CV Pathology

Question 1

What are 5 congenital defects that lead to a right to left shunt? What is the presentation of a right to left shunt (congenital)? Treatment?

Answer 1

Early cyanosis; blue babies; diagnosed prenatally or become evident soon after

Surgical correction or maintenance of PDA

5 T’s

Truncus arteriosus (1 vessel)
Transposition of great vessels (2 switched vessels)
Tricuspid atresia (Tri=3)
Tetralogy of Fallot (4)
TAPVR (5 letters)

Question 2

What is the pathophys of persistent truncus arteriosus? What usually accompanies it? Pathophys of transposition? What makes it compatible with life? Treatment? Anatomy of tricuspid atresia? What makes it compatible? What causes tetralogy of fallot? What are the 4 anatomical features? Pathophys/symptoms? What does squatting do? How? Treatment? What is TAPVR? What is it associated with?

Answer 2

PERSISTENT

Failure of aoricopulmonary septum to form
VSD

TRANSPOSITION OF GREAT VESSELS
Aorta leaves RV and pulmonary trunk leaves LV
Failure of AP septum to spiral
Must have mixing of blood (VSD, PDA, or PFO)
Surgical intervention within months to prevnt death

TRICUSPID ATRESIA
Absence of tricuspid vavle and hypoplastic RV
Requires both ASD and VSD

TETRALOGY OF FALLOT
The infundibular septum is moved more over the right atria (anterior) so the pulmonary opening is small and aorta opening is large. It’s also moved superior which causes a VSD

PROV
Pulmonary infundibular stenosis
RVH (boot shaped heart on CXR)
Overriding aorta
VSD

Pulmonary stenosis forces right to left flow across VSD leading to early cyanotic tet spells and RVH.
Squatting incr. SVR which decr. right to left shunt which incr. cyanosis

Early surgical correction

TAPVR-total anomalous pulmonary venous return
Pulmonary veins drain in right heart
ASD and sometimes PDA allow for right to left shunting which maintains CO

Question 3

What are 3 left to right shunts? How do they present? Order of commonness?

Answer 3

Late cyanosis-blue kids

VSD, ASD, PDA

Question 4

When does vsd present? What can large lesions lead to? What is heard on auscultation in ASD? What are the symptoms? What does PDA lead to? What is heard on auscultation? What happens if its uncorrected?

Answer 4

VSD
Can present after weeks after birth or always be asymptomatic.
LV overload and HF

ASD
Loud S1 and wide fixed split S2
No symtoms to HF

PDA
Decr. lugn resistance leads to left to right shunt leads to progressive RVH and/or LVH and HF
Continuous machine like murmur
Can lead to late cyanosis in lower extremities

Question 5

What is the pathophys of eisenmenger syndrome? Symptoms? What are coarctation of the aorta? What is it associated with? symptoms?

Answer 5

left to right shunt leads to incr. pulmonary blood flow leading to remodeling of vasc. leading to pulm. artery hypertension.

RVH occurs to compensate and the shunt becomes right to left

late cyanosis, clubbing, and polycythemia

COARCTATION

aortic narrowing near insertion of ductus arteriosus
bicuspid aortic valve, turners
hypertension in upper extremiteis and weak, delayed pulse in lower
Collateral arteries erode ribs (notched appearance on x ray)

Question 6

What cardiac defects are associated with fetal alcohol syndrome, congenital rubella, DS, infant of diabetic mother, marfan syndrome, prenatal lithium, turners, williams, and 22q11 syndromes?

Answer 6

fetal alcohol syndrome: VSD, PDA, ASD, TOF

congenital rubella: SEPTAL DEFECTS, PDA, pulm. artery stenosis

DS: AV septal defect (endocardial cushion defect), VSD, ASD

infant of diabetic mother: Transposition

marfan syndrome: MVP, thoracic aortic aneurysm and dissection, aortic regurgitation

prenatal lithium: ebstein anomaly

turners: Coarc, bicuspid
williams: supravalvular aortic stenosis

22q11 syndromes: truncus, TOF

Question 7

What is the definition of hypertension? Risk factors? What is primary hypertension? Relations? Percentage of cases? Secondary? Assocations? What is hypertensive urgency? What is a hypertensive emergency? Symptoms? What does hypertension presdispose to? What is the pathology of fibromuscular dysplasia and hypertensive nephropathy?

Answer 7

Persistent systolic >140 or diastolic >90

Incr. age, obesity, diabetes, inactivity, excess salt, excess alcohol, family history, black>white>asian

90% is primary (Incr. CO or TPR)
10% secondary (fibromuscular dysplasia (beads on a string of renal artery), primary hyperaldosteronism, etc.)

