Heart Pathology

Question 1

foramen ovale

Answer 1

Foramen Ovale formation: Septum primum seperates the atria with ostrium primum hole. As Septum primum continues to grow, the ostium primum gets smaller. Programmed cell death results in formation of a second hole in septum primum called ostium secundum. A second wall of tissue called septum secundum grows over the ostrium secundum in the RA but is kept open due to the high pressure in the right atrium. When the pressure drops at birth, this flap is slammed shut from higher pressure in left atria, and eventually fuses.

Question 2

LAD

Answer 2

• Left anterior descending artery (LAD): supplies most of apex of heart: most commonly infarcted (40-50%)
  o anterior wall of left ventricle near apex
  o anterior 2/3 of ventricular septum
  o apex of heart

Question 3

LCX

Answer 3

Left circumflex artery (LCX) – (15-20% of infarcts)

o lateral wall of left ventricle, except for apex

Question 4

RCA

Answer 4

• Right coronary Artery (RCA) – (30-40% of infarct)
  o inferior/posterior wall of left ventricle
  o posterior portion of ventricular septum
  o inferior/posterior right ventricular free wall
  o most often causes arrhythmias

Question 5

CHF

Answer 5

= heart is unable to pump blood at a rate sufficient to meet the metabolic demands of the tissues or can do son only at an elevated filling pressure
• due to insufficient pump rate to meet demands
• due to pump only being able to meet demands with elevated filling pressure

Question 6

cardiomegally

Answer 6

Normal: Male 300-350 gm; Female 250-300 gm

• 350-600 gm: Pulmonary HTN, IHD
• 400-800 gm: Systemic HTN, aortic stenosis, mitral regurgitation, dilated cardiomyopathy
• 600-1000 gm: Aortic regurgitation, hypertrophic cardiomyopathy

Question 7

“Concentric Hypertrophy”

Answer 7

• Pressure overload hypertrophy: “Concentric Hypertrophy” - results in new sarcomeres assembled in parallel, expanding the cross-sectional area of myocytes and causing concentric increase in wall thickness
  • left ventricle: due to systemic HTN or aortic stenosis
  • right ventricle: cor pulmonale

Question 8

“Eccentric Hypertorphy

Answer 8

• Volume-overload hypertrophy: “Eccentric Hypertorphy” new sarcomeres are assembled in series w/in existing sarcomeres leading to ventricular dilation
  • see an overall cardiac mm. mass increased even though dilation may cause chamber wall to look thinner
  • seen in valve disorders and congenital heart disease

Question 9

left sided heart failure

Answer 9

• accumulation of fluid within the lungs and pleural cavities, pleural effusions
• Chronic: slowly progressive failure which may over time develop sufficient severity to cause right sided heart failure
• Acute: Rapidly progressing fatal course – often see massive pulmonary edema and “pink frothy fluid coming out of mouth”, due to macrophages ingestion of the lungs of fluid and RBCs
• Most commonly caused by:
- Ischemic heart disease
- Hypertensive heart disease
- Aortic and mitral valvular disease
- Primary nonischemic myocardial disease (cardiomyopathy)
• “Systolic Failure” = insuff. ejection fraction, pump failure
• “Diastolic Failure” = Stiff left ventricle, that can’t fully fill during diastole → decreased CO

Question 10

path of LSHF?

Answer 10

• Cardiomegaly: hypertrophy and/or chamber dilation
o ischemic heart disease: see chamber dilation
o left sided valvular disease: mostly see LV hypertrophy
• Secondary enlargement of Left Atrium
o assoc. w/ A Fib
o High incidence of mural thrombosis due to stasis and turbulence → systemic emboli
• Tachycardia
• Third Heart Sound (S3 gallup): occurs in diastolic filling due to decreased compliance of ventricular wall – blood from atria shoots into ventricle and hits the wall making this sound. This is normal in children, but watch out for in adults that never had before
• Mitral regurgitation: heart systolic murmur – due to dilation of walls, valves no longer properly seal
Extra-Cardiac Effects:
• Pulmonary Congestion and Edema:
o hear pulmonary basilar crackles (rales) and possible pleural effusions
o “flash pulmonary edema” due to rapid onset
o Siderophages: “Heart Failure Cells” – seen in long term, macrophages in lungs that have engulfed RBCs
o Dyspnea: SOB
o Orthopnea: dyspnea when laying down
o Paroxysmal Nocturnal Dyspnea: random, severe dyspnea attacks at night
• Renal Problems:
o decreased CO → hypoperfusion of kidney → activation of RAAS → fluid retention and expansion of vascular volume (further damage to heart) → decrease in blood flow to kidney →azotemia
o “pre-renal azotemia” = inability of kidney to excrete nitrogenous waste products
• Brain: hypoxic ncephalopathy

