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Flashcards in Cardiomyopathy Deck (50)
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1
Q
Case presentation
A
19 y/o Male, presents to your office with complaints of palpitations and occasional chest pain especially when he has been practicing for his football team


Fam Hx: Uncle passed away at age 25, Cousin has heart problem
2
Q
Definition of cardiomyopathy
A
a heterogeneous group of diseases of the myocardium associated with mechanical &/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic. Cardiomyopathies either are confined to the heart or are a part of generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability
3
Q
Dilated cardiomyopathy
A
Dilatation and impaired contraction of the left or both ventricles (chamber enlargement)

-Ventricular dilation, hypokinetic left ventricle, and systolic dysfunction
-can be inherited
-congestive presentation
4
Q
Hypertrophic cardiomyopathy
A
Left and/or right ventricular hypertrophy, often asymmetrical, which usually involves the interventricular septum.

-Inappropriate myocardial hypertrophy, with or without left ventricular obstruction
5
Q
Restrictive cardiomyopathy
A
Restricted filling and reduced diastolic size of either or both ventricles with normal or near-normal systolic function.

-Abnormal ventricular filling with diastolic dysfunction
6
Q
Arrhythmogenic right ventricular cardiomyopathy
A
Progressive fibro-fatty replacement of the right ventricle

-Fibroadipose replacement of right ventricle
7
Q
Common causes of Dilated Cardiomyopathy
A
1. *Idiopathic*
2. Toxin induced (alcohol, anthracycline, cobalt, catecholamines)
3. Radiation
4. Infection (viral, HIV, parasite)
5. Metabolic (starvation, thiamine deficiency like beriberi, thyrotoxicosis)
6. Sarcoidosis
7. Hemochromatosis
8. Peripartum/postpartum
9. Genetic
8
Q
Dilated cardiomyopathy incidence
A
-most common of all
-myocyte injury and fibrosis
-Third most common cause of heart failure
-Middle age, men > women
-Annual mortality: 12% !
-Most frequent cause of heart transplantation
-complete recovery is rare
9
Q
S/S of Dilated cardiomyopathy
A
1. Pulmonary congestion-left heart failure: dyspnea (rest, excertional, nocturnal), orthopnea

2. Systemic congestion- right heart failure: edema, nausea, abdominal pain, nocturia

3. Hypotension (low CO), tachycardia, tachypnea, fatigue and weakness

4. Arrhythmia
Atrial fibrillation, conduction delays, complex PVC’s, sudden death
10
Q
During dilated cardiomyopathy cardiac exam shows
A
S3, S4 and murmur of TR and/or MR
11
Q
CXR
A
shows enlarged heart, CHF
12
Q
Treatment for dilated cardiomyopathy
A
-Limit activity based on functional status
-Salt restriction
-Fluid restriction
-Diuretics
-Beta Blockers, ACE/ARB (improve mortality)
-Hydralazine/nitrate combination if cannot tolerate ACE/ARB
-Spironolactone if EF
13
Q
Dilated cardiomyopathy is the most common indication for ________
A
cardiac transplantation
Survival after transplant is
80% one year
70% 5 years

-or can do left vent reshaping
14
Q
People with Hypertrophic Cardiomyopathy (HCM) also have:
A
-Small LV cavity
-septal hypertrophy (huge)
-systolic anterior motion of the mitral valve leaflet +/- obstruction of left ventricular outflow
-low stroke volume
-elevated EF
15
Q
Usually patients with Hypertrophic Cardiomyopathy (HCM) are
A
very young

may lead to MI, mitral regurgitation or arrhythmias
16
Q
What type of dysfunction is Hypertrophic Cardiomyopathy and what does it lead to?
A
Diastolic dysfunction (impaired filling, increased filling pressure)

Leads to pulmonary congestion (have LV outflow obstruction and hyper dynamic systolic function bc of so much muscle, especially septum)
17
Q
"Classic" feature of hypertrophic cardiomyopathy is
A
Dynamic left ventricular outflow tract obstruction

(absent in about half of people and obstruction varies greatly)
18
Q
The severity of LV outflow obstruction increases with

hypertrophic obstructive= HOCM
A
**exercise
-positive inotropic agents
volume depletion

Anything that decreases filling:
**sudden assumption of upright posture
-tachycardia
**Valsalva maneuver
19
Q
S/S of hypertrophic cardiomyopathy
A
-Asymptomatic (Echo finding only)
-Dyspnea in 90%
-Angina pectoris in 75% (ISCHEMIC CHEST PAIN)
-Fatigue, pre-syncope, SYNCOPE (incr. risk of SCD)
-Palpitation, PND, CHF, dizziness
-Atrial fibrillation, thromboembolism
-SUDDEN CARDIAC DEATH
20
Q
In HCM, ejection murmur is heard at
A
left sternal border

-does NOT radiate to the carotid arteries
21
Q
HCM, the EKG will show
A
*LVH (Strain pattern) and *abnormal Q waves

