Cardiac Tumors and Misc.

Question 1

cardiac Raynaud

Answer 1

blood vessel hyper-reactivity causing vasospasm

Question 2

Takotsubo cardiomyopathy

Answer 2

broken heart syndrome

vasospasm caused by extreme stress (emotional), myocardial ischemia, MI, or sudden death due to arrhythmia

Question 3

prinzmetal angina

Answer 3

less than 50, Japan
variant angina
episodes of angina pectoris at rest
ST elevation

Question 4

contraction band necrosis

Answer 4

reperfusion of dead myocardium, coronary artery vasospasm
cardiac myocytes that run out of ATP and then are exposed to an influx of Ca: enhances actin-myosin interaction causing hyper contraction and cells that cannot relax

Question 5

venous stasis dermatitis

Answer 5

female, standing, obesity, Hx DVT, farm. Hx of venous disease
erythematous, scaling, eczematous patches affecting lower extremities
HEMOSIDERIN accumulation
MEDIAL ANKLE

Question 6

venous stasis ulcers

Answer 6

MEDIAL ANKLE over perforating vein (along great or small saphenous vein)
irregular border, tender, shallow, exudative, granulation base

Question 7

esophageal varices

Answer 7

increased blood flow to the veins of the gastro-esophageal junction

Question 8

caput medusae

Answer 8

dilation of periumbilical veins of the abdominal wall

Question 9

hemorrhoids

Answer 9

increased blood flow in veins of rectum

Question 10

Trousseau syndrome

Answer 10

migratory thrombophlebitis

malignant tumors secrete procoagulant factors causing thrombosis

Question 11

inferior vena cava syndrome

Answer 11

neoplasms or thrombus that compress or invade inferior vena cava
hepatocellular and renal cell carcinoma grow in veins: can occlude
Sx: lower extremity edema, distention of superficial collateral veins of lower abdomen

Question 12

deep vein thrombosis

Answer 12

common, increases if male and older
can be fatal if embolizes
association: cancer, hospital, surgery
proximal: popliteal and above, associated with pulmonary emboli
distal: calf
Dx: asymmetry between legs over 2 cm, D dimer
PROLONGED IMMOBILIZATION

Question 13

Homans sign

Answer 13

forcefully dorsiflex foot
calf pain: + for DVT
not very sensitive
can send embolus to lung

Question 14

superior vena cava

Answer 14

neoplasms compress or invade superior vena cava
ex: bronchogenic carcinoma or mediastinal lymphoma
marked dilatation of veins in head, neck, arms associated with cyanosis

Question 15

chylothorax

Answer 15

lymph from digestive system accumulates in pleural cavity causing pleural effusion

Question 16

Sturge-Weber syndrome

Answer 16

encephalotrigeminal angiomatosis
facial port wine nevi, ipsilateral venous angiomas in corticol leptomeninges
mental retardation, seizures, hemiplegia, radiopacities of skull

Question 17

Osler-Weber-Rendu disease

Answer 17

autosomal dominant
hereditary hemorrhagic telangiectasia (present at birth and wide distribution)
mutation in TGF-B pathway in endothelial cells
Sx: epitaxis, GI bleed hematuria when lesions rupture

Question 18

hemangioma

Answer 18

tumor of blood vessels

Question 19

pyogenic granuloma

Answer 19

granulation tissue type hemangioma

Question 20

lymphangioma

Answer 20

lymph tumor

Question 21

bacillary angiomatosis

Answer 21

looks like Kaposi

Cause: Bartonella Henselae (cat scratch)

Question 22

Kaposi sarcoma

Answer 22

HHV-8
tumors on the borderline of benign and malignant
viral G protein induces VEGF, viral cyclin D drives proliferation, multiple KSHV proteins inhibit p53

Question 23

angiosarcoma

Answer 23

cancer of lining of blood vessels

Question 24

stent

Answer 24

tube put inside some tubular structure in the body to keep it open

Question 25

portal hypertension

Answer 25

leads to opening of portal systemic shunt increased flow in veins causes: esophageal varices, hemorrhoids, caput medusae cause: liver cirrhosis ESOPHAGEAL VARICES prone to rupture

Question 26

Classic Kaposi sarcoma

Answer 26

Mediterranean, Middle Eastern, Eastern European, older men associated with malignancy or altered immunity asymptomatic red-purple plaques in distal lower extremities that spread proximally, localized to skin\red-purple macules composed of dilated irregular endothelial cell lined vascular spaces with lymphocytes, plasma cells, macrophages-> larger raised plaques spread proximal with spindle cells, extravasated erythrocytes, hemosiderin-laden macrophages-> nodules with hemorrhage Tx: radiation and chemo good prognosis

Question 27

Endemic African Kaposi sarcoma

Answer 27

younger than 40 | indolent or aggressive course

Question 28

Transplant-associated Kaposi

Answer 28

solid organ transplants with T cell immune suppression aggressive involving lymph nodes, mucosa, viscera Tx: attenuation of immunosuppresion at risk of organ rejection

Question 29

AIDS associated Kaposi

Answer 29

2-3% of AIDS patients involves lymph nodes and disseminates to viscera early in course Tx: anti-retrovirals