Question 11

S3

Answer 11

LSHF

• Third Heart Sound (S3 gallup): occurs in diastolic filling due to decreased compliance of ventricular wall – blood from atria shoots into ventricle and hits the wall making this sound. This is normal in children, but watch out for in adults that never had before

Question 12

siderophages

Answer 12

heart failure cells, seen in LSHF

Question 13

RSHF

Answer 13

MOST COMMONLY CAUSED BY LEFT HEART FAILURE!
• peripheral edema: see accumulation of fluid in all other body sites and body cavities, liver and spleen
• left heart failure → increased pulmonary pressure → increased workload on RV → right side failure
• Cor Pulmonale = Heart disease that is secondary to lung disease
- pure/isolated right sided failure
- uncommon
- cardiac hypertrophy and filation are confined to RA and ventricle
Extracardiac Effects:
o Pitting Edema: accumulation of edema fluid in subcutaneous tissues = “anasarca” (massive generalized edema)
o Congestive Hepatomegaly: increased pressure in IVC → increased pressure in hepatic vv → congestion in hepatic sinusoids → centrilobular necrosis (due to ischemia and congestions)
o “Cardiac Cirrhosis” see increased fibrous tissue in centrilobular zone
o “nutmeg liver” appearance
o Congestive Splenomegally: usually mild with only doubling in size
o Ascites: massive peritoneal effusions
o Pleural and Pericardial Cavity Effusions (transudates)

Question 14

natriuretic peptides

Answer 14

Peptides → cause vasodilation, natriuresis and diuresis
- ANP: produced by atrial myocytes due to atrial distension
- BNP: due to ventricular mycotes: used for determination of CHF!
o if BNP 500 then it is mostly likely CHF
- CNP: due to endothelial cells b/c of shear stress

Question 15

congenital heart diseases

Answer 15

Congenital Heart Disease:

• ** defects occur: week 3-8 gestation ***
• Most common: bicuspid aortic valve, ventricular septal defect, atrial septal defect (most common ddx in adulthood), pulmonary stenosis, patent ductus arteriosus, tetraology of fallot….

Where does problem in great vessels stem from? Neural crest defect

Question 16

NKX2-5 transcription factor and TBX5

Answer 16

NKX2-5 transcription factor:

Question 17

TBX1 tx factor: del 22q11.2

Answer 17

DiGeorge Syndrome = Cardiac outflow tract defects
o “Catch-22” = Cardiac abnormalities, abnormal facies, thymic aplasia, cleft palate, hypocalcemia
o Cardiac outflow defects = persistent truncus arteriosus, aortic arch interruption/coarctation
o see T cell deficiency from thymus problems

Question 18

JAG1, NOTCH2:

Answer 18

tetralogy of fallot

Question 19

Fibrillin protein mutation:

Answer 19

Marfan’s Syndrome: Aortic aneurysm and valve abnormalities

Question 20

left to right shunts

Answer 20

: ASD, VSD, PDA, AVSD
o ** pink babies ** see increased pulmonary blood flow with no initial cyanosis
• chronically elevated volume and pressure in the right side of heart→ results in right ventricular hypertrophy
• muscular pulmonary aa. develop medial hypertrophy and vasoconstriction to normalize distal pressure
• Esenmenger syndrome: (shunt reversal): increased right heart pressure → irreversible pulmonary HTN → right to left shut and shunt reversal → cyanosis

Question 21

Esenmenger syndrome

Answer 21

(shunt reversal): increased right heart pressure → irreversible pulmonary HTN → right to left shut and shunt reversal → cyanosis

Question 22

VSD

Answer 22

Ventricular Septal Defect (VSD)
• most common!
• 90% involves membranous septum (high up)
• 10% involves muscular septum (low)
• large defects result in murmur, pulmonary HTN
Clinical:
• most assoc. with other cardiac anomalies, only 20%are isolated
• smaller lesions can be well tolerated for many years – will close spontaneously
• large lesions lead to irreversible pulmonary vascular disease→ shunt reversal, cyanosis, death

Question 23

ASD

Answer 23

• Three Major Types:
  1. Secundum (90%): involves fossa ovalis, found near the center (Note: patent foramen ovale is not ASD)
  2. Primum (5%) – adjacent to AV valve
  3. Sinus Venosus- near superior vena cava
  • ASD’s are well tolerated and may be symptomatic, 10% of untreated develop pulmonary HTN
  • May hear murmur from increased flow across pulmonary valve

Question 24

PDA

Answer 24

Patent (Persistent) Ductus Arteriosus: (PDA)
• Ductus usually closes 1-2 days due to: increased O2, decreased pulmonary resistance, decreased Prostaglandin E2
• 90% are isolated defects
• Narrow ductus defect is asymptomatic
• harsh machine-like murmur
• if chronic, can develop pulmonary HTN, due to back flow into right side of heart → cyanosis
• can get paradoxical embolisms
• Treatment: NSAID to close, or prostaglandin E to keep open until surgery
• ** note: prior to closure, must ensure that there are no other defects!

