Cardiomyopathies Flashcards Preview

Cardiology > Cardiomyopathies > Flashcards

Flashcards in Cardiomyopathies Deck (44):
1

What are symptoms of increased left atrial pressure?

dyspnea on exertion
orthopnea
PND
pulmonary crackles

2

What are symptoms of increased right atrial pressure?

elevated JVP
peripheral edema
ascites
weight gain

3

What are some physical findings of patients with cardiomyopathy?

increased JVP
murmur
crackles
edema

4

What is the most useful test in diagnosing the type and severity of cardiomyopathy?

Echo

5

What characterizes dilated CM?

dilatation
impaired contraction
dec systolic function
diameter of heart is inc, heart muscle often thins

6

What are risks associated with DCM?

prone to HF, arrythmias, LV thrombi

7

What are some causes of DCM?

viruses
idiopathic
myocarditis
CT diseases
HIV
gene mutations
alcohol, cocaine, amphetamines, cobalt, lead, mercury, CO, berylium
thyroid disease
toxic meds
pregnancy

8

What is the clinical presentation of DCM?

heart failure sx - 75-85%
anginal chest pain - 8-20%
embolic events - 1-4%
syncope - < 1%

9

What might you see on physical exam in DCM/

dec CO: tachycardia, dec bp, cool extremities, pulsus alterans

pulmonary venous congestion: crackles, pleural effusion

precordial exam: laterally displaced apex, signs of LV dilation, S3, mitral or tricuspid regurgitation murmur

systemic congestion: inc JVP, inc liver size, ascites, peripheral edema

10

What are structural findings in DCM?

four chamber dilation
impaired left +/- right ventricular systolic function
interstitial fibrosis
myocyte hypertrophy
AV regurgitation
normal coronary arteries
heart is bigger

11

What are CXR findings in DCM?

enlarged cardiac silhouette
vascular redistribution
interstitial edema (peribronchial cuffing, pulmonary edema)
pleural effusions

12

What is Rx for DCM?

Reduce mortality/improve sx: ACE-Is, beta-blockers*, +/- aldosterone antagonist, +/- ICD, +/- Bi pacemaker

improve sx: diuretics +/- digoxin +/- nitrates

*short term use only - can blunt sympathetic drive in long term

13

What is the most common genetic CV disorder?

HCM

14

HCM is familial in what % of cases? What is its pattern of inheritance?

55%
AD transmission

15

Is the primary problem in DCM systolic or diastolic dysfunction? In HCM?

DCM - systolic dysfunction
HCM - diastolic dysfunction

16

What is the most common variant of HCM?

asymmetrical septal hypertrophy

17

What type of valve insufficiency may you see with HCM?

mitral regurgitation - because of systolic anterior motion (SAM) of
the anterior leaflet of the mitral valve, which is dragged into and obstructs LVOT

18

Would you see ischemia in HCM?

Yes - hard to supply blood to all that muscle!

19

Are pts with HCM prone to arrythmias?

Yes

20

What is the physiology behind diastolic dysfunction in HCM? How does this effect SV?

increased LV cavity stiffness
impaired relaxation --> inc LV EDP at any diastolic volume --> preload decreases bc higher than normal EDP in LV --> SV decreases

hypertrophy of walls --> smaller chambers --> EDV (preload) decreases --> SV decreases

21

What can cause LVOT in HCM?

bulge of septum blocks LVOT
anterior displacement of mitral valve leaflet blocks LVOT (SAM)

22

What are sx in pts with HCM?

usually asx

if sx:
dyspnea - 90%
angina pectoris - 75%
fatigue, pre-syncope, syncope

23

What may you find on physical exam in pts with HCM?

