ICL 2.10: Endo Cardio and Valvular Heart Disease

Question 1

an 8-year-old boy brought in by his mother because of swollen, red, tender left knee and right wrist. He also has a reddish rash on his chest

he has not been feeling well since he had a sore throat a couple of weeks ago

physical examination reveals:

• temperature of 101 degrees
• pulse 100/minute
• friction rub heard on chest auscultation

Answer 1

rheumatic fever!!

3 cardinal signs of inflammation: swelling, redness, pain/tender –> in multiple joints!!

Question 2

what is rheumatic fever?

Answer 2

1. systemic disease –> skin, joint and heart involvement
2. post-strep infection
3. non-suppurative –> don’t see puss or neutrophils!
4. joint involvement is common, benign and reversible
5. heart damage can be disabling and irreversible…

disease varies in duration and severity

Question 3

which parts of the body are involved with a streptococcal pharyngitis infection?

Answer 3

1. heart
2. joints
3. tendons
4. skin
5. respiratory
6. vessels
7. tendon sheaths
8. serial membranes

all may be effected to varying degrees after a strep infection!

Question 4

what age does streptococcal pharyngitis usually effect?

Answer 4

age 5-15 with a peak at 6 years

season is late winter to early spring

Question 5

what is Jones criteria?**

Answer 5

these are the criteria used to make the diagnosis of rheumatic fever

2 major or 1 major and 2 minor = high probability of RF

MAJOR

1. carditis
2. arthritis
3. chorea
4. erythema marginatum
5. subcutaneous nodules

MINOR

1. fever
2. arthralgia
3. increased ESR
4. increased PR interval
5. leukocytosis

Question 6

what is the arthritis seen in RF?

Answer 6

PAL = polyarthritis of large joints

Question 7

what is the carditis seen in RF?

Answer 7

pancarditis! it causes pericarditis, myocarditis and endocarditis

pericarditis –> friction rub, sharp chest pain

myocarditis –> CHF, cardiomegaly

endocarditis –> murmur; valve involvement

Question 8

what are the subcutaneous nodules seen in RF?

Answer 8

painless pea sized swellings over bony prominences

they are seen in extensor tendons of the hands and feet

Question 9

what is the chorea seen in RF?

Answer 9

Sydenham’s chorea = chorea minor –> this is different from the chorea seen in Hungtingon

this chorea is sudden, aimless, irregular movement accompanies by muscle weakness and emotional instability

it’s a delayed manifestation of RF; it doesn’t appear until like 6 months later until all the other RF symptoms are gone

Question 10

what is erythema marginatum seen in RF?

Answer 10

pink evanescent rash = blanches when you push on it

rash has a clear center with red serpiginous margins on the TRUNK; never on the face!!!!

transient, migratory and nonpruritic (doesn’t itch)

Question 11

what test do you use to see if a patient had a strep infection?

Answer 11

ASO = antistreptolysin O test

this test is the most widely used and best standardized to see if the patient had preceding streptococcal infection!

you can run a single titer –> 250 Todd units in adults and 333 Todd units in children would be diagnostic of RF

HOWEVER, not having an elevated ASO titer doesn’t rule out RF if you have Jone’s criteria

Question 12

what are the pathognomonic lesions seen with RF?

Answer 12

Aschoff bodies found in the heart with myocarditis

their composition varies on which stage of RF it is:
1. early –> necrosis

2. intermediate –> proliferative
3. late –> healed

so if I had RF when I was 5 years old, you’re not going to see Aschoff bodies, you’ll see scares from where they used to be but if it’s the kid in the case, you’d see the proliferative phase of the Aschoff bodies

Question 13

what are the cell types seen in the Aschoff bodies in RF?

Answer 13

1. Antischkow cells*
2. Aschoff cells*
3. lymphocytes
4. plasma cells

Question 14

what are Antischkow cells?

Answer 14

a type of cell seen in Aschoff bodies in RF

modified myocytes that are elongated and have a nucleus that looks like a caterpillar

Question 15

what are Aschoff cells?