Severe (>180/>120) hypertension without end organ damage

Severe hypertension with end ordgan damage (papilledema, encephalopathy, stroke, retinal hemorrhages and exudates, MI, HF, aortic dissection, kidney injury, MAHA, eclampsia)

CAD, LVH, HF, a fib, aortic dissection, aortic aneurysm, stroke, chronic kidney disease (hypertensive nephropathy–renal artery hyalinosis), retinopathy

Question 8

What are xanthomas? Where do they occur? What are tendinous xanthomas? What is corneal arcus? What causes them?

Answer 8

Hyperlipidemia

Plaques or nodules composed of lipidl aden histiocytes in skin (eyelids=xanthelasma)

Lipid deposit in tendon,especially achilles

liipid deposit in cornea

Question 9

Describe arteriosclerosis. Describe the two types of arteriolosclerosis. Describe monckeberg arteriosclerosis.

Answer 9

Artery hardening with thickening of wall and loss of elasticity

Small arteries and arterioles
Hyaline: thickening of vessel walls in essential hypertension or diabetes
Hyperplastic: onion skinning in severe hypertension

Medial calcific sclerosis
Medium sized arteries
calcification of elastic lamina leading to vascular stiffening w/o obstruction.
Does not obstruct blood flow

Question 10

What is atherosclerosis? What are some modifiable and non modifiable risk factors? Describe the pathophys. Complications? Common locations? Symptoms?

Answer 10

Elastic and large and medium sized muscular arteries: arteriosclerosis caused by build up of plaques.

Modifiable: smoking, hypertension, hyperlipid, diabetes
non: age, family history, gender,

Endothelial cell dysfuntion
macrophage and LDL accumulation
Foam cell formation
Fatty streaks
Smooth muscle cell migration (PDGF and FGF)
Smooth cell proliferation
EC matrix deposition
Fibrous plaque
Complex atheromas

Aneurysm, thrombosis, embolism, Periph. vac. disease, ischemia, infarcts

Abdom aorta>coronary>popliteal>carotid

Angina, claudication

Question 11

What is an aortic aneurysm? symtpoms? What do symptoms mean? What is an abdom aortic aneur associated with? Risk Factors? How does it present? What is a thoracic aortiic aneurysm associated with? Risk factors?

Answer 11

Localized pathologic dilatation of aorta
Abdominal or back pain=sign of leak, imminent rupture, dissection

Atherosclerosis (tobacco, incr. age, male, family history)
palpable pulsatile mass

Cystic medial degeneration
Hyperension, bicuspid aortic, marfans, tertiary syphilis

Question 12

What is an aortic dissection? Assocations? Presentation? CXR findings? Results?

Answer 12

Longitudinal intimal tear forming false lumen

hypertension, bicuspid aortic, marfans

Tearing chest pain of sudden onset, radiates to back
unequal BP in arms

CXR: mediastinal widening

rupture, pericardial tamponade, and death

Question 13

What is angina? What causes the 3 different types? Triggers? What makes the better? Treatment? Lab findings? Symptoms?

Answer 13

Chest pain due to ischemic myocardium de to coronary artery narrowing or spasm. No necrosis

Stable: Pain during exertion, ST depression on ECG, resolves with rest of Nitroglycerin

Variant (prinzmetal): at rest due to spasm. transient ST elevation. Tobacco, cocaine, and triptans, unknown.
CA channel blockers, nitrates and smoking cessation

Unstable: thrombosis wit incomplete occlusion. May or may not have ST depression and T wave inversion. No biomarkers. Is an increase in frequency or intensity of the angina or when pain occurs at rest.

Question 14

What is coronary steal syndrome? Describe the pathophys. What is this principle used for?

Answer 14

Distal to coronary artery stenosis, vessels are maximally dilated. Therefore, when a vasodilator is given, they can dilate anymore, so the unstenosed vessels dilate making the stenosed area more ischemic

Pharmacological stress tests

Question 15

What is sudden cardiac death? Cause? associations? What is chronic ischemic heart disease?

Answer 15

Death from cardiac causes under 1 hour of onset of symptoms.

Arrhythmia (v fib)

CAD, cardiomyopathy, hereditary ion channelopathies

Progressive onset of HF over many years due to chronic ischemic yocardial damage

Question 16

What is order of most commonly occluded coronary arteries? What are the sytmpoms of MI? What is the gross pathology and microscopic pathology at 0-4 hr, 4-24 hr, 1-3 days, 3-14 days, and 2 weeks to several months?

