cardiomyopathy

R/I CMO is the least common of all CMO diseases.

T or F ?

A

T

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48
Q

Restrictive/Infiltrative Cardiomyopathy

signs & symptoms

A
  • excessive fatigue/poor exercise tolerance
  • swelling of feet, ankles, and/or abdomen
  • cough/SOB especially with exertion, at night, or when spine
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49
Q

Restrictive/Infiltrative Cardiomyopathy

treatment

A
  • biopsy may be necessary to confirm diagnosis
  • the goal is to control symptoms and maintain quality of life (diuretics, steroids, chemotherapy)
  • heart transplant may be considered
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50
Q

Restrictive/Infiltrative Cardiomyopathy

Types of R/I CMO?

A
  1. Amyloidosis *most common
  2. Sarcoidosis
  3. Hemochromatosis
  4. Pompes
  5. Endomyocardial fibrosis
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51
Q

Restrictive/Infiltrative Cardiomyopathy

What is amylodosis?

A
  • extracellular deposition of amyloid protein in multiple organ systems causing damage and malfunction; produce heart stiffening that prevents completes filling
  • common to have an accompanying pericardial effusion and irregular rhythms
  • can mimic constrictive pericarditis

A multisystem disease in which there is extracellular deposition of the amyloid protein in the kidney, heart, liver, nerve, skin and tongue which results in tissue damage and organ malfunction

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52
Q

Restrictive/Infiltrative Cardiomyopathy

What is sarcoidosis?

A
  • marked by abnormal inflammatory masses (granulomas) that infiltrate multiple organ systems
  • heart complications include HF, PH, and irregular rhythms
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53
Q

Restrictive/Infiltrative Cardiomyopathy

What is hemochromatosis?

A
  • most common iron overload disease that may result in multiple and tissue damage
  • heart biopsy reveals iron deposition in the myocytes
  • heart complications include HF and irregular rhythms
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54
Q

Restrictive/Infiltrative Cardiomyopathy

What is pompes?

A
  • typically occurs early in life *hereditary factor
  • excessive glycogen storage in the tissues; the heart becomes enlarged and heavily thickened
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55
Q

Restrictive/Infiltrative Cardiomyopathy

What is endomyocardial fibrosis?

A
  • an endomyocardial disease where fibrotic tissue lines the myocardium; may be diffuse or local
  • local areas of necrosis are prone to apical thrombus
  • LV function is usually preserved
  • It may involve the valves, leading to severe regurgitation and dilated aorta
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56
Q

Restrictive/Infiltrative Cardiomyopathy

echo appearance

A
  • ventricular hypertrophy, typically in both ventricles
  • ground glass appearance of the myocardium
  • small-to-normal LV size with normal to decreased LV systolic function
  • bilateral enlargement
  • regurgitation of all the valves probable

additional findings

  • pericardial effusion
  • with endomyocardial fibrosis, the endocardium and AV valves may be scarred or echogenic
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57
Q

Restrictive/Infiltrative Cardiomyopathy

Doppler evaluation

A
  • regurgitation is probable, evaluate properly
  • evaluate DD
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58
Q

Restrictive/Infiltrative Cardiomyopathy

Doppler evaluation

Mitral inflow pattern has a restrictive pattern which has….

A

large E wave and small E wave without respiratory variation

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59
Q

Restrictive/Infiltrative Cardiomyopathy

M-mode evaluation

A
  • evaluate hypertrophy
  • evaluate function and chamber size
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60
Q

A CMO is a disease that diffusely affects the ______ resulting in enlargement and/or ventricular dysfunction.

A

myocardium

61
Q

Valvular heart disease, previous MI, and long-standing HTN are all potential causes of dilated CMO.

T or F ?

A

T

62
Q

The patient presents in her third trimester of pregnancy with sudden onset of CP, hypotension, tachycardia, extreme fatigue, palpitations, and dizziness; which of the following CMO disease state is most likely?

  1. dilated CMO
  2. hypertrophic CMO
  3. hypertrophic non-obstructive CMO
  4. infiltrative CMO
A

1

63
Q

Patients with dilated CMO experience multiple complications, such as ______

  1. HTN
  2. increased CO
  3. large pericardial effusion
  4. systemic embolism
A

4

*note: dilated CMO

  • reduces CO
  • possible pericardial effusion but usually small
  • thrombus, usually located in LA or LV, and associated findings: systemic embolization, neurological events
64
Q

Every dilated CMO patient requires treatment, even if they are asymptomatic.