Answer 15

a type of cell seen in Aschoff bodies in RF

modified myocytes that are giant cells with owl-eye nucleus

Question 16

what are the characteristics of pericarditis seen with RF?

Answer 16

it’s diffuse and nonspecific

fibrinous*! which means it may resolve or organize forming thickened plaques

micro = fibrin + lymphocytes + histiocytes + occassional PMNS

fibrinous is important because that’s what gives you friction rub because the fibrin is sitting in-between the layers of the pericardium and giving you the rub

Question 17

which 3 conditions can give you fibrinous pericarditis?

Answer 17

1. RF
2. uremic pericarditis in ESRD
3. post-MI pericarditis

Question 18

what are the characteristics of myocarditis seen with RF?

Answer 18

Aschoff bodies

Question 19

what are the characteristics of endocarditis seen with RF?

Answer 19

endocarditis is important because eventually pericarditis and myocarditis will resolve and not leave long-term disability effects on the heart but endocarditis will!

it’s called verrucous endocarditis = little warts on the valves of the heart

the warts are at the line of closure of the valves on the side of blood flow so they’re on the atrial surface of the AV valves and ventricular surface of the semilunar valves

the endocarditis can lead to McCallum Patches which are thickened plaque on the valves

Question 20

what are the potential long term effects of endocarditis seen with RF?

Answer 20

1. fibrous thickening of valve leaflets at the line of closure that causes valve deformity

specifically, a “fish mouth” mitral valve

2. thickening and shortening of the chordae tendineae
3. map-like thickening of left atrial endocardium (also called McCallum’s Patch)

Question 21

what is the prognosis of RF?

Answer 21

75% recover in 6 weeks

90% recover in 12 weeks

5% persist for 6 months

you need to make sure they don’t get recurrent strep infections!

Question 22

what are the causes of death in patients with RF?**

Answer 22

1. heart failure due to valvular deformities or myocarditis
2. bacterial endocarditis
3. embolism
4. sudden death from arrhythmia or coronary angitis

Question 23

what is Kowasaki disease?

Answer 23

inflammatory disease we THINK is due to an immune reaction

it’s a systemic reaction

kids with Kowasaki can die of coronary artery disease; it’s basically a vasculitis

Question 24

what do the different types of chest pain tell you? like if it’s sharp vs. pressure vs. tearing?

Answer 24

sharp pain = pericardial (friction rub)

pressure = ischemic; MI

tearing, radiating down back = dissecting aneurysm or cardiac tamponade

Question 25

An 18 year old boy presents to the ER with fever and tachycardia. 5 days after tooth extraction. PE: - temp 102° F - new systolic murmur - hemorrhagic spot on his retina DD? What is your next step?

Answer 25

infective endocarditis need to do an echo because of the murmur and you need to do a blood culture to identify the bacteria probably didn't have prophylactic antibiotics before tooth extraction and bacteria traveled to the heart and he developed a murmur kid has a new murmur = valve involvement --> myocarditis gives us HF and pericarditis doesn't give us murmurs; only endocarditis and valvular involvement do! hemmorhagic retina = infective carditis

Question 26

this 32-year-old-man was found down in his apartment when a friend came to visit. The friend called 911. On arrival in the emergency room, the patient was febrile, with a temperature of 38.9°C physician examination revealed a palpable spleen tip, *splinter hemorrhages, needle tracks in the left antecubital fossa, and a heart murmur echocardiography reveals nodular lesions up to 1 cm involving the tricuspid valve, which also shows valvular insufficiency diagnosis?

Answer 26

infective endocarditis IV drug use probably lead to a staph infection

Question 27

what is a big risk with infective endocarditis?

Answer 27

irregular reddish tan vegetations overlie valve cusps that are being destroyed portions of the vegetation can break off and become septic emboli

Question 28

what is infective endocarditis?

Answer 28

a serious infectious disease characterized by colonization or invasion of heart valves or mural endocardium by microorganisms

Question 29

what are the 2 types of infective endocarditis?

