Myocardial pathology

Question 1

Name (a) the most common primary cardiac neoplasm, (b) the cell type of origin, (c) the most common location, and (d) possible complications

Answer 1

Myxoma is benign neoplasm of mesodermal tissue. Most common in left atrium and complications include embolization (fragments of neoplasm or thrombus associated with mass), or mitral valve obstruction. Most common primary tumor of heart in teens and adults

Question 2

List the possible primary neoplasms in heart and the cell of origin

Answer 2

Myxoma (mesoderm), lipoma (adipose), rhabdomyoma (cardiomyocyte), Angiosarcoma (endothelial cell)

Question 3

lipoma

Answer 3

benign neoplasm of adipose. Most common in LV, right atrium, atrial septum, subendocardial, subepicardial, myocardium. Symptoms include arrhythmias, valve obstruction

Question 4

Rhabdomyoma

Answer 4

Benign neoplasm of muscle fibers. Most common primary tumor in infancy/childhood. Most common in ventricle. Symptoms may include valvular obstruction

Question 5

Angiosarcoma

Answer 5

Malignant endothelial neoplasm located anywhere in heart

Question 6

Name at least one example of (a) a carcinoma and (b) a hematopoietic disease that can affect the heart

Answer 6

carcinomas: breast, lung. Hematopoietic: lymphoma and leukemia. Can affect the heart in the myocardium or pericardial space. Symptoms can include arrhythmias and restrictive cardiomyopathy. Chemo causes dilated cardiomyopathy and radiation causes restrictive cardiomyopathy.

Question 7

Can melanoma metastasize to the heart

Answer 7

yep

Question 8

Myocarditis definition

Answer 8

inflammation of myocardium and myofiber necrosis

Question 9

Name the most common form of viral disease which affects the heart- infiltrative cell types

Answer 9

Enterovirus such as coxsackie A+B, Echo virus. Cuase direct myocardial injury. Lymphocyte infiltrate with macrophages. Often present later as dilated cardiomyopathies

Question 10

Name at least one parasitic disease that affects the heart

Answer 10

Trypansoma cruzi (chagas kissing bug), trichinosis (trichinella spiralis). Mainly eosinophil infiltrate

Question 11

Name at least one fungal entity that affects the heart

Answer 11

Candida, aspergillus, cryptococcus- neutrophils early, mononuclear cells later

Question 12

Name bacteria that affect the heart

Answer 12

Corynebacterium diphtheria (toxin), Neisseria meningitis, lyme disease. Mainlly neutrophil infiltrates

Question 13

Name the bacteria associated with Rheumatic Fever / Rheumatic heart disease

Answer 13

Group A strep (pyogenes)

Question 14

Name the process by which the heart / other organ systems are injured in rheumatic disease

Answer 14

Antibodies against M protein cross-react with glycoproteins of heart

Question 15

What components of heart are affected by rheumatic disease

Answer 15

All three (pan-carditis)- endocarditis causes valve damage/necrosis and fibrosis, myocarditis shows Aschoff bodies (mononuclear cell collections), pericarditis results in fibrosis and adhesions

Question 16

What is an Anitschkow cell

Answer 16

foamy macrophages with central aggregated chromatin. AKA caterpillar cells. Found within an Aschoff body

Question 17

Jones criteria for acute rheumatic fever

Answer 17

evidence of group A strep + 2 major or 1 major and 2minor criteria. Major: Pancarditis, polyarthritis, skin and CNS problems. Minor: fever, joint pain, elevated CRP, prolonged PR interval)

Question 18

Symtpoms of rheumatic fever

Answer 18

arrhythmia, tachycardia, pericardial friction rub,

Question 19

Name (a) at least one example of an autoimmune disease that affects the heart and (b) the component of the heart affected

Answer 19

Systemic autoimmune diseases can involve the heart. Lupus causes fibrinous pericarditis, endocarditis and myocarditis. Scleroderma/systemic sclerosis causes small artery inflammation leading to infarcts and subsequent fibrosis/sclerosis (repair). Rheumatoid arthritis causes granulomatous inflammation that is centrally necrotic. Polyarteritis nodosa cuases necrotizing arteritis leading to myocardial ischemia and arrhythmias.