Answer 16

LAD>RCA>circumflex

diaphoresis, N/V, retrosternal pain, pain in left arm and jaw, SOB, fatigue

0-4
G: none
M: none

4-24
G: dark mottling, pale with tetrazolium stain
M: early coag necrosis, necrotic contents into blood; edema, hemorrhage, wave fibers, neutrophils appear, reperfusion injury? (contraction bands)

1-3 days
G: hyperemia
M: extensive coag necrosis. Tissue surrounding infarct shows acute inflammation with neutrophils

3-14 days
G: hyperemic border; central yellow brown softening-maximally yellow and soft by 10 days
M: Macrophages, then granulation tissue at margins

2 weeks to several months
G: recanalized artery, gray-white
M: contracted scar complete

Question 17

What is the gold standard for MI diagnosis in first 6 hours? What changes occur? what is the timeline for cardiac troponin? What is it useful for? What is the timeline for CK-MB? Waht is it useful for? Why?

Answer 17

ECG in first 6 hours
STEMI (transmural infarct)=ST elevation
NSTEMI (subendocardial infarct)=ST depression
hyperacute T waves, T wave inversion
New LBBB
pathologic Q waves or poor R wave progression=evolving or old transmural (STEMI) infarct

Troponin I=rises after 4 hours and is incr. 7-10 days; more specific than toher markers

CK-MB=6-12 hours. Diagnosing reinfarction after acute MI b/ levels return to normal after 48 hours.

Question 18

What leads will have ST elevations or path. q waves in an infarct in the anteroseptal location? anteroapical? anterolateral? Lateral? Inferior? Which CAs are affected?

Answer 18

anteroseptal location? V1-V2 (LAD)
 anteroapical? V3-V4 (distal LAD)
anterolateral? V5-V6 (LAD or LCX)
Lateral? I, aVL (LCX)
Inferior? II, III, aVF (RCA)

Question 19

What are some complications of an MI? When do they occur and why are they problematic?

Answer 19

arrhythmia; first few days

Post infarction fibrinous pericarditis: friction rub (1-3 days)

LV failure and pulmonary Edema

Cardiogenic shock

Ventricular free wall rupture leading to cardiac tamponade and papillary muscle rupture leading to severe mitral regurg and IV septum rupture leading to VSD (3-14 days post MI)

ventricular pseudoaneurysm formation (contained free wall rupture)=decr. CO, risk of arrhythmia, embolus from mural thrombus (3-14 days)

true ventricular aneurysm: outward bulge during contraction; associated with fibrosis (2 weeks to several months)

Dressler syndrome-autoimmune phenomenon leading to fibrinous pericarditis (several weeks post MI)

Question 20

How is unstable angina/NSTEMI treated? What else is done for STEMI?

Answer 20

anticoag, antiplatelets, beta blockers, ACE I, NO and morphine (symptom control)

Reperfusion therapy most important (percutaneous coronary intervention preferred over fibrinolysis)

Question 21

What is the most often etiology of a dilated cardiomyopathy? What are some other etiologies? What are some findings? Treatment? What kind of dysfunciton ensues? Gross/microscopic path?

Answer 21

Idiopathic of familial

ABCCCCCDD

Alcohol abuse
wet Beri beri
Coxsackie B virus myocarditis
chronic Cocaine use
Chagas
hemoChromatosis
peripartum Cardiomyopathy
Doxorubicin toxicity
sarcoiDosis

HF, S3, systolic regurgitant murmur, dilated heart on echo, balloon appearance on CXR

Na restriction, ACEI, beta blockers, diuretics, digoxin, ICD, heart transplant

Systolic dysfunction

sarcomeres added in series (eccentric hypertrophy (longer wall not thicker wall))

Question 22

What are 60-70% of hypertrophic cardiomyopathy caused by (specificallly)? Association? Symptoms? Findings? Treatment? Dysfunction? Gross/micro path? What is obstructive hypertrophic cardiomyopathy?

Answer 22

familial, autosomal dominant (beta myosin heavy chain)

Friedrichs ataxia

Syncope durig exercise and sudden death in yougn athletes

S4, systolic murmur, maybe mitral regurg

Cessation of high intensity exercise, beta blocker or verapamil. ICD.

Diastolic dysfunction

Ventricular hypertrophy (myofibrillar disarray and fibrosis)

HOCM: septal hypertrophy and systolic anterior motion of mitral valve–>outflow obstruction—>dyspnea/syncope

Question 23

What are some major causes of restrictive/infiltrative cardiomyopathy? dysfunction? What can be seen on ECG?

