Flashcards in Cardiomyopathies Deck (44):
What are symptoms of increased left atrial pressure?
dyspnea on exertion
What are symptoms of increased right atrial pressure?
What are some physical findings of patients with cardiomyopathy?
What is the most useful test in diagnosing the type and severity of cardiomyopathy?
What characterizes dilated CM?
dec systolic function
diameter of heart is inc, heart muscle often thins
What are risks associated with DCM?
prone to HF, arrythmias, LV thrombi
What are some causes of DCM?
alcohol, cocaine, amphetamines, cobalt, lead, mercury, CO, berylium
What is the clinical presentation of DCM?
heart failure sx - 75-85%
anginal chest pain - 8-20%
embolic events - 1-4%
syncope - < 1%
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
What are structural findings in DCM?
four chamber dilation
impaired left +/- right ventricular systolic function
normal coronary arteries
heart is bigger
What are CXR findings in DCM?
enlarged cardiac silhouette
interstitial edema (peribronchial cuffing, pulmonary edema)
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
What is the most common genetic CV disorder?
HCM is familial in what % of cases? What is its pattern of inheritance?
Is the primary problem in DCM systolic or diastolic dysfunction? In HCM?
DCM - systolic dysfunction
HCM - diastolic dysfunction
What is the most common variant of HCM?
asymmetrical septal hypertrophy
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
Would you see ischemia in HCM?
Yes - hard to supply blood to all that muscle!
Are pts with HCM prone to arrythmias?
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
What can cause LVOT in HCM?
bulge of septum blocks LVOT
anterior displacement of mitral valve leaflet blocks LVOT (SAM)
What are sx in pts with HCM?
dyspnea - 90%
angina pectoris - 75%
fatigue, pre-syncope, syncope
What may you find on physical exam in pts with HCM?
Bisfierans pulse (double impulse of pulse)
murmur of outflow obstruction
murmur of mitral regurgitation (SAM)
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)
diuretics can help to lower ventricular pressure and alleviate sx
Rx LVOT: alcohol septal ablation, myomectomy
if high risk for ventricular arrythmias: ICD
Progression to DCM occurs in what % of HCM patients?
What are risk factors for SCD?
abnormal blood pressure response to exercise
LV septal thickness > 30mm
severe LVOT obstruction
What are characteristics of RCM?
severe impairment of LV filling
normal systolic function
The clinical differentiation between RCM and ____ is a major issue. What imaging modality can be used to differentiate the two?
What are myocardial causes of RCM?
non-infiltrative: idiopathic, scleroderma
infiltrative: amyloidosis, sarcoid, Gaucher or Hurler disease
What are endomyocardial causes of RCM?
What is the clinical presentation of RCM?
right and left HF sx
fatigue, dyspnea, peripheral edema
How do we Rx RCM? What is the prognosis?
treat underlying cause
diuretics for Sx
What are the characteristics of arrythmogenic right ventricular CM/dysplasia? What is the pathology?
RV systolic dysfunction (contractility goes down)
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
What is Rx for ARVC? What is the prognosis?
standard HF therapy
prone to SCD and RV dysfunction
What is left ventricular non-compaction?
intrauterine arrest of LV compaction
impaired systolic function
prone to arrhythmias
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
What does an S4 indicate?
late diastolic sound, low pitched
atrial kick into a stiffened left ventricle
associated with conditions that cause LVH
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.
What are causes of LVH?
valvular disease - MR, AI can cause eccentric hypertrophy with cavity dilation - pressure overload can cause concentric hypertrophy (AS, coarctation
other rare infiltrative diseases (eg hemochromatosis)
What is the mechanism of dyspnea in HCM?
elevated EDP in LV due to diastolic dysfunction
Why are pts with HCM prone to AF?
high LA pressure due to high ventricular diastolic pressures
Why can't pts with HCM tolerate AF?
severe diastolic dysfunction
loss of atrial kick
reduced diastolic filling time
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