ICL 2.10: Endo Cardio and Valvular Heart Disease Flashcards

1
Q

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
A

rheumatic fever!!

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

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2
Q

what is rheumatic fever?

A
  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

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3
Q

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

A
  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!

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4
Q

what age does streptococcal pharyngitis usually effect?

A

age 5-15 with a peak at 6 years

season is late winter to early spring

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5
Q

what is Jones criteria?**

A

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
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6
Q

what is the arthritis seen in RF?

A

PAL = polyarthritis of large joints

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7
Q

what is the carditis seen in RF?

A

pancarditis! it causes pericarditis, myocarditis and endocarditis

pericarditis –> friction rub, sharp chest pain

myocarditis –> CHF, cardiomegaly

endocarditis –> murmur; valve involvement

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8
Q

what are the subcutaneous nodules seen in RF?

A

painless pea sized swellings over bony prominences

they are seen in extensor tendons of the hands and feet

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9
Q

what is the chorea seen in RF?

A

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

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10
Q

what is erythema marginatum seen in RF?

A

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)

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11
Q

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

A

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

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12
Q

what are the pathognomonic lesions seen with RF?

A

Aschoff bodies found in the heart with myocarditis

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

  1. intermediate –> proliferative
  2. 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

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13
Q

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

A
  1. Antischkow cells*
  2. Aschoff cells*
  3. lymphocytes
  4. plasma cells
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14
Q

what are Antischkow cells?

A

a type of cell seen in Aschoff bodies in RF

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

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15
Q

what are Aschoff cells?

A

a type of cell seen in Aschoff bodies in RF

modified myocytes that are giant cells with owl-eye nucleus

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16
Q

what are the characteristics of pericarditis seen with RF?

A

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

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17
Q

which 3 conditions can give you fibrinous pericarditis?

A
  1. RF
  2. uremic pericarditis in ESRD
  3. post-MI pericarditis
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18
Q

what are the characteristics of myocarditis seen with RF?

A

Aschoff bodies

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19
Q

what are the characteristics of endocarditis seen with RF?

A

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

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20
Q

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

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

specifically, a “fish mouth” mitral valve

  1. thickening and shortening of the chordae tendineae
  2. map-like thickening of left atrial endocardium (also called McCallum’s Patch)
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21
Q

what is the prognosis of RF?

A

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!

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22
Q

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

A
  1. heart failure due to valvular deformities or myocarditis
  2. bacterial endocarditis
  3. embolism
  4. sudden death from arrhythmia or coronary angitis
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23
Q

what is Kowasaki disease?

A

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

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24
Q

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

A

sharp pain = pericardial (friction rub)

pressure = ischemic; MI

tearing, radiating down back = dissecting aneurysm or cardiac tamponade

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25
Q

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?

A

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

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26
Q

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?

A

infective endocarditis

IV drug use probably lead to a staph infection

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27
Q

what is a big risk with infective endocarditis?

A

irregular reddish tan vegetations overlie valve cusps that are being destroyed

portions of the vegetation can break off and become septic emboli

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28
Q

what is infective endocarditis?

A

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

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29
Q

what are the 2 types of infective endocarditis?

A
  1. acute

2. subacute

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30
Q

what is acute infective endocarditis?

A
  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
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31
Q

what is subacute infective endocarditis?

A
  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

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32
Q

what is the pathogenesis of infective endocarditis?**

A

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

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33
Q

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

A
  1. rheumatic heart disease
  2. myxomatous mitral valve
  3. calcific stenosis
  4. bicuspid valves
  5. prosthetic valves
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34
Q

what are the risk factors that predispose you too endocarditis?

A
  1. neutropenia
  2. alcoholism
  3. IV drug use
  4. diabetes
  5. immunosuppression
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35
Q

which microorganisms are most likely to cause endocarditis?

A
  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
36
Q

what is the morphology of infectious endocarditis?

A

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

37
Q

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

A

the vegetations are composed of:
1. fibrin

  1. inflammatory cells
  2. bacteria
  3. granulation tissue in subacute endocarditis because there’s a chance for healing = collagen, lymphocytes and new vessels
38
Q

what is the clinical presentation of infectious endocarditis?

A
  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
39
Q

how do you diagnose infectious endocarditis?

A
  1. blood cultures

2. echo to demonstrate vegetations

40
Q

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

A
  1. erosion of myocardium (acute)
  2. systemic emboli (lung and brain)
  3. septal infarcts
41
Q

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?

A

non-infectious endocarditis = marantic endocarditis

42
Q

what is non-infectious endocarditis?

A

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

43
Q

what are the underlying causes of non-infectious endocarditis?