Answer 23

sarcoidosis, amyloidosis, postradiation fibrosis, endocardial fibroelastosis (young children), loffler syndrome (fibrosis with a prominent eosinophilic infiltrate), hemochromatosis.

diastolic dysfunction

Low voltage ECG

Question 24

What is heart failure? Symptoms? Signs? compare/contrast systolic/diastolic function and how they effect EF, EDV, and contractility/compliance (causes). What most often causes right HF? 2nd leading cause? What treatments decr. mortality? Help symptoms? improve symptoms and decr. motrality in some patients?

Answer 24

cardiac pump dysfunction—>congestion and low perfusion

dyspnea, orthopnea, fatigue

rales, JVD, pitting edema

Systolic: reduced EF, Incr. EDV, Decr. contractility due to ischemia/MI or dilated CM

Diastolic: preserved EF, normal EDV, decr. compliance due to myocardial hypertrophy.

Right HF from left HF, then from cor pulmonale

ACEI and ARBs, Beta blockers (except in acute decompensated HF) and spironolactone decr. mortality

Thiazide or loop diuretics for symptoms

hydralazine with nitrate therapy improve symptoms and mortality in some patients.

Question 25

What are 3 symptoms of left heart failure? Pathophys of each? 3 symptoms of right heart failure and pathophys?

Answer 25

Orthopnea: SOB when supine. Incr. venous return exacerbates pul. vasc. congestion

Paroxysmal nocturnal dyspnea: breathless awakening from sleep

Pulm. edema: incr. pulm. venous pressure–>transudation (hemosidern laden macrophages) in lungs. heart failure cells

hepatomegaly (nutmeg liver): incr. central venous pressure, incr. resistance to portal flow

JVD: incr. venous pressure

Periph. edema: incr. venous pressure leading to transudation

Question 26

Pathophys of HF as well as compensation?

Answer 26

decr. LV contractility leads to pulmonary congestion leading to pulmonary edema
pulm. congestoin also leads decr. RV output leading to periph. edema

Decr. LV contract. also leads to decr. CO leading to incr. RAAS leading to incr. renal na and h20 reabsorption leading to incr. systemic venous pressure leading to periph. edema as well as incr. preload

Decr. CO also leads to incr. sympathetic activity leading to incr. RAAS and incr. LV contractility

Incr. preload and LV contractility lead to compensation, albeit by causing edema.

Question 27

What are the 4 types of shock? What causes each of them? How do they affect the skin? How do they affect preload, CO, and afterload? Treatment for each? What is systemic inflammatory response syndrome? First sign of shock? End result of shock?

Answer 27

HYPOVOLEMIC

hemorrhage, dehydration, burns

Cold clammy skin

Very decr. preload, decr. CO, incr. afterload

IV fluids

CARDIOGENIC

acute MI, HF, valve dysfunction, arrhythmia

cold clammy

incr. preload, very decr. CO, incr. afterload

inotropes, diuresis

OBSTRUCTIVE

cardiac tamponade, PE

cold clammy

incr. preload, very decr. CO, incr. afterload

relieve obstruction

DISTRIBUTIVE

sepsis, CNS injury, anaphylaxis

warm, dry

decr. preload, incr. CO, very decr. afterload

pressors and IV fluids

SIRS (>=2: fever/hypothermia, tachycardia, tachypnea, leukocytosis/leukopenia

First sign of shock=tachycardia

MODS is end result

Question 28

What are the symptoms of bacterial endocarditis? What microbe causes acute endocarditis? Subacute? Which is present in colon cancer? Which presents on prosthetic valves? What are some non bacterial causes? IV drug abuse causes? culture negative causes?

Answer 28

FROM JANE

Fever
Roth spots (white spots on retina surr. by hemorrh.)
Osler nodes (tender raised lesion on finger or toe pads)
Murmur (new onset)
Janeway lesions (painless red lesions on palm or sole)
Anemia
Nail-bed hemorrhage
Emboli

acute: staph aureus-rapid onset
subacute: viridans streptococci

Strep bovis: colon cancer
S. epidermidis: prosthetic valves

Non bacterial: malignancy, hypercoag state, lupus

IV=tricuspid=staph, pseudomonas, candida

Culture neg=coxiella burnetii, bartonella spp., HACEK (haemophilus, actinobacillus, cardiobacterium, eikenella, kingella)

Question 29

What heart valves are affected in RF (common)? Early lesion? Late lesion? What are aschoff bodies? anitschkow cells? what is the pathophys? Treatment?