Answer 29

1. acute | 2. subacute

Question 30

what is acute infective endocarditis?

Answer 30

1. usually occurs on a normal valve 2. caused by highly virulent microorganisms (staph aureus) 3. destructive rapidly progressive course (destroys valves) 4. death within days to weeks in spite of aggressive therapy

Question 31

what is subacute infective endocarditis?

Answer 31

1. usually occurs on abnormal valves 2. less virulent microorganisms (streptococcal infection) 3. indolent course, weeks to months 4. full recovery with treatment in most cases less destructive lesions than in acute endocarditis

Question 32

what is the pathogenesis of infective endocarditis?**

Answer 32

high velocity flow + high pressure gradient across narrow office like valves --> endothelial denuding --> deposition of platelets and fibrin --> colonization with bacteria

Question 33

what are some of the valve abnormalities that could cause subacute endocarditis?

Answer 33

1. rheumatic heart disease 2. myxomatous mitral valve 3. calcific stenosis 4. bicuspid valves 5. prosthetic valves

Question 34

what are the risk factors that predispose you too endocarditis?

Answer 34

1. neutropenia 2. alcoholism 3. IV drug use 4. diabetes 5. immunosuppression

Question 35

which microorganisms are most likely to cause endocarditis?

Answer 35

1. staph aureus in acute endocarditis 2. strep viridian's in subacute endocarditis ``` HACEK are commensal in the oral cavity and can also cause it: Ⓗaemophilus Ⓐctinobacillus Ⓒardiobacterium Ⓔikenella Ⓚingella ```

Question 36

what is the morphology of infectious endocarditis?

Answer 36

*friable* bulky vegetations with destruction of underlying valves (specially in the acute type) friable is important because the bad part with endocarditis is when these vegetations wash off and become emboli

Question 37

what is the microscopic morphology of the vegetations seen in infectious endocarditis?

Answer 37

the vegetations are composed of: 1. fibrin 2. inflammatory cells 3. bacteria 4. granulation tissue in subacute endocarditis because there's a chance for healing = collagen, lymphocytes and new vessels

Question 38

what is the clinical presentation of infectious endocarditis?

Answer 38

1. fevers 2. chills 3. malaise 4. immunologic complications like glomerulonephritis** 5. mechanical complications = murmurs 6. microemboli** - petechiae - splinter hemorrhages on the nails - painless hemmorhagic Janeway lesions on palms and soles - painful subcutaneous Osler nodules on pulp of fingers (RF has painless nodules) - Roth spots on retina

Question 39

how do you diagnose infectious endocarditis?

Answer 39

1. blood cultures | 2. echo to demonstrate vegetations

Question 40

what are some of the complications that can develop with infectious endocarditis?

Answer 40

1. erosion of myocardium (acute) 2. systemic emboli (lung and brain) 3. septal infarcts

Question 41

84 year old woman with a history of Alzheimer disease dies at the nursing home due to acute Bronchopneumonia. The famil request an autopsy. what might you expect to see in her heart?

Answer 41

non-infectious endocarditis = marantic endocarditis

Question 42

what is non-infectious endocarditis?

Answer 42

aka marantic endocarditis a non-destricutive, sterile endocarditis with very small vegetations along the lines of the closure of the valves tends to occur in persons with a hypercoagulable state no thrombi, no inflammation, no valve damage sterile = no microorganisms

Question 43

what are the underlying causes of non-infectious endocarditis?

Answer 43

1. underlying debilitating diseases (Alzheimers) 2. malignancies (specially mucinous adenocarcinoma of pancreas *Trousseau sign*) 3. hypercoagulable states (SLE) 4. sepsis

Question 44

a 45 year old S LE patient presents with shortness of breath and Echo reveals small vegetation’s on both sides of her mitral valve diagnosis?

Answer 44

Libman-Sacks Disease

Question 45

what is Libman-Sacks disease?