T or F ?

A

F

*note: If asymptomatic, perhaps no treatment. Sometimes, dilated CMO comes and goes on its own.

65
Q

______ CMO is the most common of all the CMO disease states

A

dilated

66
Q

An ICD is used in CMO patients at high risk for sudden cardiac death.

T or F ?

A

T

*ICD: implantable cardioverter defibrillator

HCMO patients with at high risk for sudden death are the candidates for ICD

67
Q

Dilated CMO is caused by ______

  1. idiopathic
  2. familial link/genetics
  3. infectious process
  4. all of the above
A

4

*Dilated CMO cause

  • idiopathic
  • genetics *⅓ cases inherited
  • valvular heart disease
  • IHD/CAD
    long standing HTN
  • arrhythmias
  • infectious
  • thiamine deficiency
  • chemotherapy agents
  • toxin exposure
  • drugs
  • alcohol abuse
  • endocrine disease
  • Lupus
  • muscular dystrophy
  • peri-partum or post-partum
68
Q

Utilize _______ while interrogating the apex for a thrombus

  1. different windows and depths
  2. lower frequency TDR
  3. spectral Doppler
  4. all of the above
A

1

*possible thrombus primarily at the apex

use higher frequency

utilize CFD

use different windows and depths to differentiate thrombus from artifacts

69
Q

________ cause(s) HCMO

  1. chemotherapy
  2. gene mutation
  3. immune system disorder
  4. metabolic deficiency
A

2

*HCMO cause

idiopathic

gene mutations/genetic

microscopic disorganization of the myocardial fibers

70
Q

HCMO is recognized for its hypertrophic, hyperdynamic, and dilated LV

T or F ?

A

F

*HCMO is known for its hypertrophic, hyperdynamic, non-dilated LV that is frequently, but not necessarily associated with LVOTO

71
Q

Provocable HOCM is indicated when a LVOTO is provoked with the use of ______

  1. amyl nitrite
  2. exercise
  3. Valsalva maneuver
  4. all of the above
A

4

72
Q

Treatment options for HCMO include _______

  1. alcohol septal ablation in the cath lab
  2. medical therapy (beta blockers, Ca channel blockers, antiarrhythmics, anticoagulants)
  3. surgical septal myectomy
  4. all of the above
A

4

*Those at high risk for sudden death may be a candidate for an implantable cardioverter defibrillator (ICD)

73
Q

Some facilities treat patients with HOCM by injecting pure alcohol into the branch of the coronary artery that feeds the IVS in order to cause controlled damage to myocardium; what is the main goal of this procedure?

A

decrease IVS contraction and decrease LVOTO

74
Q

Stress echo is used to provoke a LVOTO

T or F ?

A

T

75
Q

A 19-year of patient present presents with dyspnea and CP on exertion, fatigue, and palpitations. The echo reveals an IVS/LVPW ratio of 1.7, systolic anterior motion of the MV, and a LVOTO with a peak gradient of 64mmHg; what is most likely diagnosis?

A

HOCM/classic IHSS

76
Q

The patient presents with a LVOTO and MR; how can the sonographer differentiate between the two Doppler jets?

  1. keep the TDR as parallel to flow as possible
  2. The LVOTO waveform encompasses the IVCT and IVRT
  3. The MR waveform is dagger shaped, late peak systolic jet
  4. all of the above
A

1

*LVOTO waveform is a dagger shaped, late peaking systolic jet

MR waveform is wider than LVOTO waveform and it encompasses the IVCT and IVRT

77
Q

How can the sonographer rule out LVOTO?

A
  • acquire and listen Doppler samples throughout the LVOT *LVOTO sound: similar to a a sponge being wrung out
  • acquire the LVOT peak velocity and PG
  • differentiate between the LVOT and probable MR
78
Q

If the LVOT peak velocity is 5 m/s, what is the peak PG?

A

100 mmHg

*PG = 4V2 (Bernouli’s equation)

79
Q

Which of the following is true regarding R/I CMO?

  1. biventricular enlargement
  2. diastolic function is normal
  3. resemble constrictive pericarditis
  4. all of the above
A

3

*R/I CMO

  • impede diastolic filling due to stiff, rigid ventricular walls
  • impeded diastolic filling causes biatrial enlargement which typically results in HF
80
Q

_______ is a multisystem disease involving extracellular deposition of amyloid protein in multiple systems causing a stiffening of the heart that prevents complete filling and may be accompanied by pericardial effusion and irregular rhythms.