Answer 29

mitral>aorta»tricuspid

early: mitral regurg
late: mitral stenosis

Aschoff: granules with giants cells
anitschkow cells: enlarge macrophages with ovoid wavy rod like nucleus

Immune mediated (type II): antibodies to m protein attack self antigens

Penicillin

Question 30

How does acute pericarditis prsent? How is it relieved? Auscultation ECG changes? Causes?

Answer 30

sharp pain, aggravated by inspiration
relieved by sitting up and leaning forward

Friction rub

Diffuse ST segment elevation and/or PR depression

idiopathic (viral), confirmed infection (cocsackievirus), neoplasia, autoimmune (SLE, RA), uremia, acute STEMI/Dressler, radiation therapy

Question 31

What is cardiac tamponade? Results? Findings? What is pulsus paradoxus? when is it seen? ECG?

Answer 31

compression of heart by fluid in pericardial space–>decr. CO
Equilibration of diastolic pressures in all 4 chambers

Beck triad (hypotension, distended neck veins, distant heart sounds), incr HR, pulsus paradoxus (decr. in amplitude of systolic BP by >10 mmHg during inspiration-tamponade, asthma, obstructive sleep apnea, pericarditis, croup)

ECG: low voltage QRS and electrical alternans (swinging movement of heart)

Question 32

What is the pathophys of syphilitic heart disease? Location? gross Appearance? Results?

Answer 32

tertiary syphilis affects the vasovasorum or the aorta leading to atrophy of vessel wall and dilatation of aorta and valve ring

calcification of aortic root and ascending aortic arch. Tree bark appearance.

Aneurysm of ascending aorta or aortic arch; aortic insufficiency.

Question 33

What is the most common heart tumor? Most common primary in adults? Most common primary in children? Describe them. Location. symptoms. associations. What is kussmall sign? explain pathophys. causes?

Answer 33

Metastasis

Myxoma: adults. 90% in atria. Ball valve obstruction leading to multiple syncopal episodes. Diastolic tumor plop

Rhabdomyomas: children (tuberous sclerosis)

Kussmaul: incr. in JVP on insp. instead of a decr.
Normally, the JVP would decr. because of neg. IT pressure leading to greater filling of the heart. However, if the side of the heart isn’t able to fill, the JVP accepts the extra blood that should be going into the heart.

Constrictive pericarditis, restrictive cardiomyopathies, right atrial or ventricular tumors

Question 34

What is an angiosarcoma? Locations? Age? Associations? Hepatic angiosarcoma associations? Prognosis? What is bacillary angiomatosis? Cause? epidemiology? Diagnosis? What is a cherry hemangioma? prognosis? What is a cystic hygroma? Association? What is a glomus tumor? Origin? What is kaposi sarcoma? Locations? Assocations? Diagnosis? What is a pyogenic granuloma? symptoms? Associations? What is a strawberry hemangioma? Presentation? prognosis?

Answer 34

Angiosarcoma: head, neck, breast; elderly; sun exposed areas, radiation therapy, chronic postmastectomy lymphedema; hepatic; vinyl chloride and arsenic; very aggressive, difficult to resect due to delayed diagnosis

bacillary: benign capillary skin papules; HIV and B. henselae. Mistaken for kaposi, but has neutrophilic infiltrate

cherry hemangioma: benign capillary hemangioma of elderly; does not regress, incr. in frequency with age

Cystic hygroma: cavernous lymphangioma of neck; turners

glomus tumor: benign, painful red blue tumor under fingernails. modified SM cells of thermoreg. glomus body

Kaposi: endothelial malignancy most commonly of skin, but mouth, GI, and resp. tract; HHV-8 in HIV pts; mistaken for bacillary but has lymph. infiltrate

Pyogenic granuloma: Polypoid capillary hemangioma that can ulcerate and bleed; trauma and pregnancy

Strawberry hemangioma: benign capillary hemangioma of infancy; first weeks of life; grows rapidly and regresses by age 5-8.

Question 35

What is raynauds phenomenon? Presentation? Location? What is raynauds disease? syndrome? Associations? Treatment?

Answer 35

decr. blood flow to skin due to arteriolar vasospasm in response to cold or stress

white (ischemia) to blue (hypoxia) to red (reperfusion)

Fingers and toes

Disease=primary

syndrome: association to a disease such as MCTD, SLE, or CREST.

Ca channel blockers