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76 Cards in this Set
- Front
- Back
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What are the 2 main types of primary myocardial diseases?
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1. Cardiomyopathy
2. Myocarditis |
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What is Cardiomyopathy?
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A disease that is due to a primary abnormality of the myocardium
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What do doctors mean when they use the diagnosis Cardiomyopathy without an adjective?
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Failure of the heart with unknown etiology
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What is myocarditis?
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Damage and injury of the myocardium due to inflammation
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What are the 3 types of Cardiomyopathy? Which is most common?
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1. Dilated cardiomyopathy**
2. Hypertrophic cardiomyopathy 3. Restrictive cardiomyopathy Dilated is most common. |
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What is the dysfunction in Dilated cardiomyopathy?
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Systolic dysfunction
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What is dilated cardiomyopathy really?
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Congestive heart failure
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How do we assess cardiomyopathy?
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By assessing functional capacity
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What is the functional capacity seen in patients with dilated cardiomyopathy?
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They are short of breath even at rest
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What is the typical ejection fraction seen in patients with dilated cardiomyopathy?
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<40%
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What is a normal EF?
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50-65%
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What is the main dysfunction in Hypertrophic and Restrictive cardiomyopathies?
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Diastolic dysfunction; systolic function is really quite good.
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How do we know that systolic function is good in Hypertrophic and Restrictive cardiomyopathy?
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The EF's are normal or even above normal.
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What is the age of patients that typically will be seen with Dilated cardiomyopathy? What is it called clinically?
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20-60 yo
Progressive CHF |
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What is the hallmark symptom of dilated cardiomyopathy?
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EF <25% and even down into the single digits
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What happens to 50% of patients with dilated cardiomyopathy and progressive CHF within 2 years?
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Death
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What are the 3 main causes of death in patients with dilated cardiomyopathy?
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-CHF
-Arrythmia -Embolism |
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What are the 3 main changes that we see in the heart as dilated cardiomyopathy progresses?
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-Progressive myocardial hypertrophy
-Chamber dilation -Contractile dysfunction |
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What do quite a large number of patients with dilated cardiomypathy have as an etiology?
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It's not known - idiopathic
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What is a possible cause of the idiopathic cases of dilated cardiomyopathy?
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End stage myocarditis that occured years or decades prior to the dilated cardiomyopathy.
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What is the evidence that end stage myocarditis can be manifested as dilated cardiomyopathy?
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Morphologic findings of small areas of inflammation in the heart due to an antecedent viral infection of Coxsackie A or B.
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What is supporting evidence that Coxsackie A/B myocarditis is the etiology of "idiopathic" dilated cardiomyopathy?
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Serologic findings of antibody to Coxsackie A/B.
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What are 3 toxins that can cause dilated cardiomyopathy?
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1. Alcohol
2. Adriamycin 3. Catecholamines |
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What type of alcohol in particular causes dilated cardiomyopathy?
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Beer
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What is a good thing about Beer cardiomyopathy?
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It is reversible - stopping drinking can dramatically improve stage 3-4 patients so that they don't even need heart transplants.
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What is Adriamycin?
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A chemotherapeutic agent used in osteosarcoma of children
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Why is Adriamycin a cause of dilated cardiomyopathy?
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It causes a particular pathology in the heart that can be seen on EM
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Why is Adriamycin toxicity particularly bad?
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Because it is not reversible after causing damage to the heart.
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So what is the sad thing about Adriamycin?
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It can cure a child's osteosarcoma, only to have them develop dilated cardiomyopathy and die from that or have a heart transplant.
(talk about a bad day) |
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What % of dilated cardiomyopathies are due to genetic abnormalities?
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20-30%
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What is the most common metabolic cause of dilated cardiomyopathy, and in what populations is it seen?
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Hemochromatosis
-seen in northern/central europeans |
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Why does hemochromatosis cause dilated cardiomyopathy?
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Iron in the ferric form is deposited in the heart which is very toxic and leads to progressive heart failure.
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Is dilated cardiomyopathy due to hemochromatosis reversible? How?
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Yes - by taking the iron away by bleeding the person
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What is another metabolic disease that can cause dilated cardiomyopathy?
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Thyroid disease - either
-Hyperthyroidism or -Hypothyroidism |
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What is hyperthyroid caused cardiomyopathy also called? Why?
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High output heart failure - because it stimulates the heart to contract more and more vigorously - like running a marathon where you collapse at the end.
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What is hypothyroid-caused cardiomyopathy similar to?
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All the other cardiomyopathies we're talking about - low output.
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What neuromuscular diseases cause dilated cardiomyopathy?
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Muscular dystrophies
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How do we treat muscular dystrophy induced cardiomyopathies? Why?
