Cardiomyopathy

Question 1

what are the 3 types of cardiomyopathy

Answer 1

Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy

Question 2

Cardiomyopathy

Answer 2

Primary abnormality of the myocardium

Cardiac dysfunction not attributable to pressure or volume overload (issue with the muscle itself)

Question 3

which type of cardiomyopathy is the most common

Answer 3

dilated cardiomyopathy

Question 4

features of dilated cardiomyopathy

Answer 4

Contractile (systolic) dysfunction (biventricular failure)
Four chamber dilatation
2-3x normal weight

Question 5

treatment for dilated cardiomyopathy

Answer 5

medical therapy (meds)
heart transplant

Question 6

Hypertrophic cardiomyopathy is also called what 2 other names

Answer 6

“Idiopathic hypertrophic subaortic stenosis” (IHSS)

“Hypertrophic obstructive cardiomyopathy” (HOCM)

Question 7

what is the main feature of hypertrophic cardiomyopathy

Answer 7

Marked LV myocardial hypertrophy

Septum > free wall hypertrophied = Banana shaped LV cavity

Question 8

how does hypertrophic cardiomyopathy affect the hearts function

Answer 8

Abnormal diastolic filling

Question 9

describe the gross and histologic findings in hypertrophic cardiomyopathy

Answer 9

Asymmetric septal hypertrophy - narrow LV chamber

histological : Hypertrophied myocytes (large cells and nuclei), Haphazard pattern, Interstitial fibrosis (hypertrophy + disorder is unique to hypertrophic cardiomyopathy)

Question 10

is there a genetic component to Hypertrophic cardiomyopathy

Answer 10

yes - nearly 100% familial
Autosomal dominant (Variable expression)
Mutations in genes that encode proteins of sarcomeres

Question 11

both dilated and hypertrophied cardiomyopathy lead to higher risks of

Answer 11

heart failure
sudden death
atrial fibrillation
stroke

Question 12

signs of Hypertrophic cardiomyopathy

Answer 12

Diastolic heart failure
Exertional dyspnea
Harsh systolic ejection murmur
Anginal pain
Intractable heart failure
Arrhythmias

Question 13

Hypertrophic cardiomyopathy treatment

Answer 13

Medical therapy enhancing ventricular relaxation (beta blockers, and Ca channel blockers)
Surgical excision of muscle (septum)

Question 14

Restrictive Cardiomyopathy

Answer 14

Primary decrease in ventricular compliance

Impeded left ventricular filling during diastole
Systolic function preserved

Question 15

Restrictive Cardiomyopathy characteristics of the heart anatomy

Answer 15

Firm/stiff myocardium
-Ability of LV cavity to expand markedly limited

LV cavity normal size
LA size increased

Question 16

Amyloid

Answer 16

Misfolded protein
Deposits in extracellular space
Causes tissue damage

Multiple proteins can deposit as amyloid

Question 17

Common features of amyloid deposits, regardless of the protein

Answer 17

“β-pleated sheet” configuration

Congo red staining in tissue- looks salmon
“apple-green” under polarized light

Question 18

restrictive cardiomyopathy is caused by

Answer 18

amyloid deposits in the heart muscle

Question 19

restrictive cardiomyopathy signs and symptoms are due to

Answer 19

reduced myocardial compliance and stroke volume

amyloids make the tissue stiff and less contractile

Question 20

Myocarditis

Answer 20

Inflammatory process resulting in myocardial injury

Question 21

myocarditis is most commonly caused by

Answer 21

Enteroviruses - Coxsakie A and B

Most common etiology in US

Question 22

myocarditis is caused by what types of organisms

Answer 22

viruses
bacteria
parasites

can also be immune mediated

Question 23

common causes of immune mediated myocarditis

Answer 23

hypersenstivity reactions
rheumatic fever (characteristicly has aschoff bodies)

Question 24

what are the histologic differences between etiologies of myocarditis

Answer 24

viral - see lots of lymphocytes
hypersensitivity - see eosinophils
parasites- see the organism within the myocytes

Question 25

myocarditis clinical presentations

Answer 25

large range may be asymptomatic or have nonspecific symptoms like fatigue or fever or can be critically ill or sudden cardiac death Acute congestive heart failure Arrhythmias Progression to dilated cardiomyopathy (can occur years after the infection)

Question 26

Pericardial Effusion

Answer 26

accumulation of fluid within the pericardial sac. Can be clear yellow serous fluid, blood, or pus

Question 27

symptoms of slow accumulating pericardial effusion

Question 28

rapidly developing pericardial effusion

Answer 28

Compress atria and vena cavae Compress ventricles Restrict cardiac filling Cardiac tamponade*

Question 29

Pericarditis

Answer 29

Inflammation of pericardium | Usually secondary to cardiac, thoracic, or systemic process

Question 30

causes of pericarditis

Answer 30

Infections Viruses, Bacteria, TB, Fungi, Parasites Immune-Mediated Reactions Rheumatic fever, SLE, Post-cardiotomy, Post-myocardial infarction, drug hypersensitivity Uremia Neoplasia Trauma Radiation

Question 31

Dressler syndrome

Answer 31

is a secondary form of pericarditis that occurs in the setting of injury to the heart or the pericardium (the outer lining of the heart). It consists of fever, pleuritic pain, pericarditis and/or a pericardial effusion.

Question 32

Fibrinous Pericarditis characteristics

Answer 32

Pericardial surface irregular, “shaggy” Acute viral pericarditis Uremia Acute rheumatic fever (bread and butter pericarditis) Exam: Pericardial friction rub

Question 33

Suppurative (Fibrinopurulent) Pericarditis

Answer 33

Acute bacterial infection (lots of neutrophils) Extension (primarily of the heart) Seeding

Question 34

Hemorrhagic Pericarditis

Answer 34

Tuberculosis | Malignancy

Question 35

Pericarditis symptoms

Answer 35

``` Silent Chest pain (worse laying down, better sitting up) Systemic complaints Friction rub EKG changes (diffuse ST elevation) ```

Question 36

Pericarditis symptoms

Answer 36

``` Silent Chest pain (worse laying down, better sitting up) Systemic complaints Friction rub EKG changes (diffuse ST elevation) ```

Question 37

Pericarditis healing process

Answer 37

Focal plaque-like thickening Mild adhesions Constrictive pericarditis Pericardial space obliterated - Heart surrounded by dense scar - Diastolic expansion limited - Reduced cardiac output (TB, suppurative) ultimately a fibrotic process - makes the pericardia constrictive

Question 38

Constrictive Pericarditis treatment

Answer 38

Pericardiectomy | Surgical removal of constricting pericardium

Question 39

Cardiac Transplantation complications

Answer 39

Acute Rejection Chronic rejection Infections Post transplant lymphoma (Epstein-Barr virus) Late progressive diffuse stenosing intimal proliferation of coronary arteries 70-80% 1 year survival >60% 5 year survival

Question 40

How are “heart biopsies” obtained?

Answer 40

Bioptome inserted transvenously into right side of heart | Septal endomyocardium biopsied