A

Amylodosis

81
Q

________ is marked by granulomas that infiltrate the heart and various organs; complications include HF, PH, and irregular rhythms.

A

Sarcoidosis

82
Q

_______ typically occurs early in life and causes the heart to become enlarged and heavily thickened due to excessive glycogen in the tissues.

A

Pompes

83
Q

Hemochromatosis is the most common ____ overload disease that may result in multiple organ and tissue damage.

A

iron

84
Q

The sonographer should rule out ____ in the patient with endomycardial fibrosis.

  1. areas of necrosis and possible thrombus
  2. decreased LV function
  3. preserved valve function
  4. all of the above
A

1

*Endomyocardial fibrosis

  • an endocardial disease where fibrotic tissue lines the myocardium; may be diffuse or local
  • local areas of necrosis are prone to apical thrombus
  • LV function is usually preserved
  • may involve the valves, leading to severe regurgitation and dilated aorta
85
Q

Anatomical Variants of HCM

A

neutral

86
Q

Anatomical Variants of HCM

A

reverse curvature

87
Q

Anatomical Variants of HCM

A

Sigmoid

88
Q

Anatomical Variants of HCM

A

apical

89
Q

Uniform “sparkling”, “ground glass” granular myocardial appearance describes dilated cardiomyopathy.

T or F ?

A

F

90
Q

Restrictive/Infiltrative Cardiomyopathy is the _____ common of all types of cardiomyopathy.

A

least

91
Q

_____________ is a hereditary factor associated with infiltrative/restrictive CM and typically occurs early in life.

Characteristics are: excessive glycogen storage in tissues; heart becomes enlarged and heavily thickened; and is autosomal recessive.

A

Pompes

92
Q

List 4 storage disorders associated with restrictive cardiomyopathy

A

amyloidosis

sarcoidosis

hemochromatosis

pompes

93
Q

Reduced systolic function; decreased EF, < 30% describes which type of cardiomyopathy?

A

dilated CM

94
Q

An iron storage disease that affects multiple organ and tissue systems which may result in tissue damage and organ malfunction (the iron is stored within the cardiac cell rather than extracellular) is ______________________.

A

hemochromatosis

95
Q

A multisystem disease in which there is extracellular deposition of the amyloid protein in the kidney, heart, liver, nerve, skin and tongue which results in tissue damage and organ malfunction.

A

amyloidosis

*most common R/I CMO

96
Q

List 4 causes of Dilated Cardiomyopathy (DCM) or Congestive CMO:

A
  • idiopathic *most common primary etiology
  • IHD/CAD/MI
  • alcohol abuse (ETOH: ethyl alcohol) *most common second etiology
  • congenital *endocardial fibroelastosis
  • some drugs used in chemotherapy
  • metabolic *thiamine deficiency
  • viral (Coxsackie B) bacterial, fungal, parasitic (Chagas)
96
Q

List 4 causes of Dilated Cardiomyopathy (DCM) or Congestive CMO:

A
  • idiopathic *most common primary etiology
  • IHD/CAD/MI
  • alcohol abuse (ETOH: ethyl alcohol) *most common second etiology
  • congenital *endocardial fibroelastosis
  • some drugs used in chemotherapy
  • metabolic *thiamine deficiency
  • viral (Coxsackie B) bacterial, fungal, parasitic (Chagas)
97
Q

True idiopathic and familial forms of dilated CM are relatively rare. Secondary causes of dilated CM are more common.

T or F ?

A

T

98
Q

List 4 signs/symptoms that a patient with hypertrophic cardiomyopathy will present with.

A
  • Chest pain
  • Syncope
  • Dyspnea
  • Fatigue
  • Arrhythmia
  • (PVCs, atrial fibrillation)
99
Q

When discussing dynamic LVOT obstruction traits

Hydrodynamic drag forces (______?_____) describes the reduction in fluid pressure that results when a fluid flows through a constricted section (or choke) of a pipe.

A

Venturi effect

100
Q

What is Venturi effect?

A

the reduction in fluid pressure that results when a fluid flows through a constricted section of a pipe.