(may see giant T wave inversions or LA enlargement, bbb or arrhythmias)
22
Q
In HCM, the valsava will
A
decrease LV volume and INCREASE THE MURMUR

-standing will do this too
23
Q
In HCM, squatting will
A
increase LV volume and DECREASE THE MURMUR
24
Q
Echo findings in HCM
A
-Echocardiogram is best test
-Left ventricular hypertrophy > 1.3 cm
-Septal to posterior wall ratio > 1.3:1
-Mitral regurgitation
-Systolic anterior motion of the mitral valve (SAM)
-Premature midsystolic closure of the aortic valve
-Asymmetric septal hypertrophy (ASH)
-Diastolic dysfunction
-Left ventricular outflow tract obstruction (LVOT)
25
Q
Recommendation when there is a family history of HCM
A
Serial ECHOs are recommended up to the age of 20 yr where there is a family history of HCM

-LVH usually develops between 5-15 y/o
26
Q
Accounts for 36% of deaths in
A
athletes
27
Q
Management of HCM
A
-careful family history focused on sudden cardiac death
-counseling regarding avoidance of strenuous exercise, avoidance of dehydration
-all first-degree family members should be periodically screened with an echo
28
Q
HCM treatment
A
1. FIRST- Beta blockers
-CCB
-Anti-arrhythmics – Amiodarone, disopyramide
-Pacemakers (ICD) / Defibrillators (AICD)
***Myomectomy (resection of septum)
***Alcohol septal ablation (controlled MI through septal perforator perfusing basal septum), leads to wall thinning which decreases LV outflow obstruction
29
Q
Beta blockers help HMC by
A
*NEGATIVE inotropic effect decreases outflow gradient
-decreased myocardial demand results in reduced
ischemia
-prolonged diastolic filling time results in improved LV
filling as well as improved coronary perfusion
-may have an antiarrhythmic effect
30
Q
IN HCM, usually, signs and symptoms _____ with
age
Leading cause of death: _____________
A
increase

sudden death in asymptomatic patients
31
Q
Restrictive Cardiomyopathy (RCM) background
A
-rare in western world
-can affect both ventricles
-systolic fx usually normal (especially early on)
-SMALL ventricular cavity size
32
Q
RCM is a ________ dysfunction and involves __________
A
-DIASTOLIC DYSFUNCTION
-elevated vent filling pressures (small ventricular cavity size)
33
Q
RCM has a ________ with impaired ventricular filling
A
rigid ventricular wall
34
Q
RCM bears some functional resemblance to _________
A
constrictive pericarditis

-Importance lies in its differentiation from operable constrictive pericarditis
35
Q
RCM is much less common then DCM or HCM outside the tropics, but frequent cause of death in ________ primarily because of the high incidence of endomyocardial fibrosis in those regions
A
Africa, India, South and Central America and Asia
36
Q
**Non-infiltrative causes of RCM**
A
*Idiopathic
*Scleroderma
Familial
Hypertrophic
37
Q
Infiltrative causes of RCM
A
**Amyloid** (most common)
*Sarcoid
Gaucher’s
38
Q
Storage disease causes of RCM
A
*Hemochromatosis*
Glycogen storage disease
Fabry’s
39
Q
Endomyocardial causes of RCM
A
*Endomyocardial fibrosis
*Hypereosinophilic syndrom
*Radiation
*Chemotherapy toxicity
Carcinoid
Metastatic malignancy
40
Q
Rigid myocardium --> ______ diastolic vent pressure ---> venous congestion --> ________
A
increased

-Jugular venous distention, Hepatomegaly, Ascites
41
Q
Rigid myocardium--> _____ ventricular filling -->
_____ CO --> ________
A
decreased
decreased
fatigue and weakness
42
Q
in RCM, Echo shows
A
**thick walls, reduced systolic fxn**
-Abnormal mitral inflow pattern
-Prominent E wave (rapid diastolic filling)
-Reduced deceleration time (increased LA pressure)
43
Q
procedure that is commonly necessary in RCM
A
Right heart cath

-can help with endomyocardial biopsy
44
Q
Consider RCM in any patient with
A
R sided heart failure without evidence of either cardiomegaly or systolic dysfunction
45
Q
RCM diagnosis
A
possibly biopsy
46
Q
RCM treatment
A
CHF THERAPY
-No Clear medical therapy
-Treat reversible causes
-Transplant maybe only option
47
Q
GOOD TABLE COMPARING 3 ON SLIDE 56
A
slide 56
48
Q
Arrhythmogenic RV Cardiomyopathy is characterized by
A
fibroadipose replacement of segments of the free wall of the right ventricle
49
Q
Arrhythmogenic RV Cardiomyopathy is predominately found in ____
A
young adults; cause of young adult sudden death
50
Q
Arrhythmogenic RV Cardiomyopathy- all patients who are symptomatic with arrhythmias get
A
ICD implantation