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
2
Q
The main types of CM includes:
A
dilated
hypertrophic
restrictive
3
Q
Treatment for CM
A
medications
surgically implanted devices
(for severe case) heart transplant
4
Q
Dilated Cardiomyopathy
Dilated CMO (aka: congestive CMO) is the most common CMO.
T or F ?
A
T
5
Q
Dilated Cardiomyopathy
Dilated CMO is known for……..
A
multi-chamber enlargement
decreased systolic & diastolic function
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
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)
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
9
Q
Dilated Cardiomyopathy
Sometimes dilated CMO comes and goes on its own.
T or F ?
A
T
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!
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
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).
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)
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
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
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
17
Q
Hypertrophic Cardiomyopathy (HCMO)
Types of HCMO:
A
- hypertrophic obstructive cardiomyopathy (HOCM)
- provocable HOCM
- non-obstructive HCMO
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
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.
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
21
Q
Hypertrophic Cardiomyopathy (HCMO)
What is non-obstructive HCMO?
A
LVH that is apical or other, without LVOTO
22
Q
Hypertrophic Cardiomyopathy (HCMO)
LVH severity scale
normal values for men & women?
A
- men: 6-10 mm
- women: 6-9 mm
23
Q
Hypertrophic Cardiomyopathy (HCMO)
LVH severity scale
mild values for men & women?
A
men: 11-13 mm
women: 10-12 mm
24
Q
Hypertrophic Cardiomyopathy (HCMO)
LVH severity scale
severe values for men & women?
A
men: ≥17mm
women: ≥16mm
25
_Hypertrophic Cardiomyopathy (HCMO)_
signs & symptoms
* **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**
26
_Hypertrophic Cardiomyopathy (HCMO)_
complications
* infective endocarditis
* embolus \*likely due to LA embolus
* HF
* sudden death especially with exertion
27
_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 ?
T
28
_Hypertrophic Cardiomyopathy (HCMO)_
testing/diagnosis
* 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
29
_Hypertrophic Cardiomyopathy (HCMO)_
Echo is the gold standard to diagnose and assess HCMO.
T or F ?
T
30
_Hypertrophic Cardiomyopathy (HCMO)_
treatment
* 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
31
_Hypertrophic Cardiomyopathy (HCMO)_
\_\_\_\_\_\_\_\_\_\_ is a potential complication of alcohol septal ablation
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.
32
_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**
* HOCM with **IHSS (**idiopathic hypertrophic subaortic stenosis)
* HOCM with **concentric LVH**
* HOCM with **midventricular LVH**
* HOCM with **apical/other LVH**
33
_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
HOCM with IHSS
HOCM with concentric LVH
34
_Hypertrophic Cardiomyopathy (HCMO)_
What is ASH?
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**)
35
_Hypertrophic Cardiomyopathy (HCMO)_
Which type of HOCM (hypertrophic obstructive CM) is this?
* HOCM with ASH + SAM +associate findings with LVOT
HOCM with IHSS
36
_Hypertrophic Cardiomyopathy (HCMO)_
Which type of HOCM is this?
* small, hyperdynamic LV
myocardial brightening
* MR
* LAE
* LVOTO
* SAM
* MV thickening/scarring
* MVP
* possible MAC
HOCM with concentric LVH + SAM + other findings associated with LVOTO
37
_Hypertrophic Cardiomyopathy (HCMO)_
Which type of HOCM is this?
* small, hyperdynamic LV
* myocardial brightening
* MR
* LAE
* LVOTO
HOCM with midventricular LVH
38
_Hypertrophic Cardiomyopathy (HCMO)_
Which type of HOCM is this?
* small, hyperdynamic LV
* myocardial brightening
* MR
* LAE
Non-obst HCMO with apical/other hypertrophy without LVOTO
39
_Hypertrophic Cardiomyopathy (HCMO)_
Which type of HOCM is this?