101
Q

Dynamic LVOT Obstruction Traits

A
  • Septal hypertrophy
  • Systolic anterior motion of mitral valv=e leaflets *SAM
  • Systolic anterior displacement of entire mitral valve complex (including papillary muscles)
  • Redundant (unnecessary) mitral valve leaflets
  • Hydrodynamic drag forces *Venturi effect
102
Q

List 4 conditions associated with SAM:

A
  • Hypertrophic cardiomyopathy
  • Left ventricular hypertrophy
  • Infiltrative cardiomyopathies with septal involvement
  • Hypercontractile states
  • Mechanical causes
103
Q

________________is a rare, genetic, recessive disease originates from mutations in the “coding” of the mitochondria.

A

Friedreich’s Ataxia

Friedreich’s ataxia (FRDA) is a genetic, progressive, neurodegenerative movement disorder, with a typical age of onset between 10 and 15 years. Initial symptoms may include unsteady posture, frequent falling, and progressive difficulty in walking due to impaired ability to coordinate voluntary movements (ataxia).

104
Q

What is Friedreich’s Ataxia?

A

Friedreich’s ataxia (FRDA) is a rare, genetic, progressive, neurodegenerative movement disorder, with a typical age of onset between 10 and 15 years. Initial symptoms may include unsteady posture, frequent falling, and progressive difficulty in walking due to impaired ability to coordinate voluntary movements (ataxia).

Originates from mutations in the “coding” of the mitochondria.
Both parents must have the dominant trait for a 25% chance of passing to offspring

105
Q

The main types of cardiomyopathy include __1__, ___2__, and __3__ cardiomyopathy. Treatment — which might include medications, surgically implanted devices or, in severe cases, a ___4__ — depends on which type of cardiomyopathy you have and how serious it is.

A
  1. dilated CMO
  2. hypertrophic CMO
  3. restrictive CMO
  4. heart transplant
106
Q

Heart Transplant is never a surgical consideration for restrictive CM.

T or F ?

A

F

107
Q

Amyloidosis is the most common infiltrative disorder.

T or F ?

A

T

108
Q

Dynamic LVOT obstruction; “dagger-shaped” CW profile; mitral regurgitant (accompanying SAM) with posteriorly directed jet; relaxation abnormality / diastolic dysfunction describe which type of CM?

A

hypertrophic CMO

109
Q

Conditions that decrease preload, lower afterload, and increase contractility generally increase LVOT gradient (and the systolic murmur).

T or F ?

A

T

110
Q

Provocative maneuvers that influence LVOT gradient in HCM assessment

A

Valsalva

Amyl nitrite

111
Q

True idiopathic and familial forms of dilated cardiomyopathy are relatively rare.

T or F ?

A

T

112
Q

In cases of severe dilated cardiomyopathy, the increased EPSS (E-point to septal separation) is indicative of increased left ventricular systolic function.

T or F ?

A

F

*EPSS –increased E-point to septal separation indicative of decreased left ventricular systolic function

113
Q

Secondary causes of dilated cardiomyopathy are rare.

T or F ?

A

F

*secondary causes of dilated cardiomyopathy are more common

*note: most common secondary etiology: ethyl alcohol

114
Q

Cardiomyopathy is a disease of the heart muscle that makes it easier for your heart to pump blood to the rest of your body. Cardiomyopathy can lead to heart failure.

T or F ?

A

F

*Cardiomyopathy is a disease of the heart muscle that makes it harder for your heart to pump blood to the rest of your body. Cardiomyopathy can lead to heart failure.

115
Q

CM

What are

  • Primary
  • Cross over
  • Secondary

meaning?

A

primary: congenital/genetic i.e. HCM

cross over: idiopathic i.e. DCM & RCM

secondary: acquired i.e. ICM

116
Q

Cardiomyopathies: Three Major Functional Types

Which CM has the following clinical presentation?

Often asymptomatic; syncope; sudden death; genetic mutation (1 in 500)

A

HCM

117
Q

Cardiomyopathies: Three Major Functional Types

Which CM has the following clinical presentation?

Reduced systolic function; decreased EF, < 30%

A

DCM

118
Q

Cardiomyopathies: Three Major Functional Types

Which CM has the following clinical presentation?