Question 25

AVSD

Answer 25

Atrioventricular Septal Defect (AVSD): • partial = ASD and cleft anterior mitral leaflet with mitral insufficiency • complete = large combined AV valve with all 4 chambers communicating * MOST common defect in Down syndrome >1/3 patients have AVSD*** • 40-60% of children with Trisomy 21 have CHD: AVSD>VSD>ASD>PDA>TF

Question 26

right to left shungs

Answer 26

TF, TPGA, persistent truncus arteriosus, tricuspid atresia • ** see hypoxia and cyanosis** = hypoxic from day 1 • pulmonary circulation is bypassed and poorly O2 venous blood shunts directly into systemic arterial supply, decreased amout of blood going to lungs to be oxygenated • paradoxical embolism: emboli from peripheral vv. can bypass lung and enter systemic circulation • “clubbing” of tips of fingers and toes: “hypertrophic osteoarthropathy” • polycythemia due to hypoxia

Question 27

tetrology of fallot

Answer 27

**Tetrology of Fallot: 1) Ventricular Septal Defect (VSD) 2) Subpulmonic (+/- pulmonic valve) stenosis = obstruction of right ventricular outflow tract • subpulmonic stenosis protects lung from increased pressure, and pulmonary arteries may be hypoplastic 3) Aorta overrides the VSD – heart is laying down on its side 4) Right ventricular hypertrophy • note: marked right ventricular hypertrophy: “boot-shaped” heart, results in blood to be shunted from right side to left side • if subpulmonic stenosis is balanced with left ventricular pressure, then it behaves like a VSD and lungs are perfused = pink tetralogy of Fallot • as child grows, the subpulmonic stenosis does not increase in size, making it progressively worse What determines if baby is cyanotic or pink? the degree of stenosis in the outflow tract - too much stenosis, have right to left shunting à blue cyanotic baby - not too much stenosis, don’t have the right to left shunting à pink baby

Question 28

TGA

Answer 28

Transposition of the Great Arteries: • defect with truncal and aorta pulmonary septae • results in separation of pulmonary and systemic circulations, incompatible with life unless pt. has VSD, patent foramen ovale or patent ductus arteriosus • Patient is stable with VSD (35%), but PDA or patent foramen ovale can close • if PDA infuse with prostaglandin E and perform atrial septostomy followed by surgical correction

Question 29

coarctation of aorta

Answer 29

Coarcation of the Aorta: (coarcation = narrowing/constriction) • more common in males • Infantile form (With PDA): symptomatic in early childhood – see hypoplasia of aorta prior to patent ductus arteriosus (cyanosis of inferior body and weak femoral pulses) • Adult form (Without PDA): ridge-like fold opposite ligamentum arteriosus, closed ductus arteriosus → encroachment of aortic lumen (HTN in upper extremities, low pressure and pulses in lower extremities) o disease may go unrecognized until adult life o 50% of cases accomp. by bicuspid aortic valve, ASD, VSD, mitral regurg, berry aneurysms • Coarctation of aorta, postductal adult type o Murmurs are often present with coarctations o may develop collateral circulation b/w pre-coarctation and post-coarctation arteries through enlarged intercostal and internal mamm. aa.: results in “notching” visible erosions on radiographs in undersurface of ribs o Kidneys will sense low pressure → turn on RAAS o high pressure in head, low pressure in extremities

Question 30

aortic stenosis/atresia

Answer 30

(80% isolated congenital finding) • Valvular aortic stenosis – have hypoplastic, dysplastic, or abnormal number of cusps (if severe get hypoplastic left heart syndrome) • Subaortic stenosis – ring or collar below cusps: hear prominent systolic murmur and sometimes a thrill • Supravalvular aortic stenosis – elastin gene mutation with aortic dysplasia (thickening) • Williams–Beuren syndrome -deletion of about 28 genes from chromosome 7 with ELN gene (elastin) haploinsufficiency, hypercalcemia, glucose intolerance, facial and cognitive defects.