* small, hyperdynamic LV
* myocardial brightening
* MR
* LAE
* LVOTO
* SAM
* MV thickening/scarring
* MVP
* possible MAC
HOCM with IHSS
40
_Hypertrophic Cardiomyopathy (HCMO)_
M-Mode findings:
identify 1 - 5
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
41
_Hypertrophic Cardiomyopathy (HCMO)_
M-Mode findings:
identify 6 - 7
6. AOV notching (partial mid-systolic closure) of the AOV due to a sudden decrease in cardiac output
7. LAE resulting from MR and/or DD due to a non-compliant LV
42
_Hypertrophic Cardiomyopathy (HCMO)_
Explain HCMO Doppler assessment steps:
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**
2. _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
3. _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
43
_Hypertrophic Cardiomyopathy (HCMO)_
The LVOTO is a late peaking _______ shape systolic jet that sounds like \_\_\_\_\_\_\_\_
dagger
a sponge being wrung out
44
_Hypertrophic Cardiomyopathy (HCMO)_
HCMO Doppler assessment steps continued:
Perform ______ or administer medications while acquiring LVOT Doppler samples if a provocable HOCM is suspected.
Valsalva maneuver
45
_Restrictive/Infiltrative Cardiomyopathy_
What is R/I CMO?
Infiltration of the myocardium that results in stiff, rigid ventricular walls that impedes diastolic filling and cause bilateral enlargement; typically results in heart failure
## Footnote
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.**
46
_Restrictive/Infiltrative Cardiomyopathy_
What is the difference between R/I CMO and constrictive pericarditis?
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.
47
_Restrictive/Infiltrative Cardiomyopathy_
R/I CMO is the least common of all CMO diseases.
T or F ?
T
48
_Restrictive/Infiltrative Cardiomyopathy_
signs & symptoms
* excessive fatigue/poor exercise tolerance
* swelling of feet, ankles, and/or abdomen
* cough/SOB especially with exertion, at night, or when spine
49
_Restrictive/Infiltrative Cardiomyopathy_
treatment
* 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
50
_Restrictive/Infiltrative Cardiomyopathy_
Types of R/I CMO?
1. **Amyloidosis** \***most common**
2. **Sarcoidosis**
3. **Hemochromatosis**
4. **Pompes**
5. **Endomyocardial fibrosis**
51
_Restrictive/Infiltrative Cardiomyopathy_
What is amylodosis?
* 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**
52
_Restrictive/Infiltrative Cardiomyopathy_
What is sarcoidosis?
* marked by abnormal inflammatory masses (granulomas) that infiltrate multiple organ systems
* heart complications include HF, PH, and irregular rhythms
53
_Restrictive/Infiltrative Cardiomyopathy_
What is hemochromatosis?
* 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
54
_Restrictive/Infiltrative Cardiomyopathy_
What is pompes?
* typically occurs early in life \*hereditary factor
* excessive glycogen storage in the tissues; the heart becomes enlarged and heavily thickened
55
_Restrictive/Infiltrative Cardiomyopathy_
What is endomyocardial fibrosis?
* 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
56
_Restrictive/Infiltrative Cardiomyopathy_
echo appearance
* **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
57
_Restrictive/Infiltrative Cardiomyopathy_
Doppler evaluation
* regurgitation is probable, evaluate properly
* evaluate DD
58
_Restrictive/Infiltrative Cardiomyopathy_
Doppler evaluation
Mitral inflow pattern has a restrictive pattern which has….
large E wave and small E wave without respiratory variation
59
_Restrictive/Infiltrative Cardiomyopathy_
M-mode evaluation
* evaluate hypertrophy
* evaluate function and chamber size
60
A CMO is a disease that diffusely affects the ______ resulting in enlargement and/or ventricular dysfunction.
myocardium
61
Valvular heart disease, previous MI, and long-standing HTN are all potential causes of dilated CMO.
T or F ?
T
62
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
1
63
Patients with dilated CMO experience multiple complications, such as \_\_\_\_\_\_
1. HTN
2. increased CO
3. large pericardial effusion
4. systemic embolism
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
Every dilated CMO patient requires treatment, even if they are asymptomatic.
T or F ?
F
\*note: If asymptomatic, perhaps no treatment. Sometimes, dilated CMO comes and goes on its own.
65
\_\_\_\_\_\_ CMO is the most common of all the CMO disease states
dilated
66
An ICD is used in CMO patients at high risk for sudden cardiac death.
T or F ?