Answer 45

1. SLE endocarditis 2. small vegetations on BOTH sides of the valves 3. fibrinoid necrosis 4. hematoxylin bodies = naked nuclei in the lesions

Question 46

a 55 year old male presents with the chief complaint of flushing and diarrhea he has been treated for Asthma for the last six months diagnosis? tests? echo results?

Answer 46

carcinoid heart disease a tumor associated with over production of serotonin: 1. diarrhea = increased intestinal motbility 2. asthma = bronchoconstrictor 3. flushing = vasodilator

Question 47

what is carcinoid heart disease?

Answer 47

a tumor associated with over production of serotonin patients have lesions on the tricuspid valve, thickening of the valve due to plaques formed by the deposition of mucopolysaccharides clinical presentation = diarrhea, asthma and flushing

Question 48

what is mitral valve prolapse?

Answer 48

aka floppy valve syndrome, Barlow's syndrome or myxomatous degeneration myxomatous degeneration = messed up extracellular matrix remodeling usually due to abnormalities in connective tissue like Marfans

Question 49

what are the clinical features of mitral valve prolapse?

Answer 49

1. mostly asymptomatic 2. mid systolic click** 3. usually found on echocardiography 4. rarely chest pain

Question 50

what are the gross morphological changes seen with mitral valve prolapse?

Answer 50

1. ballooning of leaflets with elongated thin chordae = parachute valves** 2. rarely annular dilatation with resulting insufficiency

Question 51

what are the gross microscopic changes seen with mitral valve prolapse?

Answer 51

1. attenuation of the fibrous layer of the valve 2. thickening of the spongiosa layer 3. myxomatous degeneration**

Question 52

what are the complications seen with mitral valve prolapse?

Answer 52

1. infective endocarditis 2. mitral insufficiency 3. stroke or other systemic infarcts 4. arrhythmias both ventricular and atrial THESE ARE RARE; mitral valve prolapse is usually non-symptomatic

Question 53

what is mitral regurgitation? what murmur will you hear?

Answer 53

blood returning to the left atrium you will hear a holosystolic murmur that increases with squatting due to increased systolic resistance which decreases left ventricular emptying and increases return to left atrium can happen after MI with rupture of papillary muscles

Question 54

what do you hear with mitral stenosis?

Answer 54

opening snap followed by diastolic rumble this is seen in RF with the fish mouth mitral valve you can get subacute endocarditis because the valve is abnormal!

Question 55

what is calcified aortic stenosis?

Answer 55

calcification of cusps with resulting stenosis due to fusion of aortic valve commissures commonly seen prematurely in bicuspid aortic valve anomalies due to aging wear and tear clinical features --> angina, CHF, syncope**

Question 56

what are the clinical features of aortic stenosis?

Answer 56

1. angina 2. CHF 3. syncope** --> due to reduced systemic outflow, specially to the brain

Question 57

a 75-year-old man presents with chest pain, and he states that he has “passed out a couple of times”, once hitting his head on a kitchen counter. over the last two years, he has had progressive difficulty breathing when he lays down and has had to prop himself up so that he is virtually sitting up when he sleeps. He has no known history of ischemic heart disease physical examination revealed a coarse late-peaking systolic ejection murmur, a weakened and delayed upstroke of the carotid artery pulsations. explain the above findings on the basis of cardiovascular pathophysiology

Answer 57

aortic stenosis passed out = syncope = aortic stenosis difficulty breathing = CHF from calcified aorta and increased venous return when he lays down which is a big load on the heart the murmur is due to increased pressure gradient in the stenotic valve the weak carotid pulse from the obstruction to the left ventricular outflow get an echocardiogram to see the stenotic valve!

Question 58

which conditions can cause aortic stenosis?

Answer 58

1. rheumatic fever 2. coexisting mitral stenosis due to fusion of aortic valve commissures

Question 59

what are the complications seen with aortic stenosis?