Question 31

stable angina

Answer 31

Typical) Angina: increases in myocardial O2 demand that outstrip ability of stenosed coronary arteries to increase O2 delivery; not usually associated with plaque disruption • stenosis is often > 75%, no plaque disruption or assoc. thrombus, no cellular necrosis • occurs during emotional excitement, stress or physical activity • most common form, usually relieved by sublinqual nitroglycerine

Question 32

unstable angina

Answer 32

2. Unstable (Crescendo, Pre-infarction) angina: caused by acute plaque disruption that results in thrombosis and vasoconstriction and leads to severe but transient reductions in coronary blood flow • variable stenosis with plaque disruption and nonocclusive thromboemboli present • see pattern of increasingly frequent, prolonged, severe angina that does not occur at high levels of activity • may be relieved by rest or sublingual nitroglycerine

Question 33

MI

Answer 33

often result of acute plaque change that induces an abrupt thrombotic occlusion, resulting in myocardial ischemia • Transmural MI: stenosis, with plaque disruption leading to complete block due to thrombus • Subendocardial MI: variable stenosis with partial blockage

Question 34

SCD

Answer 34

Unexpected death from cardiac causes early after onset of symptoms (1 to 24 hours) or sudden death from cardiac cause without antecedent acute symptoms • usually due to lethal arrhythmia or ventricular fib • see non-ischemic SCD in younger people (most commonly due to hypertrophic cardiomyopathy)

Question 35

CRP

Answer 35

C Reactive Protein = non-specific measure of chronic inflammation- when elevated its indicative of risk for coronary heart disease Other types of Angina:

Question 36

Prinzmetal variant Angina:

Answer 36

episodic myocardial ischemia usually caused by sustained coronary artery vasospasm • relieved by rest, nitroglycerine or CCB’s

Question 37

- Takotsubo cardiomyopathy:

Answer 37

dilated cardiomyopathy secondary to emotional or physical stress with normal angiogram

Question 38

channelopathies

Answer 38

: Disorders of K+, Na+, or Ca++ channel structure or accessory proteins involved in channel function • **Mostly autosomal dominant • ex: Long QT syndrome, Short QT syndrome, Brugada syndrome

Question 39

Chronic IHD

Answer 39

Chronic Ischemic Heart Disease: Ischemic Cardiomyopathy • Fairly severe atherosclerosis → ischemic myocytes and necrosis → build scars → CHF • onset of congestive heart failure in patients who have had past myocardial infarcts (MIs) or angina • Cardiomegaly with left ventricular hypertrophy & dilatation • Evidence of previous healed MIs or ischemia (areas of myocardial fibrosis) • Develop arrhythmias, congestive heart failure and MIs

Question 40

transmural infarct

Answer 40

* Transmural Infarction: Ischemic necrosis involves >50% of the ventricular wall thickness - Commonly associated with acute plaque change with thrombosis

Question 41

subendocardial infarct

Answer 41

: area of ischemic necrosis is limited to the inner 1/3 and thus is <50% - May occur as a result of acute plaque change and thrombosis - May result from prolonged and severe reduction in systemic blood pressure ,as encountered in shock

Question 42

pathogenesis of MI

Answer 42

• Initial sudden change in plaque • Immediate formation of initial platelet plug over plaque • Vasospasm from platelet adhesion (vasoactive substances from platelets) • Propagation of platelet plug into stable clot via extrinsic clotting system • Within minutes, clot occludes lumen of the involved vessel (thrombosis with coronary occlusion) Note: adrenergic stimulation can induce MI: peak incidence at 6am-noon Note: subendocardium is first infarcted

Question 43

timing of MI

Answer 43

* Seconds: ATP depletion due to decreased glycolysis * 2 minutes: loss of contraction * 20-40 minutes: irreversible cell injury – necrosis * >1 hr: microvacular injury * 6 hours = complete unsalvageable necrosis

Question 44

morphology of MI

Answer 44

* 0-1/2 hr: no gross features, no LM changes * ½ -4 hr: no gross features, see variable waviness of fibers in LM * 4-12 hr: usually see no gross features (maybe dark mottling); LM shows early coagulation necrosis, edema, hemorrhage * 12-24 hours: grossly see dark mottling, LM shows early neutrophilic infiltrate * 1-3 days: mottling with yellow tan infarct, see large infiltrate of neutrophils * 3-7 days: see central yellow-tan softening; LM shows dying neutrophils, early phagocytosis of macrophages * 7-10 days: see early formation of fibrovascular granulation tissue at margins * 10-14 days: see red-gray depressed infarct grossly; granulation tissue w/ new blood vessels and collagen * 2-8 week: see gray white scar, LM shows collagen deposition * >2 mos: see dense collagenous scar