A
  1. underlying debilitating diseases (Alzheimers)
  2. malignancies (specially mucinous adenocarcinoma of pancreas Trousseau sign)
  3. hypercoagulable states (SLE)
  4. sepsis
44
Q

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?

A

Libman-Sacks Disease

45
Q

what is Libman-Sacks disease?

A
  1. SLE endocarditis
  2. small vegetations on BOTH sides of the valves
  3. fibrinoid necrosis
  4. hematoxylin bodies = naked nuclei in the lesions
46
Q

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?

A

carcinoid heart disease

a tumor associated with over production of serotonin:

  1. diarrhea = increased intestinal motbility
  2. asthma = bronchoconstrictor
  3. flushing = vasodilator
47
Q

what is carcinoid heart disease?

A

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

48
Q

what is mitral valve prolapse?

A

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

49
Q

what are the clinical features of mitral valve prolapse?

A
  1. mostly asymptomatic
  2. mid systolic click**
  3. usually found on echocardiography
  4. rarely chest pain
50
Q

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

A
  1. ballooning of leaflets with elongated thin chordae = parachute valves**
  2. rarely annular dilatation with resulting insufficiency
51
Q

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

A
  1. attenuation of the fibrous layer of the valve
  2. thickening of the spongiosa layer
  3. myxomatous degeneration**
52
Q

what are the complications seen with mitral valve prolapse?

A
  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

53
Q

what is mitral regurgitation? what murmur will you hear?

A

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

54
Q

what do you hear with mitral stenosis?

A

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!

55
Q

what is calcified aortic stenosis?

A

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**

56
Q

what are the clinical features of aortic stenosis?

A
  1. angina
  2. CHF
  3. syncope** –> due to reduced systemic outflow, specially to the brain
57
Q

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

A

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!

58
Q

which conditions can cause aortic stenosis?

A
  1. rheumatic fever
  2. coexisting mitral stenosis

due to fusion of aortic valve commissures

59
Q

what are the complications seen with aortic stenosis?

A
  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

60
Q

which conditions are associated with aortic regurgitation?

A
  1. syphilis
  2. infective endocarditis
  3. early blowing diastolic murmur
61
Q

what are the effects of aortic regurgitation on the heart?

A
  1. increased pulse pressure
  2. decreased diastolic pressure due to regurgitation
  3. increased systolic pressure due to increased stroke volume
62
Q

what is the clinical presentation of aortic regurgitation?

A
  1. bounding pulse = water hammer pulse
  2. pulsating nail bed = Quincke pulse
  3. head bobbing
63
Q

what are the complications associated with artificial valves?

A
  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
64
Q

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?

A

PMH:

  1. Arthralgias
  2. Sore throat
  3. Fevers in the last several weeks

diagnosis = RF

65
Q

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?

A

subacute infective endocarditis

RF probably caused abnormal valves

he also has Osler nodules which are painful and on not on bony prominences

66
Q

hemorrhagic painless lesion on palms and soles

A

Jennaway lesions

67
Q

myxomatous degernation

A

mitral valve prolapse

68
Q

syncope

A

aortic stenosis

69
Q

mid systolic click

A

mitral valve prolapse

70
Q

marantic endocarditis

A

non-infectious endocarditis

pancreatic tumor, Trousseau sign*

71
Q

vegetations on both sides of the valves

A

Libman-Sacks Disease

SLE

72
Q

hematoxylin bodies

A

Libman-Sacks Disease

73
Q

caterpillar nucleus

A

Antischkow cells in RF

74
Q

fish mouth mitral valve

A

RF

75
Q

cause of stroke in infective endocarditis?

A

septic emboli

76
Q

enterococcus endocarditis association?

A

colonic diseases

77
Q

tricuspid endocarditis is associated with what?

A

IV drug use

staph aureus

78
Q

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

A

red on the edges of the rash and it’s on the TRUNK, not on the face!!

blanches

associated with RF

79
Q

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

A

inflammation of subcutaneous fat = painful nodules on the shin

associated with inflammatory bowel diseases and sarcoidosis

80
Q

what is erythema multiforme associated with?

A

associated with antibiotics, malignancies and mycoplasma pneumonea

81
Q

Trousseau sign

A

marantic endocarditis

82
Q

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?

A

no

arthritis = major

fever = minor

you need 2 major or 1 major and 2 minor

83
Q

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

A

mitral valve prolapse

valve will look like a parachute!

84
Q

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

A

aortic regurgitation

check if he’s had prior syphilis

85
Q

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?

A

Libman-Sacks

hematoxylin bodies