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18 Cards in this Set

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Define Cardiomyopathy
CARDIOMYOPATHY:
1) Denotes numerous conditions of varying etiology

2) Primary disease of the muscle tissue of the heart

3) Secondary disease that specifically damages the muscle tissue of the heart
Most common symptom of cardiomyopathy?
Most common symptom of CARDIOMYOPATHY:

Dyspnea, with other Si/Sx of heart failure
How do you diagnose cardiomyopathy?
-Echocardiography is important in all patients

-Diagnostic test will depend to some extent on the specific case
What are the physiologic classifications of cardiomyopathy?
1) Congestive/Dilated
2) Restrictive
3) Hypertrophic
-Obstructive: HOCM
-Nonobstructive: HCM
Define Congestive/Dilated cardiomyopathy

What is the heart's common adaptive mechanism to this?
CONGESTIVE/DILATED CARDIOMYOPATHY:

Denotes numerous causes of a heart with markedly depressed systolic performance

-Common adaptive mechanism to this is for the heart to move further right in the pressure-volume plane

-Tissue does not undergo compensatory hypertrophy
Etiologies of Congestive/Dilated cardiomyopathy
ETIOLOGY CONGESTIVE/DILATED CARDIOMYOPATHY

1) Idiopathic

2) Viral
-May have viral prodrome of fever, flu-like symptoms, followed weeks later by presentation of heart failure
-Most often Coxsackie B virus

3) Post-partum cardiomyopathy
CHF develops weeks-to-months after delivery
-May recover – pregnancy may again cause this problem

4) Alcoholic cardiomyopathy
EtOH is an acute cardiodepressant
Some controversy whether this exists
What are two other subtypes of Congestive/Dilated cardiomyopathy ?
CONGESTIVE/DILATED CARDIOMYOPATHY SUBTYPES:

1) Hypertensive cardiomyopathy
-in longstanding, poorly controlled hypertension the compensatory capacity is ultimately lost and heart failure occurs

2) Ischemic cardiomyopathy
-Occasionally in a patient with severe, diffuse CAD and no anginal history the presentation is that of heart failure
Define Restrictive cardiomyopathy

What is the heart's common adaptive mechanism to this?
RESTRICTIVE CARDIOMYOPATHY:
Conditions that lead to stiffness of the muscle

Compensation:
1) Filling of the left ventricle thus requires higher pressure
2) This leads to elevated pulmonary capillary pressure with venous congestion and symptoms of dyspnea
Etiologies of Restrictive cardiomyopathy
RESTRICTIVE CARDIOMYOPATHY ETIOLOGIES:

1) Amyloidosis
Proteinaceous material infiltrates the myocardium

2) Sarcoidosis
Systemic granulomatous disease that infrequently
involves the heart

3) Hemochromatosis
Abnormal iron homeostasis – may involve the heart
How does Sarcoidosis lead to Restrictive cardiomyopathy?
SARCOIDOSIS-

-95% of cases have extra cardiac involvement

-Abnormal CXR (hilar adenopathy)

-Common Cardiac manifestations include heart block, dysrhythmia, congestive heart failure
How does Hemochromatosis lead to Restrictive cardiomyopathy?
HEMOCHROMATOSIS:

-Markedly increased deposition of iron

-Generally a genetic condition (HFE gene)

-May be secondary in patients requiring many transfusions (thalassemia)

-May or may not have dilated cardiomyopathy

-Liver, pancreas, skin also involved
How to clinically distinguish restrictive cardiomyopathy from constrictive pericarditis
In Restrictive:
Pressure equilibration at end-diastole much closer in constriction
Hypertrophic Cardiomyopathy defined, etiology, clinical consequences
HYPERTROPHIC CARDIOMYOPATHY
-Presence of myocardial hypertrophy that is not compensatory for altered loading condition

-Numerous genetic substrates – these are often familial

-These conditions are often the cause of sudden death in young athletes
Non-obstructive Hypertrophic Cardiomyopathy defined

Symptoms similar to?
Ultimately deteriorate to?
Often associated with?
NON-OSBSTRUCTIVE HYPERTROPHIC CARDIOMYOPATHY
1) Muscle bound heart that contracts strongly but can’t fill

2) Similar symptoms to restrictive cardiomyopathy

3) Ultimately may deteriorate to dilated state

4) Associated with increased incidence of ventricular dysrhythmias
Obstructive Hypertrophic Cardiomyopathy defined

What phase of the cardiac cycle is most affected?
HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY
1) Specific distribution of pathological hypertrophy on the intraventricular septum beneath the aortic valve

2) During systole this tissue causes a pressure gradient in the outflow tract of the LV

3) Dynamic outflow obstruction
Histologic picture and associated pathology of Hypertrophic Obstructive Cardiomyopathy
HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY:

1) Specific histological picture of myocardium: whorls of myocytes are seen, not typical linear pattern

2) This distribution occurs throughout the LV, not only in the septum

3) Associated with increased ventricular dysrhythmia and sudden cardiac death
Physical exam findings of Hypertrophic Obstructive Cardiomyopathy
HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY:

1) Physical exam will vary depending on how full the heart is at the onset of systole

2) The fuller the heart, the longer until the murmur is heard

3) Can be detected on physical examination
Treatment of Hypertrophic Obstructive Cardiomyopathy
HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY:

1) Treatment is septal resection or septal ablation

2) Mechanical abatement of the gradient does not reduce incidence of dysrhythmias