Question 45

clinical features of MI

Answer 45

* Severe substernal chest pain with radiation of pain down left arm, neck, jaw, epigastrium * Weak, rapid pulse * Sweating profusely (diaphoretic) * Nausea * Dyspnea (difficulty breathing) secondary to pulmonary congestion and edema

Question 46

myoglobin

Answer 46

``` : can be due to any muscular stress… • elevated 1-4 hours after AMI • peaks in 6 hours • returns to baseline after 18-24 hours • best for reading MI’s <6 hours ```

Question 47

CK-MB

Answer 47

* elevated 3-12 hours after AMI * peaks in 10-24 hours * returns to baseline after 48-72 hours

Question 48

CTnI/CTNT

Answer 48

most specific, though not good for recurrent MI • elevated 3-12 hours after • peaks 10-24 hours after Ami • returns to baseline after 5-10 days, thus best for determining if there was an MI in past week

Question 49

reperfusion injury

Answer 49

Pathologic changes due to Reperfusion: • Reperfusion-induced arrhythmias • Myocardial hemorrhage with contraction bands • Irreversible myocardial damage in addition to that caused by the initial episode of ischemia (reperfusion injury) • Microvascular injury with endothelial swelling (no-reflow) • Reversible “Myocardial stunning” (prolonged ischemic dysfunction) with heart failure for days

Question 50

cardiac tamponade

Answer 50

can occur after myocardial rupture due to MI Free wall (usually anterior) → cardiac tamponade: Cardiac tamponade is pressure on the heart that occurs when blood or fluid builds up in the space between the heart muscle (myocardium) and the outer covering sac of the heart (pericardium).

Question 51

Systemic (left sided) hypertensive heart disease:

Answer 51

Criteria for Diagnosis: • Left ventricular hypertrophy (usually concentric) in the absence of other cardiovascular pathology that may have induce it. • A history or pathologic evidence of systemic hypertension (BP > 140/90 mm Hg) • May present clinically with CHF or atrial arrhythmias

Question 52

Pulmonary (Right-sided) Hypertensive Heart Disease:

Answer 52

``` Cor Pulmonale results from pulmonary disorders that cause chronic severe pulmonary hypertension. Occurs with: • Pulmonary Parenchyma Disorders • Chronic Obstructive Pulmonary Disease (COPD) • Diffuse Interstitial Lung Disease • Pulmonary Vessel Disorders • Recurrent Pulmonary Embolism • Primary Pulmonary Hypertension • Chest Movement Disorders • Kyphoscoliosis ```

Question 53

aortic stenosis

Answer 53

Calcification of anatomically normal and congenitally bicuspid aortic valves (more common in US)

Question 54

aortic insufficiency

Answer 54

: Dilatation of the ascending aorta due to hypertension and aging

Question 55

mitral stenosis

Answer 55

Rheumatic heart disease (more common worldwide)

Question 56

mitral insufficiency

Answer 56

Myxomatous degeneration (mitral valve prolapse)

Question 57

senile calcific aortic stenosis

Answer 57

(Senile) Calcific Aortic Stenosis: • most common of all valvular abnormalities • see nodular masses of Ca2+ heaped within the sinuses of valsalva Clinical features: • seen in ages 60-80 (7th to 9th) • pressure hypertrophy results from flow obstruction and patient develops left ventricular concentric hypertrophy • Left ventricular cardiac mass ischemia → CHF, syncope or angina pectoris

Question 58

Calcific Stenosis of Congenitally Bicuspid Aortic Valve

Answer 58

* Congenital bicuspid aortic valve occurs in ~ 2% population * Two cusps frequently not equal size: larger cusp may have a raphe” (seam) – result of incomplete separation during development * Bicuspid valves more susceptible to progressive degenerative calcification; develop significant calcification earlier * Develop clinical symptoms and signs of cardiac dysfunction earlier, 5th and 6th decades (7th to 9th decades with tricuspid aortic valves)

Question 59

Mitral Annular Calcification:

Answer 59

“looks like a ring around the heart” • Occurs in three types of patients – Women over 60 years of age – Individuals with myxomatous mitral valves – Patients with elevated left ventricular pressure - e.g., hypertension • Generally does not affect valvular function • Occasionally associated with arrythmias • Most diagnosed because: Large calcium deposits are incidentally detected on radiography done for other reasons