  • Progressive dyspnea, right- sided heart failure; features of underlying disorder
  • Reduced ventricular cavity size; dilated atria – marked; dilated right sided chambers
A

RCM

119
Q

2D Criteria Hypertrophic Cardiomyopathy

SAM: mitral to septal distance > 10 mm

A

mild

120
Q

2D Criteria Hypertrophic Cardiomyopathy

SAM: < 10 mm or brief mitral leaflet-septal contact

A

moderate

121
Q

2D Criteria Hypertrophic Cardiomyopathy

prolonged SAM with septal contact, lasting more

than 30 % of systole

A

severe

122
Q

2D Criteria Hypertrophic Cardiomyopathy

A
  • SAM
  • Venturi effect (as the fluid speed increases past the point of obstruction a drag is created which moves the mitral leaflet or chordae anterior systole), anterior papillary muscle and mitral apparatus displacement occurs
  • Elongated and increased area of anterior mitral leaflet (AML) and reduced tension on the leaflet causes typical SAM.
  • Calcified mitral annulus
  • LA enlargement due to decreased left ventricular compliance and / or MR
  • Narrowed LVOT diameter due to IVS hypertrophy
  • Altered texture of myocardium (ground glass appearance, amyloid appearance)
  • Basal septal endocardial thickening (contact lesion)
123
Q

M – mode Criteria HCM

A

Abnormal MV motion:

  1. Diastolic reduction of E-F slope with normal posterior leaflet motion
  2. Mitral annular calcifications
  3. Systolic anterior motion (SAM) may reflect LV outflow tract gradient, but can occur in other clinical conditions (e.g., MVP, apical infarction)
  4. “B” bump on the mitral valve due to elevated LVEDP
  5. Aortic valve motion may show mid-systolic closure if significant systolic outflow gradient is present
124
Q

Doppler Criteria HCM:
Diastolic dysfunction assessment Characteristics

A

Impaired relaxation
 Decreased compliance
 Increased LVEDP
 Decreased E/A ration
 A normal filling pattern suggests:

  • Normalization by MR
  • Normalization by increased LA pressure
125
Q

HCM Limitations and pitfalls

Differential diagnosis

A
  • chronic HTN
  • cardiac amyloid
  • pheochromocytoma
  • inferior MI with previous LV dysfunction
  • Freidreich’s ataxia
126
Q

Rare subtype involving predominantly the apex. Common is Asia ( 41% of HCM China, 30% HCM Japan, 38% Korea)

A

Apical Hypertrophic CM

127
Q

Things that may mimic Apical HCM:

A
  • Hypertrophied papillary muscles
  • Noncompaction cardiomyopathy
  • Apical thrombus
  • Left ventricular tumor
  • Hypereosinophilic syndrome

Hypereosinophilic syndrome (HES) is a group of blood disorders that occur when you have high numbers of eosinophils — white blood cells that play an important role in your immune system. Over time, the excess eosinophils enter various tissues, eventually damaging your organs.

128
Q

Assymetric Septal Hypertrophy - ASH

A
129
Q

Acquired LVH

A
  • athletes’ heart
  • arterial HTN
  • infiltrative disorders i.e. Amyloidosis, Sarcoidosis
  • AV disease
130
Q

Familial LVH (primary/congenital)

A
  • sarcomeric HCM: involves an increase in the amount of the sarcoplasmic fluid that surrounds the muscle. The increase in fluid leads to the size of the muscle being increased and appearing bigger. There is no increase in strength as the amount of contractile proteins (actin and myosin) stays the same.
  • syndromic HCM
    • Noonan syndrome: a genetic disorder that prevents normal development in various parts of the body. A person can be affected by Noonan syndrome in a wide variety of ways. These include unusual facial characteristics, short stature, heart defects, other physical problems and possible developmental delays.
    • Leopard syndrome: a very rare inherited disorder. People with this condition have problems with the skin, head and face, inner ear, and heart. The genitals may also be affected.
    • Friedeich ataxia: a genetic condition that affects the nervous system and causes movement problems.
  • lysomal storage disease
    • Anderson-Farbry disease: Without functioning alpha-GAL enzymes, harmful levels of sphingolipids build up in blood vessels and tissues. Fabry disease affects the heart, kidneys, brain, central nervous system and skin.
    • Gaucher disease:missing an enzyme that breaks down fatty substances called lipids. Lipids start to build up in certain organs such as your spleen and liver. This can cause many different symptoms
    • Hurler disease: lack an enzyme that the body needs to digest sugar. As a result, undigested sugar molecules build up in the body, causing progressive damage to the brain, heart, and other organs.
  • Mitochondrial myopathies
  • glycogen storage disease (GSD)
    • Pompe disease (GSD II)
    • Cori disease (GSD II)
    • PRKAG2 disease: PRKAG2 syndrome (PS) is a rare, early-onset autosomal dominant inherited disease, characterized by ventricular pre-excitation, supraventricular arrhythmias, and cardiac hypertrophy.
      • Danon disease: a condition characterized by weakening of the heart muscle (cardiomyopathy ); weakening of the muscles used for movement, called skeletal muscles, (myopathy); and intellectual disability. Males with Danon disease usually develop the condition earlier than females and are more severely affected.
131
Q