Answer 59

1. concentric left ventricular hypertrophy --> CHF 2. microangiopathic hemolytic anemia --> schistocytes on periperhal blood smear from RBCs fragmenting through the stenotic aortic valve HUS, TTP, DIC can all cause schistocytes too

Question 60

which conditions are associated with aortic regurgitation?

Answer 60

1. syphilis 2. infective endocarditis 3. early blowing diastolic murmur

Question 61

what are the effects of aortic regurgitation on the heart?

Answer 61

1. increased pulse pressure 2. decreased diastolic pressure due to regurgitation 3. increased systolic pressure due to increased stroke volume

Question 62

what is the clinical presentation of aortic regurgitation?

Answer 62

1. bounding pulse = water hammer pulse 2. pulsating nail bed = Quincke pulse 3. head bobbing

Question 63

what are the complications associated with artificial valves?

Answer 63

1. thrombosis and thromboembolism* 2. hemorrhage secondary to anti-coagulant therapy* 3. endocarditis 4. intravascular hemolysis* 5. leakage 6. obstruction 7. structural deterioration of mechanical valves or calcification of biosynthetic valves

Question 64

A 7-year-old boy was brought to the neurology clinic because of increased irritability and restlessness at school. He distracts his classmates by constantly jerking his head to one side and moving his fingers aimlessly. What should the neurologist ask the parents? Diagnosis?

Answer 64

PMH: 1. Arthralgias 2. Sore throat 3. Fevers in the last several weeks diagnosis = RF

Question 65

A 16-year-old patient with a history of rheumatic fever when he was five years old presents with tender nodules on the palmar surface of his fingers for one week and a fever of 101° what should you worry about?

Answer 65

subacute infective endocarditis RF probably caused abnormal valves he also has Osler nodules which are painful and on not on bony prominences

Question 66

hemorrhagic painless lesion on palms and soles

Answer 66

Jennaway lesions

Question 67

myxomatous degernation

Answer 67

mitral valve prolapse

Question 68

syncope

Answer 68

aortic stenosis

Question 69

mid systolic click

Answer 69

mitral valve prolapse

Question 70

marantic endocarditis

Answer 70

non-infectious endocarditis pancreatic tumor, Trousseau sign*

Question 71

vegetations on both sides of the valves

Answer 71

Libman-Sacks Disease SLE

Question 72

hematoxylin bodies

Answer 72

Libman-Sacks Disease

Question 73

caterpillar nucleus

Answer 73

Antischkow cells in RF

Question 74

fish mouth mitral valve

Answer 74

RF

Question 75

cause of stroke in infective endocarditis?

Answer 75

septic emboli

Question 76

enterococcus endocarditis association?

Answer 76

colonic diseases

Question 77

tricuspid endocarditis is associated with what?

Answer 77

IV drug use staph aureus

Question 78

what is erythema marginatum? what's it associated with?

Answer 78

red on the edges of the rash and it's on the TRUNK, not on the face!! blanches associated with RF

Question 79

what is erythema nodosum? what's it associated with?

Answer 79

inflammation of subcutaneous fat = painful nodules on the shin associated with inflammatory bowel diseases and sarcoidosis

Question 80

what is erythema multiforme associated with?

Answer 80

associated with antibiotics, malignancies and mycoplasma pneumonea

Question 81

Trousseau sign

Answer 81

marantic endocarditis

Question 82

6 year old boy presents with fever and swollen right knee. Temperature is 101 F. can you call this RF based on what we learned?

Answer 82

no arthritis = major fever = minor you need 2 major or 1 major and 2 minor

Question 83

25 year old med student has mid-systolic click. diagnosis? what will you see on the echo?

Answer 83

mitral valve prolapse valve will look like a parachute!

Question 84

65 year old male presents with water hammer pulse, head bobbing and pulsating nail beds. diagnosis?

Answer 84

aortic regurgitation check if he's had prior syphilis

Question 85

55 year old female dies of acute MI. H/o alzheimers. vegetations are seen on both sides of mitral valve. diagnosis? what will you see microscopically in the vegetations?

Answer 85

Libman-Sacks hematoxylin bodies