Question 60

MVP

Answer 60

Myxomatous Degeneration of Mitral Valve = Mitral Valve Prolapse • One or both mitral valve leaflets are “floppy” and prolapse into the left atrium during systole (midsystolic click +/- regurgitant murmur) • Affects ~2-3% adults in US, most often young women (7F:1M) • Seen in Marfan syndrome (fibrillin-1/elastic fibers) • Vast majority patients clinically asymptomatic, but small subset (3%) may develop one of four serious complications (infective endocarditis, mitral insuffic, stroke, arrhythmias, atypical chest pain) Pathology: • Intercordal ballooning of mitral valve leaflets • Affected leaflets are enlarge, thick and rubbery Secondary changes: • dilation of annulus, fibrosis of valve leaflets and endocardial surfaces of atrium and ventricle (jet lesions); thrombi on atrial surfaces of mitral leaflets, and focal calcification at base of posterior leaflet

Question 61

Acute Rheumatic fever

Answer 61

1. Group A streptococcal infection that is not treated 2. Acute rheumatic fever: acute systemic autoimmune disease with ASO Abs - thought to be hypersensitivity reaction to M protein cross-reacting with tissue glycoproteins) • Migratory polyarthritis of large joints (swollen, painful joints) • Acute carditis with cardiac enlargement and diminished function • Subcutaneous nodules • Erythema marginatum of skin • Sydenham chorea (involuntary, purposeless movements of extremities) • Occurs 10 days – 6 weeks after clinical episode of Strep infection • Jones Criteria” Evidence of group A strep + 2 major systemic finding • patient should receive long term antiobitic prophylaxis

Question 62

vegetations, aschoff bodies, endocarditis, pericardial rub

Answer 62

acute rheumatic fever Aschoff body - Collection of large activated histiocytes Anitschkow cells – “mononuclear” Aschoff cells - multinucleated forms caterpillar" cells - unique linear chromatin pattern

Question 63

mitral valve stenosis

Answer 63

chronic rheumatic heart disease

Question 64

rheumatoid arthritis and fibrinous pericarditis

Answer 64

rheumatoid heart disease

Question 65

RHD

Answer 65

Chronic rheumatic heart disease (RHD): longer term sequellae of ARG • affects mitral and aortic valve mostly • 99% of all mitral stenosis is caused by RHD Note: Cardiac involvement is present in 20-40 cases of rheumatoid arthritis, usually fibrinous pericarditis!

Question 66

criteria for ddx of endocarditis?

Answer 66

major: + blood cutures, EKG finding, murmur thats new (get 3 blood cultures in 24 hours) minor: fever, lesions, IV drug use, jaeway lesions, rothspots, petechial

Question 67

janeway lesions

Answer 67

new lesions on palms and soles --> infective endocarditits

Question 68

olser nodes

Answer 68

noldues on fingers --> IE

Question 69

roth spots

Answer 69

retinal hemorrhages w/ pale centers --> IE

Question 70

petechial hemorrhage under hails

Answer 70

IE

Question 71

infective endocarditis

Answer 71

- see vegetations on leaflets --> vegetations can break and become septic emboli - • usually involves the left valves (except in IV drug users) • S. viridans (50-60%)>S. aureus (20-30%)>HACEK (Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella) • #1 in artificial valves is S. epidermidis

Question 72

#1 infection in artificial valves

Answer 72

S. epidermidis

Question 73

acute bacterial endocarditis

Answer 73

: Staph Aureus • rapidly progressive destruction of cardiac valves • usually due to S. aureus (IV drug abusers) • 50% of pt. die w/in days or weeks of onset of sx

Question 74

subacute bacterial endocarditis

Answer 74

Streptococcus viridans • endocarditis of native but previously damaged valves • involved valve is usually deformed of abnormal (RHD, mitral valve prolapse #1) • most pt. recover with Antibiotic therapy

Question 75

small warty verrucae along lines of closure of valve leaflets

Answer 75

RHD

Question 76

large irregular masses on valve cusps that can extend into chordae

Answer 76

IE

Question 77

NBTE

Answer 77

hypercoagulable state --> non bacterial thrombotic endocarditis - may produce systemic emboli

Question 78

small, bland vegetations usually attached to line of closure

Answer 78

NBTE

Question 79

medium sized vegetations on either side of valve leaflets

Answer 79

Libman- Sacks Disease (Endocarditis of SLE): • small (1-4 mm) sterile, pink vegetations with warty appearance • located on undersurfaces of AV valves, valvular endocardium, chordae tendinae, ventricular or atrium • may be due to immune complex deposition with inflammation will also see malar rash, and murmur