T
\*ICD: implantable cardioverter defibrillator
HCMO patients with at high risk for sudden death are the candidates for ICD
67
Dilated CMO is caused by \_\_\_\_\_\_
1. idiopathic
2. familial link/genetics
3. infectious process
4. all of the above
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
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
1
\*possible thrombus primarily at the apex
use higher frequency
utilize CFD
use different windows and depths to differentiate thrombus from artifacts
69
\_\_\_\_\_\_\_\_ cause(s) HCMO
1. chemotherapy
2. gene mutation
3. immune system disorder
4. metabolic deficiency
2
\*HCMO cause
idiopathic
gene mutations/genetic
microscopic disorganization of the myocardial fibers
70
HCMO is recognized for its hypertrophic, hyperdynamic, and dilated LV
T or F ?
F
\*HCMO is known for its hypertrophic, hyperdynamic, **non-dilated** LV that is frequently, but not necessarily associated with LVOTO
71
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
4
72
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
4
\*Those at high risk for sudden death may be a candidate for an implantable cardioverter defibrillator (ICD)
73
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?
decrease IVS contraction and decrease LVOTO
74
Stress echo is used to provoke a LVOTO
T or F ?
T
75
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?
HOCM/classic IHSS
76
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
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
How can the sonographer rule out LVOTO?
* 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
If the LVOT peak velocity is 5 m/s, what is the peak PG?
100 mmHg
\*PG = 4V2 (Bernouli's equation)
79
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
**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
\_\_\_\_\_\_\_ 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.
Amylodosis
81
\_\_\_\_\_\_\_\_ is marked by granulomas that infiltrate the heart and various organs; complications include HF, PH, and irregular rhythms.
Sarcoidosis
82
\_\_\_\_\_\_\_ typically occurs early in life and causes the heart to become enlarged and heavily thickened due to excessive glycogen in the tissues.
Pompes
83
Hemochromatosis is the most common ____ overload disease that may result in multiple organ and tissue damage.
iron
84
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
**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
_Anatomical Variants of HCM_
neutral
86
_Anatomical Variants of HCM_
reverse curvature
87
_Anatomical Variants of HCM_
Sigmoid
88
_Anatomical Variants of HCM_
apical
89
Uniform “sparkling”, “ground glass” granular myocardial appearance describes dilated cardiomyopathy.
T or F ?
F
90
Restrictive/Infiltrative Cardiomyopathy is the _____ common of all types of cardiomyopathy.
least
91
\_\_\_\_\_\_\_\_\_\_\_\_\_ 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.
Pompes
92
List 4 storage disorders associated with restrictive cardiomyopathy
amyloidosis
sarcoidosis
hemochromatosis
pompes
93
Reduced systolic function; decreased EF, \< 30% describes which type of cardiomyopathy?
dilated CM
94
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 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_.
hemochromatosis
95
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.
amyloidosis
\***most common R/I CMO**
96
List 4 causes of Dilated Cardiomyopathy (DCM) or Congestive CMO:
* 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
List 4 causes of Dilated Cardiomyopathy (DCM) or Congestive CMO:
* 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
True idiopathic and familial forms of dilated CM are relatively rare. Secondary causes of dilated CM are more common.
T or F ?
T
98
List 4 signs/symptoms that a patient with hypertrophic cardiomyopathy will present with.
* Chest pain
* Syncope
* Dyspnea
* Fatigue
* Arrhythmia
* (PVCs, atrial fibrillation)
99
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.
Venturi effect
100
What is Venturi effect?
the reduction in fluid pressure that results when a fluid flows through a constricted section of a pipe.
101
Dynamic LVOT Obstruction Traits
* **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
List 4 conditions associated with SAM:
* Hypertrophic cardiomyopathy
* Left ventricular hypertrophy
* Infiltrative cardiomyopathies with septal involvement
* Hypercontractile states
* Mechanical causes
103
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_is a rare, genetic, recessive disease originates from mutations in the “coding” of the mitochondria.
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
What is **Friedreich’s Ataxia**?
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
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.
1. dilated CMO
2. hypertrophic CMO
3. restrictive CMO
4. heart transplant
106
Heart Transplant is never a surgical consideration for restrictive CM.
T or F ?
F
107
Amyloidosis is the most common infiltrative disorder.
T or F ?