Question 30

arterial ulcers

Answer 30

painful, punched out or stellate, surrounding skin is red and taut ulcer is pale or has black or yellow eschar

Question 31

cardiac arrhytmia

Answer 31

disturbance in electrical signaling for coordinated cardiac myocyte contraction

Question 32

long QT syndromes

Answer 32

phase 2 early after-depolarization left ventricle causes: ischemic heart disease, low K, Ca, Mg, chanelopathy, etc. complication: ventricular tachycardia presents in childhood: syncope or sudden death

Question 33

Brugada syndrome

Answer 33

autosomal dominant: Na channel blunted: shortened or failed AP young asian males phase 2 re-entry right ventricle present at 40 yrs: syncope or sudden cardiac death due to ventricular tachycardia esp. in SLEEP abnormal EKG at rest elevated ST descending with upward convexity to inverted T wave in V1-V3 Tx: ICD

Question 34

catecholeminergic polymorphic ventricular tachycardia

Answer 34

genetic phase 4 delayed after-afterdepolarization left ventricle mutations in RYANODINE receptor: high cystolic Ca leaked from SR, get PVT with high levels of catecholamines present in childhood: syncope, sudden cardiac death due to ventricular tachycardia during extreme PHYSICAL or EMOTIONAL STRESS Tx: ICD or beta blockers

Question 35

cardiomyopathy

Answer 35

diverse group of heart disease primarily involving myocardium classified by function and structure: can move from one functional category to another

Question 36

dilated cardiomyopathy

Answer 36

impaired contractility and systolic function | when structural classification: idiopathic (some due to myocarditis or alcohol)

Question 37

hypertrophic cardiomyopathy

Answer 37

when structural classification: autosomal dominant impaired compliance and diastolic function Do NOT confuse with hypertensive heart disease

Question 38

restrictive cardiomyopathy

Answer 38

impaired compliance and diastolic function | cause: amyloidosis (and others)

Question 39

alcoholic cardiomyopathy

Answer 39

dilated cardiomyopathy due to alcohol

Question 40

arrhymogenic right ventricular cardiomyopathy

Answer 40

disease due to mutations in genes encoding desmosomal proteins second hit: enteric viral infection of right heart re-entrant ventricular tachycardia originating from right ventricle related to abnormalities in myocyte adhesion FATTY replacement of myocytes EKG: epsilon wave (notch in terminal QRS most prominent in V1 Tx: ICD

Question 41

myocarditis

Answer 41

infectious agents and/or inflammatory processes primarily target myocardium VIRAL most common can occur in Lyme disease can get hypersensitivity myocarditis complications: acute HF and dilated cardiomyopathy Sx: asymptomatic to dyspnea, fatigue, pain, fever, arrhythmia, etc.

Question 42

cardiac myxomas

Answer 42

rare, females, left atrium benign gelatinous mesenchymal neoplasms of the endocardium Sx: dyspnea, cough, transient neurological symptoms fatigue, FEVER (produce IL-6) signs: loud S1, diastolic rumble, diastolic tumor plop, holosystolic murmur other: orthopnea, hemoptysis, syncope complication: mitral obstruction, embolization, MI, sudden death Dx: echo Tx: surgical excision

Question 43

rhabdomyomas

Answer 43

most frequent pediatric heart tumor, preferentially in ventricles most regress spontaneously discovered in 1st year due to obstruction in heart cause: 1. sporadic mutation 2. tuberous sclerosis with mutations in TSC1/2 (hamartin and tuberin) tumor suppressor genes that inhibit mTOR SPIDER cells

Question 44

most common primary sites of cardiac metastases

Answer 44

lung and breast, melanomas, leukemias, lymphomas retrograde lymphatic extension hematagenous seeding, direct contiguous extension, venous extension usually silent or nonspecific features: defect in contractility or compliance

Question 45

heart transplant rejection

Answer 45

``` lots of inflammatory cells arteriopathy is the most important limitation (will need new transplant eventually): inflammatory cells attack intima and thicken it dx: endomyocardial biopsy tx: more immunosuppression complication: silent MI (denervated) ```

Question 46

ventricular tachycardia adults

Answer 46

cause: ischemic heart disease (old MI) re-entry around scar, usually monomorphic complicatoin: sudden death

Question 47

normal QT

Answer 47

less than or equal 440ms

Question 48

congenital long QT syndrome type 1 (LQT1)

Answer 48

loss of function in depolarizing outward K channel (Iks)

Question 49

congenital long QT syndrome type 2 (LQT2)

Answer 49

loss of function in depolarizing outward K channel (Ikr)

Question 50

congenital long QT syndrome type 3 (LQT3)

Answer 50

inward Na channel fails to close or inactivate (Ina)

Question 51

torasades de pointes

Answer 51

polymorphic ventricular tachycardia typical of congenital long QT syndrome early after depolarization from multiple sites twisted ribbon Tx: ICD

Question 52

asteroid body

Answer 52

cytoplasmic inclusion in giant cells of granulomas | seen in: SARCOIDOSIS, TB, leprosy, fungal infection, schistosomiasis, foreign body

Question 53

cardiac sarcoidosis

Answer 53

noncaseating granulomas in heart favors base of heart and often involves conduction system complications: arrhythmias, HF, sudden death