Question 80

carcinoid tumors

Answer 80

Carcinoid Heart Disease: - carcinoid syndrome = systemic disorder marked by flushing, diarrhea, dermatitis, bronchoconstriction caused by release of bioactive compounds like serotonin from carcinoid tumors (serotonin, kallikrein, bradykinin, histamine, prostaglandin) --> irritate heart * Fibrous intimal thickening of the endocardial surfaces of the right side of the heart, particularly right ventricle, and the tricuspid and pulmonic valves * Histo: see glistening white intimal plaquelike thickenings tumor in gut --> affects right heart tumor in lungs --> affects left heart

Question 81

problems w/ artifical valves

Answer 81

- thromboembolism is major consideration with mechanical valves - structural deterioration is main problem of bioprosthesis - infective endocarditis is potentially serious complication of any valve replacement * pt. needs to be on coumadin/warfarin b/c are in high coagulable state

Question 82

cardiomyopathies

Answer 82

“heart muscle disease” - Cardiomyopathy = heart disease resulting from primary abnormality in the myocardium (including inflammatory, immunologic disorders, muscular dystrophies and genetic disorders of myocardium)

Question 83

dilated cardiomyopathy

Answer 83

large flabby heart, hypocontracting • DCM → poor heart contraction → slowly progressive end stage disease • familial in 30-50% of cases • can be due to myocarditis (Cocksakie B), alcohol abuse, peripartum cardiomyopathy, iron overload, supraphysiologic stress, autosomal dominant inheritance • LVEF: <25% pt. will soon die Most common indication for cardiac transplantation Histology: • see markedly increased size, large and flabby mass with dilation of all chambers. Mural thrombi are common and see coronary arteries free of narrowing with normal valves

Question 84

defects in cytoskeletal proteins; anchoring proteins

Answer 84

DCM

Question 85

defects in myocytes, they are unorganized and problems with contractile proteins

Answer 85

hypertrophic cardiomyopathy

Question 86

hypertrophic cardiomyopathy

Answer 86

= marked thickened left ventricle, hypercontracting • poorly compliant left ventricle → abnormal diastolic filling and ventricular outflow obstruction • heart is thick-walled, heavy, hypercontracting • see massive myocardial hypertrophy without ventricular dilation • assymetric septal hypertrophy: Disproportionate thickening of left ventricular septum in comparison to LV free wall • ventricular cavity may be compressed into “banana-like” shape • see myofiber disarray and haphazard disarray of myocytes • LVEF 50-80%: impairment of compliance and diastolic function: mimics HHD, aortic stenosis • mostly dominant mutations involving proteins of the sarcomere (mutations of Beta-myosin heavy chain on Ch 14) Clinical: • stable and nonprogressive course: pt. often live until 7th/8th decade • Majority of patients have reduced stroke volume due to impaired diastolic filling • exertional dyspnea, • harsh systolic ejection murmur, • can lead to A fib and sudden death • *** one of most common causes of SCD in young athletes **** Histology: • Marked myocyte hypertrophy, haphazard myofiber disarray • interstitial and replacement fibrosis in myocardium

Question 87

SCD in someone active?

Answer 87

think hypertrophic cardiomyopathy

Question 88

What are differences b/w hypertrophic vs. Dilated cardiomyopathy?

Answer 88

- In HCM the ventricle is firms, small, thick walled, hypercontracting, diastolic dysfunction, shows exertional dyspnea and mutations exist in sarcomere proteins - In DCM the ventricle is dilated and flabby, hypocontracting, systolic dysfunction, leads to CHF, and dysfunction exists in cytoskeletal proteins - Both HCM and DCM can lead to heart failure, sudden death, A fib, and stroke

Question 89

Restrictive cardiomyopathy?

Answer 89

= mild increase in cardiac mass without increase in volume of left ventricle • characterized by decrease in ventricular compliance → impaired ventricular filling during diastole • ventricles are normal sized or slightly enlarged, but not dilated – and systolic function usually unaffected • myocardium is firm and noncompliant • LVEF: 45 to 90%, impaired compliance (diastole), caused by amyloidosis, induced fibrosis: mimics pericardial constriction Heart findings: • ventricles are normal size or slightly enlarged • ventricular chambers are normal size • Biatrial enlargement is common Associated Disorders: • Amyloidosis • Endomyocardial fibrosis: - endocardial and subendocardial fibrosis with mural thrombi, occurs in African children/young adults and in other tropical areas (most common worldwide) • Loeffler endomyocarditis - endomyocardial fibrosis with large mural thrombi that occurs worldwide and is associated with eosinophilic leukemia [some have platelet derived growth factor receptor abnormalities (Rx tyrosine kinase inhibitor imatinib) or may be secondary to basic protein release] • Endocardial fibroelastosis - multifactorial left ventricular endocardial fibrosis that usually occurs in first 2 years of life

