Other cardiovascular pathology Flashcards

1
Q

What are the three main forms of valvular heart disease?

A

pure or mixed
affect single or multiple valves

1) stenosis = valve is too narrow and fails to open completely, impeding blood flow forwards
2) incompetence (regurgitation) = failure of the valve to close completely, reverse flow of blood
3) functional regurgitation = valve becomes incompetent due to dilatation of a ventricle

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2
Q

What factors are important to consider in terms of the clinical consequences of a valvular disorder?

A

dependent upon:

  • which valve is impaired
  • degree of impairment
  • rate of disease development (rate and quality of compensatory mechanisms)
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3
Q

How does the severity of valvular heart disease vary?

A

range from physiologically unimportant (rheumatic mitral stenosis) to severe and rapidly fatal (acute aortic incompetence)

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4
Q

How can valvular incompetence arise?

A

due to intrinsic disease of the valve cusps, or damage to/distortion of supporting structures (aorta, papillary muscles and tendinous cords)
Can be superimposed on underlying valve abnormality
Acute or chronic condition

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5
Q

What is the usual underlying cause of valvular stenosis including examples?

A

Valvular stenosis almost always has an underlying valve abnormality (chronic)

  • valvular calcification = form of stenosis that affects aortic and mitral valves
    e. g. mitral annular calcification, calcific aortic stenosis (common with age) and calcification of a congenitally bicuspid aortic valve (normally 3 cusps)
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6
Q

What is the mechanism causing valvular stenosis?

A

heart valves subject to high repetitive mechanical stress, especially at the hinge points of the cusps therefore cumulative damage is further complicated by dystrophic calcification

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7
Q

What is mitral incompetence?

A

myxomatous degeneration and mitral valve prolapse
- very common
often both mitral valves will become enlarged, hooded and redundant causing them to prolapse back into the left atrium during systole
- very rarely it can lead to sudden death

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8
Q

What is a common cause of mitral stenosis?

A

rheumatic fever - due to acute immunologically mediated inflammatory disease that follows infection with group A beta-hemolytic strep

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9
Q

Other than mitral stenosis what are the other associated symptoms caused by rheumatic fever?

A

migratory polyarthritis of large joints, carditis, subcutaneous nodules, skin rash, and sydenham chorea

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10
Q

What can happen to the heart in acute rheumatic heart disease?

A

pancarditis

  • endocardium will form vegetations
  • myocardium will form inflammatory foci with aschoff bodies
  • pericardium will be inflamed

this damage can accumulate with repeated infections leading to chronic rheumatic heart disease

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11
Q

What is chronic rheumatic heart disease characterized by?

A

deforming fibrotic valvular disease (fish mouth) with leaflet thickening
commissural fusion and shortening
thickening and fusion of the tendinous cords

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12
Q

What is infective endocarditis?

A

serious (usually bacterial) infection that colonise the heart valves = results in the formation of friable bulky vegetations with underlying tissue destruction
- acute and subacute forms

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13
Q

What are the characteristics of acute infective endocarditis ? (virulence, valve, presentation, outcome, lesion, organisms)

A
virulence= high 
valve= previously normal
presentation= acute onset
outcome = 50% mortality in days-weeks
lesion= necrotising, ulcerative, invasive 
organisms= staph aureus
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14
Q

What are the characteristics of subacute infective endocarditis ? (virulence, valve, presentation, outcome, lesion, organisms)

A
virulence= low 
valve= usually abnormal
presentation= insidious onset
outcome = recover in weeks-months
lesion= less destructive 
organisms= alpha hemolytic strep, mouth commensals, staph epidermis
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15
Q

What factors predispose you to infective endocarditis?

A
abnormal valves 
immunosuppression 
diabetes
alcoholism 
IV drug use 
e.g. anything that can lead to bacteraemia - dental procedures
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16
Q

Which valves are most commonly affected by endocarditis?

A

aortic and mitral valves are most commonly affected - however tricuspid is often affected with IV drug use

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17
Q

What endocarditis in the tricuspid valve due to IV drug use lead to ?

A
formation of vegetations
myocardial abscess
valve rupture
systemic emboli 
septic emboli 
immune complex formation
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18
Q

What does non-bacterial thrombotic endocarditis involve?

A

deposition of fibrin and platelet thrombi on valve leaflets - can be on either side of the heart and will affect previously normal valves
As it is sterile it is a non-destructive process but can cause emboli

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19
Q

When does non-bacterial thrombotic endocarditis typically occur?

A

hypercoaguable states such as DIC, cancer and sepsis

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20
Q

What are the 2 main forms of artificial valves?

A

mechanical and bioprothesis (often porcine xenografts)

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21
Q

What are the complications of artificial valves?

A

thromboembolic hence long term anticoagulants must be taken
increase susceptibility to infective endocarditis and
structure deteriorates through age

22
Q

What is cardiomyopathy?

A

heart disease resulting from a primary abnormality in the myocardium
caused by: inflammation, immunological disease, systemic metabolic disorders, muscular dystrophies and genetic myocyte abnormalities or it can be idiopathic

23
Q

What are the 3 main clinical pictures of cardiomyopathy?

A

1) dilated cardiomyopathy
2) hypertrophic cardiomyopathy
3) restrictive cardiomyopathy
- each has a spectrum of severity and there can be overlap between them

24
Q

How are cardiomyopathies diagnosed?