Question 20

lab testing for autoimmune diseases

Answer 20

antinuclear antibody and anti-neutrophil cytoplasmic antibody test for collagen vascular and connective tissue diseases

Question 21

Name the key histologic finding in sarcoid involvement of the heart

Answer 21

Systemic non-caseating (non-necrotizing) granulomatous disease (idiopathic) with multinucleated giant cell macrophages and lymphocyte infiltration. Patients can have granuloma in heart (left ventricle), but few have symptoms of heart involvement. May have ACE elevation but ANA and RF are negative

Question 22

Name a medication associated with toxic myopathy

Answer 22

Adriamycin (doxorubicin-anthracycline) is a chemo drug that has a cumulative dose dependent toxicity on muscle

Question 23

List other non-medication associated toxins

Answer 23

Ethanol (metabolite acetaldehyde) if person gets most of their calories from ethanol. Also cobalt, exogenous or in some forms of artificial hips

Question 24

Name a glycogen storage disease associated with heart disease

Answer 24

Pompe disease: An acid maltase deficiency results in glycogen accumulation within lysosomes of myocytes. Results in energy deficits.

Question 25

Name an iron metabolism disease associated with heart and other organ system disease and the gene responsible

Answer 25

Hemochromatosis: Increased iron storage in cardiac myocytes most commonly from HFE gene which is mutated (inactivated) thus there is increased transferrin uptake. Also could be due to mutations in transferrin receptor 2, Hemojuvelin, Hepcidin genes. This disease causes dilated cardiomyopathy with impairment of contractility, and variable involvement of other organs (pituitary, liver, skin, pancreas)

Question 26

Hemosiderosis

Answer 26

Secondary hemochromatosis: red cell break down results in iron deposition or excess iron intake results in iron deposition, eventually in the heart

Question 27

Name the disease process where proteins deposit as beta-pleated sheets around blood vessels and in the parenchyma of heart and other organs

Answer 27

Amyloidosis: involves heart, kidneys, nerves, liver, spleen, bone marrow. Can be systemic or local. Systemic includes MGUS (primary amyloidosis with Ig LC deposited in tissues), Rheumatoid arthritis (secondary, acute phase protein)

Question 28

Name at least one dystrophy process that involves the heart

Answer 28

Duchenne and Becker muscular dystrophy: Duchenne has NO dystrophin expression. Becker has reduced dystrophin expression. Long term there is loss of cardiomyocytes

Question 29

Name the form of “Cardiomyopathy” (dilated / restrictive / hypertrophic) associated with a. Dystrophin mutations b. Myosin heavy chain mutationsName the form of “Cardiomyopathy” (dilated / restrictive / hypertrophic) associated with a. Dystrophin mutations b. Myosin heavy chain mutations

Answer 29

Dystrophin mutations: dilated cardiomyopathy. Myosin HC mutations: hypertrophic cardiomyopathy

Question 30

Name possible incidents resulting in traumatic injury to the heart

Answer 30

Blunt trauma(resulting in myocardial damage, arrhythmias, conduction defects, cardiogenic shock and hemopericardium -cardiac tamponade), radiation trauma (resulting in fibrosis and restrictive cardiomyopathy), commotio cordis (blow to chest resulting in cardiac arrest)

Question 31

Name possible sequela of trauma to the heart resulting in: a. Contusion b. Coronary artery tear or thrombosis c. Ventricular rupture d. Aortic rupture

Answer 31

Contusion(bruise): Focal injury to heart. Can also lacerate the heart depending on whether heart is in systole or diastole. Coronary artery tear or thrombosis: Can cause MI. Ventricular rupture: from focal injury during diastole. Aortic rupture: Causes hemopericardium with potential for tamponade.

Question 32

Describe arrhythmogenic right ventricular cariomyopathy

Answer 32

Right ventricle is composed mainly of adipose and connective tissue, with minimal muscle. Results in right ventricular failure, rhythm alterations (Vtach and Vfib)

Question 33

Myxoma: name (a) the most common location and (b) complications

Answer 33

Myxoma is neoplasm of mesodermal tissue. Most common in left atrium and complications include embolization (fragments of neoplasm or thrombus associated with mass), mitral valve obstruction

Question 34

Trauma: what is the name applied when a baseball / hockey puck / punch to the chest results in cardiac arrest and (usually death) (causing a commotion!)

Answer 34

Commotio cordis: Occurs at a vulnerable palce in ventricular rhythm resulting in cardiac arrest.