Question 90

Arrythmogenic Right Ventricular Cardiomyopathy

Answer 90

(Right ventricular Dysplasia): subtype of DCM * see mm. replaced by fat and collagen * See right sided heart failiure and rhythm disturbances (V-tach) * one of the causes of unexpected death found in 2-5% of deaths of young people * results in right ventricle (and sometimes left ventricle) being severely thinned with decreased myocytes and replacement with adipocytes and interstitial fibrous tissue * Most due to autosomal dominant mutations: Plakoglobin and desmoplakin genes * Naxos syndrome: – palmoplantar keratoderma with arrhythmogenic right ventricular cardiomyopathy and woolly hair (recessive plakoglobin mutation) * Carvajal syndrome - palmoplantar keratoderma with left ventricular cardiomyopathy and woolly hair (recessive desmoplakin mutation) *** make ddx by looking at right ventricle, its all fat!

Question 91

mutations in plakoglobin and desmoplakin genes

Answer 91

Arrythmogenic Right Ventricular Cardiomyopathy (Right ventricular Dysplasia):

Question 92

Amyloidosis:

Answer 92

interstitial deposition of amyloid protein resulting in restrictive cardiomyopathy which may be complicated by arrhtymias transtheritin protein deposit most common

Question 93

Systemic Senile (Cardiac) Amyloidosis

Answer 93

* Amyloidosis primarily in heart: Occurs in “aged” patients (>60 yrs.) * May be localized to both ventricular and atrial myocardium - or limited to atria (isolated atrial amyloidosis) * Far more common cause of cardiac amyloidosis than primary systemic amyloidosis * Much better prognosis and milder clinical course * Amyloid composed of transthyretin product * More common in African-Americans (transthyretin mutation)

Question 94

myocarditis

Answer 94

inflammatory process due to infectious microorganisms → myocardial injury Major Causes Myocarditis in US • Viral: Coxsackieviruses A and B and other enteroviruses account for vast majority of cases - see lymphatic invasion • Lyme Disease (Borrelia burgorferi): Occurs in 5% patients with clinically symptomatic Lyme disease (AV block) • Hypersensitivity (eosinophilic) myocarditis – see esoinophils! • Trichinosis (Trichinella spiralis and others): helminth acquired from eating infected undercooked meat from bears, pigs, boars, deer, warlus, dogs, cats, cougar, horses, wolves, foxes, mice, rats, humans and other animals.

Question 95

chagas disease

Answer 95

Major Cause of myocarditis South America: Chagas Disease (Trypanosoma cruzi) : a parasitic disease that affects 50% of patients in endemic areas of parasite. In Brazil, Chagas Disease is a more common cause of cardiac disease than ischemic heart disease = cardiomyopathy and hx of travel

Question 96

round mononuclear cells

Answer 96

lymphocytes or macrophages = viral infection

Question 97

lobated w/ two lobes

Answer 97

eos = allergic txn

Question 98

4 lobes

Answer 98

neutrophil

Question 99

most common cardiac tumor?

Answer 99

metastatic malignancy

Question 100

myxoma

Answer 100

cardiac tumor made of mucoid material - often in left atria and fossa ovalis - can cause fever and malaise

Question 101

most common cardiac tumor in children?

Answer 101

benign • Rhabdomyoma (#1 in children and 50% associated with tuberous sclerosis/ TSC1 and TSC2 genes)

Question 102

pericardial effusions

Answer 102

* Serous: CHF, hypoalbuminemia - pale yellow, transparent * Serosanguinous: Malignancy, trauma, ruptured MI, aortic dissection * Sanguinous: Hemopericardium (aortic/cardiac rupture) - just blood * Purulent: Infection - neutrophils, yellowish green * Chylous: Lymphatic obstruction - white, look for malignancies! * Malignant (neoplastic): associated with malignant cells

Question 103

#1 systemic cause of pericarditis?

Answer 103

uremia - renal faiure, looks like a shaggy heart

Question 104

types of pericarditis?

Answer 104

* Serous: RF*, SLE**, scleroderma, tumors, uremia, Dressler syndrome (immune reaction post myocardial/pericardial injury) * Fibrinous/serofibrinous: Myocardial infarct, Dressler syndrome, uremia, radiation RF, SLE, trauma, cardiac surgery * Purulent/supperative: Infections * Constrictive: pericardium is rigid, thickened, scarred, and less elastic than normal * Hemorrhagic: Neoplasia, bacteria, TB***, bleeding diathesis, cardiac surgery * Casseous: TB or fungus * Adhesive: fibrous or fibroelastic scar, if severe is called concretio cordis and may calcify * Malignant (neoplastic): associated with malignant cells