A

endomyocardial biopsy of the right ventricle

25
Q

What are the characteristics of dilated cardiomyopathy?

A

progressive hypertrophy
dilation
contractile dysfunction
leads to congestive cardiac failure

26
Q

What are the causes of dilated cardiomyopathy?

A
most cases are idiopathic
alcohol 
peripartum 
genetic
myocarditis 
hemochromatosis 
chronic anaemia 
chemotherapy 
sarcoidosis
27
Q

What are the morphological changes seen in dilated cardiomyopathy?

A

2-3x heavier than normal with dilation of all chambers - may be mural thrombi as well as mitral and tricuspid regurgitation
coronary arteries will be normal

28
Q

Histologically, what can be seen in dilated cardiomyopathy?

A

fibres will be hypertrophied and attenuated

there will also be fibrosis

29
Q

What are the clinical features of dilated cardiomyopathy ?

A

slowly progressive congestive heart failure occur at any age
Ejection fraction can be as low as 25% leading to a high rate mortality from arrhythmia or heart failure
- treatment is cardiac transplant

30
Q

What are the characteristics of hypertrophic cardiomyopathy?

A

myocardial hypertrophy with abnormal diastolic filling - consists of heavy muscular hyper contracting heart

  • no ventricular dilation
  • asymmetrical septal hypertrophy
  • extensive myocyte hypertrophy and disarray with interstitial fibrosis
31
Q

What type of inheritance is hypertrophic cardiomyopathy?

A

usually autosomal dominant with variable penetrance there are many different mutations in four genes that encode sarcomeres

  • 1) beta myosin heavy chain
  • 2) cardiac troponin T
  • 3) alpha tropomyosin
  • 4) myosin binding protein C
32
Q

What are the clinical features of hypertrophic cardiomyopathy?

A

decreased chamber size coupled with poor compliance = decreased SV

33
Q

What clinical problems are associated with hypertrophic cardiomyopathy?

A
angina 
atrial fibrillation 
cardiac failure 
ventricular arrhythmia 
sudden death
34
Q

What is restrictive cardiomyopathy due to?

A
35
Q

What can cause restrictive cardiomyopathy?

A

caused by radiation fibrosis, amyloidosis, sarcoidosis, metastatic tumor, inborn error in metabolism, endomyocardial fibrosis and endocardial fibroelasotosis

36
Q

What are the clinical features of restrictive cardiomyopathy?

A

heart ventricles and chambers will be normal sized

both atrial will be dilated and the myocardium will be fine

37
Q

What is myocarditis?

A

inflammatory process of the myocardium = injury to myocytes

38
Q

What can cause myocarditis?

A

viral infection
sarcoidosis
immune causes: post-viral, post-streptococcal, SLE, drug hypersensitivity and transplant rejection

39
Q

Clinically how can myocarditis present?

A

either be asymptomatic and later result in DCM
or
lead to arrthymia and acute heart failure with sudden death

40
Q

What is pericardial effusion?

A

fluid of a variety of compositions occupies the pericardial sac
- if the fluid is large enough and rapidly develops it can lead to cardiac tamponade

41
Q

What are the different compositions of fluid that can occur in pericardial effusion?

A
  • transudate = clear fluid forms against a pressure gradient
  • exudate = forms in inflammation and contains proteins
  • haemopericardium = fills the pericardium with blood and can be due to ventricular rupture
  • purulent pericarditis = thick pussy exudate
42
Q

What is pericarditis?

A

inflammation of the pericardium - can be secondary to cardiac disease, thoracic or systemic disorders or metastasis from a distant site

43
Q

What are the causes of pericarditis?

A

infections
immune mediated
miscellaneous

44
Q

What are the different types of pericarditis?

A
serous 
fibrinous 
purulent 
hemorrhagic 
caseous
45
Q

What can happen to the fluid in pericarditis?

A

it can be reabsorbed, resolve itself or become organized and obliterate the pericardial space - this can lead to constrictive pericarditis if the heart becomes surrounded by dense fibrous tissue scar tissue

46
Q

What is the most common type of neoplasm of the heart?

A

primary are very rare but secondary are much more common

- most common primary tumor= benign atrial myxoma - attached to the endocardium and projects out into the heart lumen

47
Q

What can benign atrial myxoma cause?

A

ball-valve obstruction, injury to the valve and embolisation

48
Q

What are the 3 main categories of congenital heart disease?

A

1) R-L shunts
2) L-R shunts
3) Obstructive

49
Q

What are R-L shunts?

A

reduce oxygenation of the blood causing cyanosis
can be due to tetralogy of fallot, transposition of great vessels, persistent trunks arterioles, tricuspid atresia and total anomalous pulmonary venous connection

50
Q

What are L-R shunt?

A

lead to pulmonary hypertension by increasing pulmonary blood flow
with time shunt reverses
can lead to eisenmenger’s syndrome and late cyanosis = due to atrial and ventricular septal defects or patent ductus arteriosus

51
Q

What is obstructive congenital heart defects?

A

block flow due to an abnormal narrowing of a chamber, valve or blood vessel
eg. coarctation of the aorta

52
Q

What is vasculitis?

A

inflammation of the wall of a blood vessel

  • can be caused by infection, immune complex formation, ANCA antibodies, T-cell mediated destruction, inflammatory bowel disease or neoplasms
  • different patterns of disease occur, affecting specific sizes of vessels as well as causing rashes and affecting the kidneys