Bisfierans pulse (double impulse of pulse)
S4
murmur of outflow obstruction
murmur of mitral regurgitation (SAM)

24

What are Rx for HCM?

beta-blockers (reduce myocardial oxygen demand, lessen any LV outflow gradient during exercise by reducing the force of contractin, inc passive diastolic filling by dec heart rate, dec frequency of ventricular ectopic beats)
CCBs
disopyramide (antiarrythmic)
diuretics can help to lower ventricular pressure and alleviate sx

Rx LVOT: alcohol septal ablation, myomectomy

if high risk for ventricular arrythmias: ICD

25

Progression to DCM occurs in what % of HCM patients?

10-15%

26

What are risk factors for SCD?

family hx
gene mutations
aborted SCD
syncope
abnormal blood pressure response to exercise
non-sustained VT
LV septal thickness > 30mm
severe LVOT obstruction

27

What are characteristics of RCM?

non-dilated ventricles
severe impairment of LV filling
diastolic dysfunction
normal systolic function

28

The clinical differentiation between RCM and ____ is a major issue. What imaging modality can be used to differentiate the two?

constrictive pericarditis
Echo

29

What are myocardial causes of RCM?

non-infiltrative: idiopathic, scleroderma
infiltrative: amyloidosis, sarcoid, Gaucher or Hurler disease
storage disease

30

What are endomyocardial causes of RCM?

fibrosis
hypereosinophilic syndrome
carcinoid syndrome
metastatic malignancies
radiation therapy
anthracycline

31

What is the clinical presentation of RCM?

right and left HF sx
fatigue, dyspnea, peripheral edema
elevated JVP

32

How do we Rx RCM? What is the prognosis?

treat underlying cause
diuretics for Sx
prognosis poor

33

What are the characteristics of arrythmogenic right ventricular CM/dysplasia? What is the pathology?

RV systolic dysfunction (contractility goes down)
ventricular arrhythmias

myocardium of the right ventricular free wall is replaced by fibrous and/or fibro-fatty tissue
inherited problem with proteins that join cell to cell

34

What is Rx for ARVC? What is the prognosis?

standard HF therapy
ICD
prone to SCD and RV dysfunction

35

What is left ventricular non-compaction?

embryological condition
intrauterine arrest of LV compaction
DCM
impaired systolic function
prone to arrhythmias

36

What is stress-induced (takotsubo) cardiomyopathy? What is the prognosis? What modality can you use to differentiate it from MI?

transient systolic dysfunction of LV often provoked by stress - catecholamine rush - tend to get impaired LV function - can fully recover - typically have chest discomfort

ECG makes it difficult to differentiate from MI, get cath

37

What does an S4 indicate?

late diastolic sound, low pitched
atrial kick into a stiffened left ventricle
associated with conditions that cause LVH

38

In a pt with LVH and systolic murmur, what is the DDx? What can be done to determine which is the correct Dx?

aortic stenosis or HCM

early peaking --> HCM

late peaking --> severe AS with LVH

Valsalva maneuver: murmur would be louder with HCM and quieter or the same with AS.

39

What are causes of LVH?

htn**
valvular disease - MR, AI can cause eccentric hypertrophy with cavity dilation - pressure overload can cause concentric hypertrophy (AS, coarctation
HCM
Fabry's disease
other rare infiltrative diseases (eg hemochromatosis)

40

What is the mechanism of dyspnea in HCM?

elevated EDP in LV due to diastolic dysfunction

41

Why are pts with HCM prone to AF?

high LA pressure due to high ventricular diastolic pressures

42

Why can't pts with HCM tolerate AF?

severe diastolic dysfunction
loss of atrial kick
reduced diastolic filling time

43

How can pts with HCM develop pulmonary htn? Peripheral edema/ascites? How can this be treated and what cautions must be taken?

rise in LV EDP
LA pressure rises
pulmonary venous pressures rise
pulmonary congestion & edema
pressures can back up into right heart --> peripheral edema & ascites
rise in venous pressures also occurs due to activation of RAAS
Rx: diuretics with caution bc elevated venous pressures are needed to fill the less compliant ventricle

44

What is the most NB sx for pts with HCM to look out for?

syncope or weak spells