Cardiomyopathies

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cardiomyopathies

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-heart muscle disease
-mostly idiopathic in nature

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terms

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S1 - sound of mitral valve closure
S2 - Sound of aortic valve closure
S3 - Sound of blood hitting ventricular wall
S4 - Sound of atrial contraction

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ejection fraction

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-the comparison between the amount of blood in the left ventricle at the very beginning of systole and the very end of systole

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systolic dysfunction

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decreased myocardial contractility resulting in a lower EF

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diastolic dysfunction

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abnormal ventricular filling resulting in elevated filling pressure of the left ventricle

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documenting cardiomyopathy

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1. type of cardiomyopathy
2. disease processes involved
3. systolic or diastolic dysfunction
4. ejection fraction- esp with CHF
5. PMH

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dilated cardiomyopathy

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-enlarged left and right ventricles
-thinned myocardium, aka congestive cardiomyopathy
-dec contractility, dec EF, systolic dysfunction
-50% cases idiopathic; result of toxic, genetic, metabolic, AI, infectious
-reversible and non-reversible

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dilated cardiomyopathy- reversible causes

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1. alcohol abuse (cure-complete abstinence)
2. pregnancy
3. hypophosphatemia
4. hypocalcemia
5. uncontrolled tachycardia

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dilated cardiomyopathy non-reversible causes

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1. ischemic causes
2. infectious processes
3. muscular dystrophy
4. chemotherapy agents
5. thoracic radiation treatments

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Right ventricular dysplasia

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-unique for of dilated cardiomyopathy where there is a progressive replacement of right ventricular wall with adipose tissue (fat)

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dilated cardiomyopathy ssx

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1. similar to CHF
2. left sided failure
-PND
-orthopnea
-narrow pulse pressure
-rales
S3
-systolic murmur
3. right sided heart failure- later and ominous
-elevated JVP
-edema
-abd distension
-wt gain
-ascites
4. CP rare!

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dilated cardiomyopathy - testing

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1. CXR- enlarged heart
2. EKG - biphasic p waves, BBB, shift of the axis
3. echo - BEST
4. cardiac angiogram -nml coronaries

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dilated cardiomyopathy- tx and prognosis

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-identical to CHF
1. diuretics
2. digoxin
3. ACEI
4. bblockers
5. nitrates
6. surgery- most common reason for heart transplant
-Poor EF leads ironically to cardiac hypertrophy to maintain homeostasis. CHF with pulmonary congestion provide poor mortality after onset
-mortality related to heart failure and arrhythmias

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dilated cardio- complications

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1. arrhythmias
-tx with ionotropic agents
2. embolization of thrombi
-most pts on long term anticoagulation

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restrictive cardiomyopathy

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-loss of ventricular elasticity
-results in rigidity, impeded ventricular filling, decreased preload
-increased ventricular filling pressure; diastolic dysfunction
-rarer
-Caused by a variety of diseases classified by cause, most genetic in origin

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restrictive cardiomyopathy- infiltrative

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-systemic diseases where proteins accumulate in body tissues

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restrictive cardiomyopathy- storage

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-errors of metabolism that cause accumulation of products in tissues

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restrictive-cardio- Fibrotic

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-radiation treatments
-scleroderma- loss of collagen elasticity

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restrictive cardiomyopathy- metabolic

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-carnitine deficiency

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restrictive cardiomyopathy - idiopathic

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-Endomyocardial Fibrosis - occurs in children and young adults in the tropics
-Loeffler’s Syndrome - Eosinophilia with infiltration into the heart muscle and other tissues

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restrictive cardiomyopathy - ssx

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-right sided heart failure - the result of persistently elevated venous P
1. elevated JVP
2. Kussmauls sign
3. DOE
4. palpitations
5. irregular HR
6. dependent edema
7. ascites
8. hepatomegaly
9. S3, S4

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restrictive cardio- testing

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1. EKG
2. CXR
3. CT/MRI
4. cardiac angiogram
5. endomyocardial bopsy

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Restrictive Cardiomyopathy - tx

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-like CHF
1. diuretics
2. digoxin
3. anticoagulation
4. PPM/AICD placement
5. heart transplant

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Restrictive Cardiomyopathy- prognosis

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Poor prognosis, 30% survival rate at 5 years.
Again, all treatments are palliative except in the case of restrictive cardiomyopathy caused by hemochromatosis

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hypertrophic cardiomyopathy

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-enlargement of the heart muscle
-thick septum
-thickening of the myocardium, esp left ventricle without dilation
-occurs heterogeneously in ventricle
-decreased ventricular vol, inc filling pressures- diastolic dysfunction
-idiopathic in nature: 1/2 of all cases linked to families; 1/3 of echo studies show links to 1st degree relatives

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hypertrophic cardiomyopathy- types

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1. disease state hypertrophic cardiomyopathy
-ischemic heart dz
-aortic stenosis
-HTN
2. idiopathic hypertrophic cardiomyopathy
-idiopathic hypertrophic subaortic stenosis

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systolic anterior motion (SAM)

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-hypertrophied septum lies near mitral valve leaflet
-dec ventricular vol with nml blood vol increases contractility
-dec afterload worsens outflow tract obstruction by allowing higher blood velocity
-the faster the blood moves, the worse the SAM will be (worse the outflow tract obstruction)

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hypertrophic cardiomyopathy- ssx

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1. sudden death
2. dyspnea
3. irregular heartbeat
4. chest pain
5. fatigue
6. syncope, near syncope
7. systolic murmur heard best at R sternal border
8. S4
9. "jerky" carotid pulse

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hypertrophic cardiomyopathy - testing

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1. EKG - may present as a Q wave
2. CXR
3. echo - GOLD STANDARD
4. cardiac angiogram

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Hypertrophic Cardiomyopathy - treatment

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1. lifestyle mod
2. drugs:
-Abx- prevent infective endocarditis
-b-agonists- protect against syncope, not against sudden death
-amiodarone
-CCBs (verapamil and diltiazem)

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Hypertrophic Cardiomyopathy - drugs to avoid

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-Ionotropic agents
1. digoxin
2. diuretics - decreases afterload, blood can leave heart easier, blood leaves faster, increases outflow tract obstruction
3. nitrates - decrease afterload
4. b-blockers

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Hypertrophic Cardiomyopathy - surgical treatments

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1. AICD - prevent arrhythmias
2. myomectomy - removal of hypertrophic muscle
3. ethanol ablation - cardiac catheter injects ETOH into hypertrophied septum

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Hypertrophic Cardiomyopathy - prognosis

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-pts can show stabilization and improvement
-sudden death always a risk from ventricular arrhythmias
1. age <30
2. vent arrhythmias at rest
3. marked hypertrophy
4. FH of sudden death