Cardiac Tumors and Misc. Flashcards
cardiac Raynaud
blood vessel hyper-reactivity causing vasospasm
Takotsubo cardiomyopathy
broken heart syndrome
vasospasm caused by extreme stress (emotional), myocardial ischemia, MI, or sudden death due to arrhythmia
prinzmetal angina
less than 50, Japan
variant angina
episodes of angina pectoris at rest
ST elevation
contraction band necrosis
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
venous stasis dermatitis
female, standing, obesity, Hx DVT, farm. Hx of venous disease
erythematous, scaling, eczematous patches affecting lower extremities
HEMOSIDERIN accumulation
MEDIAL ANKLE
venous stasis ulcers
MEDIAL ANKLE over perforating vein (along great or small saphenous vein)
irregular border, tender, shallow, exudative, granulation base
esophageal varices
increased blood flow to the veins of the gastro-esophageal junction
caput medusae
dilation of periumbilical veins of the abdominal wall
hemorrhoids
increased blood flow in veins of rectum
Trousseau syndrome
migratory thrombophlebitis
malignant tumors secrete procoagulant factors causing thrombosis
inferior vena cava syndrome
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
deep vein thrombosis
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
Homans sign
forcefully dorsiflex foot
calf pain: + for DVT
not very sensitive
can send embolus to lung
superior vena cava
neoplasms compress or invade superior vena cava
ex: bronchogenic carcinoma or mediastinal lymphoma
marked dilatation of veins in head, neck, arms associated with cyanosis
chylothorax
lymph from digestive system accumulates in pleural cavity causing pleural effusion
Sturge-Weber syndrome
encephalotrigeminal angiomatosis
facial port wine nevi, ipsilateral venous angiomas in corticol leptomeninges
mental retardation, seizures, hemiplegia, radiopacities of skull
Osler-Weber-Rendu disease
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
hemangioma
tumor of blood vessels
pyogenic granuloma
granulation tissue type hemangioma
lymphangioma
lymph tumor
bacillary angiomatosis
looks like Kaposi
Cause: Bartonella Henselae (cat scratch)
Kaposi sarcoma
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
angiosarcoma
cancer of lining of blood vessels
stent
tube put inside some tubular structure in the body to keep it open
portal hypertension
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
Classic Kaposi sarcoma
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
Endemic African Kaposi sarcoma
younger than 40
indolent or aggressive course
Transplant-associated Kaposi
solid organ transplants with T cell immune suppression
aggressive involving lymph nodes, mucosa, viscera
Tx: attenuation of immunosuppresion at risk of organ rejection
AIDS associated Kaposi
2-3% of AIDS patients
involves lymph nodes and disseminates to viscera early in course
Tx: anti-retrovirals
arterial ulcers
painful, punched out or stellate, surrounding skin is red and taut
ulcer is pale or has black or yellow eschar
cardiac arrhytmia
disturbance in electrical signaling for coordinated cardiac myocyte contraction
long QT syndromes
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
Brugada syndrome
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
catecholeminergic polymorphic ventricular tachycardia
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
cardiomyopathy
diverse group of heart disease primarily involving myocardium
classified by function and structure: can move from one functional category to another
dilated cardiomyopathy
impaired contractility and systolic function
when structural classification: idiopathic (some due to myocarditis or alcohol)
hypertrophic cardiomyopathy
when structural classification: autosomal dominant
impaired compliance and diastolic function
Do NOT confuse with hypertensive heart disease
restrictive cardiomyopathy
impaired compliance and diastolic function
cause: amyloidosis (and others)
alcoholic cardiomyopathy
dilated cardiomyopathy due to alcohol
arrhymogenic right ventricular cardiomyopathy
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
myocarditis
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.
cardiac myxomas
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
rhabdomyomas
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
most common primary sites of cardiac metastases
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
heart transplant rejection
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)
ventricular tachycardia adults
cause: ischemic heart disease (old MI)
re-entry around scar, usually monomorphic
complicatoin: sudden death
normal QT
less than or equal 440ms
congenital long QT syndrome type 1 (LQT1)
loss of function in depolarizing outward K channel (Iks)
congenital long QT syndrome type 2 (LQT2)
loss of function in depolarizing outward K channel (Ikr)
congenital long QT syndrome type 3 (LQT3)
inward Na channel fails to close or inactivate (Ina)
torasades de pointes
polymorphic ventricular tachycardia typical of congenital long QT syndrome
early after depolarization from multiple sites
twisted ribbon
Tx: ICD
asteroid body
cytoplasmic inclusion in giant cells of granulomas
seen in: SARCOIDOSIS, TB, leprosy, fungal infection, schistosomiasis, foreign body
cardiac sarcoidosis
noncaseating granulomas in heart
favors base of heart and often involves conduction system
complications: arrhythmias, HF, sudden death