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With transplant - because some of these inherited diseases have a relatively long life expectancy.
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What is Peripartum cardiomyopathy?
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Development of severe congestive heart failure on a mother just after or during childbirth.
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What is the mechanism of peripartum cardiomyopathy?
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Unknown
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What are 3 gross changes seen in dilated cardiomyopathy?
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-Enlarged heart (2-3x normal)
-Dilated chambers -Mural thrombi |
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What are the vessels and valves like in dilated cardiomyopathy?
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Normal
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What are 3 microscopic changes seen in dilated cardiomyopathy?
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-Hypertrophic myocardial fibers
-Large hyperchromatic boxcar nuclei -Patchy interstitial fibrosis |
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So what % of cardiomyopathies are Dilated cardiomyopathy?
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95%
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What are the other 2 types of cardiomyopathy which are much much less common?
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-Hypertrophic cardiomyopathy
-Restrictive cardiomyopathy |
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What are 2 other names for hypertrophic cardiomyopathy?
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-Idiopathic hypertrophic subaortic stenosis
-Hypertrophic obstructive cardiomyopathy |
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So what basically is going on in hypertrophic cardiomyopathy?
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There is hypertrophy of the OUTFLOW tract so that when the heart contracts, it can't push the blood out through all that muscle.
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What are the 2 main problems in hypertrophic cardiomyopathy?
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-Abnormal diastolic filling
-LV outflow obstruction |
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What are the 2 main clinical symptoms seen in patients with hypertrophic cardiomyopathy?
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Exertional dyspnea
Angina |
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What causes the angina?
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Ischemia due to lack of filling during diastole
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When do the coronary arteries have the most blood flow?
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During diastole
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What are the 3 complications seen in hypertrophic cardiomyopathy?
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-Atrial fibrillation / emboli
-Intractible CHF -Ventricular arrythmias |
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What patients is hypertrophic cardiomyopathy commonly seen in? How is it manifested?
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Young athletes, manifested as sudden death
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What causes hypertrophic cardiomyopathy in half of the patients with it?
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A familial autosomal dominant gene that causes protein abnormalities.
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What are the 2 hallmark findings on gross exam of hypertrophic cardiomyopathic hearts?
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-Septal muscle hypertrophy
-Left ventricle that is banana shaped |
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So how is it that the EF is high in hypertrophic cardiomyopathy, but ventricular filling is low during diastole?
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The ventricle is so small bc it is banana shaped; all the blood in it gets pumped out, but that's not a lot of blood.
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Where are the microscopic findings in hypertrophic cardiomyopathy seen?
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In the SEPTUM
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What are the 3 microscopic findings in hypertrophic cardiomyopathy?
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-Myocytes of septum in disarray
-Interstitial fibrosis of septum -Hypertrophy of the myocytes |
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What is the major problem in restrictive cardiomyopathy?
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Decreased myocardial compliance resulting in impaired diastolic filling
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What are 5 conditions associated with Restrictive cardiomyopathy?
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-Post-radiation therapy fibrosis
-Amyloidosis -Sarcoidosis -Metastatic tumors -Inborn errors of metabolism |
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What is sarcoidosis?
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A noncaseating granulomatous disease
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Where are the noncaseating granulomas found in sarcoidosis?
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In a number of organs in the body - more commonly in lymph nodes and the lung
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What is the etiology of sarcoidosis? How is it treated?
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Unknown
Not usually treated; the granulomas tend to just dissolve and go away. |
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What do you have to do if the heart is involved in sarcoidosis? Why?
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Treat it - because the granulomas tend to change into dense scars in the heart.
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Why is it bad when granulomas change into dense scars in the heart?
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It leads to diastolic filling problems
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What part of the heart do the granulomas in sarcoidosis have a propensity for?
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The conducting system
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So what is seen in patients with sarcoid heart disease?
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Serious Arrythmias
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What is used to treat/manage sarcoidosis? In heart disease?
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Immunosuppressive therapy - except sarcoid heart disease doesn't respond well.
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So what is particularly bad about sarcoid heart disease?
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It is a life threatening situation.
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What people are particularly interested in right ventricular cardiomyopathy? Why?
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Medical examiners - because it is commonly manifested as sudden death.
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What is another name for RV cardiomyopathy?
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Arrythmogenic RV dysplasia
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What is seen grossly in RV cardiomypathy?
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Hypoplastic thin right ventricle
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What is the right ventricle basically made of in people with RV cardiomyopathy?
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Fat and some fiber
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Can you lead an apparently normal life without a RV?
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yes
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When does RV cardiomyopathy become evident?
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When there are other problems with the heart ie fatal ventricular arrythmia
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What are half of the cases of RV cardiomyopathy?
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Familial defects in chromosome 14
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