Athletic heart vs HCM

Echo findings

A
132
Q

Athletic heart vs HCM

A

Athletes’ heart

  • Myocardial systolic velocities, strain and strain rate are normal
  • Diastolic function is enhanced in AH; Normal TDI and increase in twist and untwist
  • Preserved left atrial dilatation

HCM

  • attenuation is diastolic tissue Doppler velocities and delayed untwist.
  • Increased LA volume and reduction in strain and strain rates.
133
Q

HCM vs Athletes’ Heart

A
134
Q

R/I CM Physical Examination

What are the clinical signs?

A
  1. RHF
  • jugular venous distension
  • hepatomegaly
  • peripheral edema
  • ascites
  • anasarca-generalized swelling: Anasarca is a medical condition that leads to general swelling of the whole body. It happens when your body tissues retain too much fluid due to several reasons. It differs from other types of edema that affect one or two parts of the body
  1. Increased BNP - suggest restrictive cardiomyopathy (levels five times higher as compared to constrictive pericarditis) *BNP: Brain natriuretic peptide (BNP) test is a blood test that measures levels of a protein called BNP that is made by your heart and blood vessels. BNP levels are higher than normal when you have heart failure
  2. Swelling of feet, ankles, and/or abdomen
135
Q

What is BMP?

A

Basic Metabolic Panel (group of 8 tests that measures metabolic levels –sugar/glucose, electrolyte , fluid balance , kidney function, BUN)

136
Q

Classification of Restrictive Cardiomyopathy according to Cause

A
137
Q

Amyloidosis Echo Findings

A
138
Q

Sacoidosis is Multisystem granulomatous disease; involves the heart in about 25% of cases and occurs in twice as many females as males.

T or F ?

A

T

139
Q

etiology of Sarcoidosis

A

unknown

140
Q

other clinical signs of Sarcoidosis

A

Progressive heart failure, Sudden death

ECG: complete heart block

141
Q

Loeffler endocarditis is seen in patients with Hypereosinophilic syndrome. (>1500 eosinophils/mL)

T or F ?

A

T

142
Q

Sarcoidosis is multisystem granulomatous disease; involves the heart in about 25% of cases and occurs in twice as many females as males

T or F ?

A

T

143
Q

What is Loeffler endocarditis?

A

Loeffler endocarditis is a form of heart disease characterized by a stiffened, poorly-functioning heart caused by infiltration of the heart by white blood cells known as eosinophils.

Loeffler endocarditis is seen in patients with Hypereosinophilic syndrome. (>1500 eosinophils/mL)

144
Q

What is Fabry disease?

A

a rare genetic disease with a deficiency of an enzyme called alpha-GAL.

A lipid storage disorder that is X-linked and affects mainly males.

Cardiovascular problems (heart failure, arrhythmia, heart attack, faulty heart valves, enlarged heart) happen because years of GL- 3 buildup results in damage to the heart and blood vessels that supply the heart.

Identification of a binary endocardial wall is 94% conclusive

145
Q

What is Danon disease?

A

glycogen storage disease; X-linked; associated with CM, muscle weakness, intellectual disability; LV hypertrophy with depressed EF

Danon disease is a condition characterized by weakening of the heart muscle (cardiomyopathy ); weakening of the muscles used for movement, called skeletal muscles, (myopathy); and intellectual disability.

Males with Danon disease usually develop the condition earlier than females and are more severely affected.

146
Q

What is Oxalosis?

A

Oxalosis is a rare metabolic disorder that occurs when the kidneys stop eliminating calcium oxalate crystals from the body through the urine. Because the kidneys stop functioning, oxalate crystals are deposited elsewhere in the body.

causes kidney stones; renal failure-Thickening of bilateral walls with speckling

147
Q

Infiltrative Disorders

A

Hurler syndrome is an inherited condition caused by a faulty gene. Children with Hurler syndrome lack an enzyme that the body needs to digest sugar. As a result, undigested sugar molecules build up in the body, causing progressive damage to the brain, heart, and other organs.

Gaucher disease is a rare genetic disorder passed down from parents to children (inherited). When you have Gaucher disease, you are missing an enzyme that breaks down fatty substances called lipids. Lipids start to build up in certain organs such as your spleen and liver. This can cause many different symptoms.

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