T
108
Dynamic LVOT obstruction; “dagger-shaped” CW profile; mitral regurgitant (accompanying SAM) with posteriorly directed jet; relaxation abnormality / diastolic dysfunction describe which type of CM?
hypertrophic CMO
109
Conditions that decrease preload, lower afterload, and increase contractility generally increase LVOT gradient (and the systolic murmur).
T or F ?
T
110
Provocative maneuvers that influence LVOT gradient in HCM assessment
Valsalva
Amyl nitrite
111
True idiopathic and familial forms of dilated cardiomyopathy are relatively rare.
T or F ?
T
112
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 ?
F
\*EPSS –_increased E-point_ to septal separation indicative of **decreased** left ventricular systolic function
113
Secondary causes of dilated cardiomyopathy are rare.
T or F ?
F
\*secondary causes of dilated cardiomyopathy are more common
\*note: most common secondary etiology: ethyl alcohol
114
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 ?
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
_CM_
What are
* Primary
* Cross over
* Secondary
meaning?
primary: congenital/genetic i.e. **HCM**
cross over: idiopathic i.e. **DCM & RCM**
secondary: acquired i.e. **ICM**
116
_Cardiomyopathies: Three Major Functional Types_
Which CM has the following clinical presentation?
Often asymptomatic; syncope; sudden death; genetic mutation (1 in 500)
HCM
117
_Cardiomyopathies: Three Major Functional Types_
Which CM has the following clinical presentation?
Reduced systolic function; decreased EF, \< 30%
DCM
118
_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
RCM
119
_2D Criteria Hypertrophic Cardiomyopathy_
SAM: mitral to septal distance \> 10 mm
mild
120
_2D Criteria Hypertrophic Cardiomyopathy_
SAM: \< 10 mm or brief mitral leaflet-septal contact
moderate
121
_2D Criteria Hypertrophic Cardiomyopathy_
prolonged SAM with septal contact, lasting more
than 30 % of systole
severe
122
_2D Criteria Hypertrophic Cardiomyopathy_
* 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
_M – mode Criteria HCM_
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
_Doppler Criteria HCM:_
_Diastolic dysfunction assessment Characteristics_
Impaired relaxation
Decreased compliance
Increased LVEDP
Decreased E/A ration
A normal filling pattern suggests:
- Normalization by MR
- Normalization by increased LA pressure
125
_HCM Limitations and pitfalls_
_Differential diagnosis_
* chronic HTN
* cardiac amyloid
* pheochromocytoma
* inferior MI with previous LV dysfunction
* **Freidreich’s ataxia**
126
Rare subtype involving predominantly the apex. Common is Asia ( 41% of HCM China, 30% HCM Japan, 38% Korea)
**Apical Hypertrophic CM**
127
Things that may mimic Apical HCM:
* **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
_Assymetric Septal Hypertrophy - ASH_
129
_Acquired LVH_
* athletes' heart
* arterial HTN
* infiltrative disorders i.e. Amyloidosis, Sarcoidosis
* AV disease
130
Familial LVH (primary/congenital)
* **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
_Athletic heart vs HCM_
Echo findings
132
_Athletic heart vs HCM_
**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
HCM vs Athletes' Heart
134
_R/I CM Physical Examination_
What are the clinical signs?
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
2. 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
3. Swelling of feet, ankles, and/or abdomen
135
What is BMP?
Basic Metabolic Panel (group of 8 tests that measures metabolic levels –sugar/glucose, electrolyte , fluid balance , kidney function, BUN)
136
Classification of Restrictive Cardiomyopathy according to Cause
137
Amyloidosis Echo Findings
138
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 ?
T
139
etiology of Sarcoidosis
unknown
140
other clinical signs of Sarcoidosis
Progressive heart failure, Sudden death
ECG: complete heart block
141
Loeffler endocarditis is seen in patients with Hypereosinophilic syndrome. (\>1500 eosinophils/mL)
T or F ?
T
142
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 ?
T
143
What is Loeffler endocarditis?
**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/m**L)
144
What is Fabry disease?
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
What is Danon disease?
**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
What is Oxalosis?
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-T**hickening of bilateral walls with speckling**
147
**Infiltrative Disorders**
**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.
