Cardiac Path Robbins Part 3

Question 1

valvular heart disease- may present with?

Answer 1

• stenosis

- insufficiency

Question 2

valvular stenosis

Answer 2

• valve doesn’t open completely, occurs chronically
• impedes fwd flow
• chronic stenosis- may cause P overload hypertrophy- CHF

Question 3

valvular insufficiency

Answer 3

• valve doesn’t close completely, may occur acutely or chronically
• allows reversed flow
• chronic insufficiency- may cause volume overload hypertrophy- CHF

Question 4

most frequent causes of the major fxnal valvular lesions?

Answer 4

• aortic stenosis- calcification and sclerosis
• aortic insufficiency- dilation of asc aorta, often secondary to HTN/aging
• mitral stenosis- rheumatic heart disease
• mitral insufficiency- myxomatous degeneration (mitral valve prolapse)

Question 5

calcific valvular degeneration- types

Answer 5

• calcific aortic stenosis
• calcific stenosis of congenitally bicuspid aortic valve
• mitral annular calcification

Question 6

calcific aortic stenosis- occurs due to?

Answer 6

• most common valve abnormality!
• prevalence inc with age (70-80)
• “wear and tear” assoc with chronic HTN, hyperlipidemia, infl

Question 7

calcific aortic stenosis- affects? effects?

Answer 7

• bicuspid valves- accelerated course (50-60’s)
• affected valves contain osteoblast-like cells, which deposit an osteoid-like substance- ossifies
• calcifications in cusps- prevent complete opening of the valve
• pressure overload hypertrophy, CHF

Question 8

calcific aortic stenosis- clinical featurs

Answer 8

• left ventricular hypertrophy- becomes ischemic
• onset of symptoms (angina, CHF, syncope)- poor prognosis!!!
• die within 5 yrs of angina development (if untreated); 3 yrs of syncope; 2 yrs of CHF

Question 9

mitral annular calcification- what happens? occurs in who?

Answer 9

• calcific deposits in fibrous annulus
• normally doesn’t affect valve fxn
• nodules may become sites for thrombus formation of infective endocarditis
• F > M, >60 males
• mitral valve prolapse

Question 10

Calcific Stenosis of Congenitally Bicuspid Aortic Valve- caused by? clinical?

Answer 10

• Bicuspid aortic valve (BAV)- developmental abnormality
• chromosomes 18q, 5q, 13q!!
• NOTCH1 loss-of-fxn
• 2 fxnal cusps- the larger cusp as a midline raphe (where incomplete separation occurred)
• raphe- site of calcific deposits
• asymptomatic early in life
• late complications- aortic stenosis or regurgitation, infective endocarditis, aortic dilation

Question 11

mitral annular calcification

Answer 11

• calcific deposits develop in fibrous annulus!!
• doesnt affect valvular fxn
• site for thrombus formation- inc risk of embolic stroke
• most common- women, >60, pts with mitral valve prolapse

Question 12

mitral valve prolapse- what happens?

Answer 12

• valve leaflets prolapse back into LA during systole
• 2-3% adults in US; 7:1 female; usually incidental
• leaflets become thickened and rubbery, due to proteoglycan deposits (myxomatous degeneration) and elastic fiber disruption
• may occur as a complication of other causes of regurgitation (dilated hypertrophy)

Question 13

mitral valve prolapse- symptoms

Answer 13

• most are asymptomatic!!
• incidentally found- mid-systolic clicks!!
• a minority experience- pain mimicking angina, dyspnea

Question 14

mitral valve prolapse- complications

Answer 14

(rare)

• infective endocarditis
• mitral insufficiency
• thromboembolism
• arrhythmias

Question 15

only cause of mitral stenosis??

Answer 15

RHD (rheumatic heart disease)

Question 16

rheumatic fever-caused by?

Answer 16

• multisystem infl disorder following pharyngeal infection with group A streptococcus
• can cause chronic rheumatic heart disease

Question 17

rheumatic heart disease- pathogenesis

Answer 17

• immune response to streptococcal M proteins- cross reacts with cardiac self-antigens
• acute RF- 10 days-6 wks after grp A strep infection (anti-streptolysin O; anti-DNase B)

Question 18

rheumatic fever- can include?

Answer 18

• pancarditis
• migratory polyarthritis
• subcutaneous nodules
• rash
• Sydenham chorea

Question 19

cardiac features of acute RF

Answer 19

• pancarditis- Aschoff bodies! (lymphocytes, macrophages)
• infl and fibrinoid necrosis of endocardium and left-sided valves, with verrucae (vegetations)
• repeated streptococcal infections- cause these to recur
• LA enlargement- atrial fib/thrombosis; pulm congestion/RHF

Question 20

chronic Rheumatic heart disease

Answer 20

chronic Rheumatic heart disease -mitral leaflet thickening

• fusion and shortening of commissures
• fusion and thickening of tendinous cords
• results in mitral stenosis!!!
• “fish mouth” stenoses- calcification and fibrous bridging across valvular commissures

Question 21

infective endocarditis

Answer 21

-infection of valves and endocardium- characterized by vegetations consisting of microbes and debris, and tissue destruction

Question 22

acute infective endocarditis

Answer 22

• rapidly progressive, destructive infection
• previously normal valve
• Staph aureus!!
• requires surgery and antibiotics

Question 23

subacute infective endocarditis

Answer 23

• slower-progressing infection
• previously deformed valve (ex- chronic RHD)
• viridans streptococci!!
• can be cured with antibiotics alone

Question 24

nfective endocarditis- predisposing conditions

Answer 24

-valvular abnormalities- RHD
(used to be the major antecedent disorder! less common now), prosthetic valves, MV prolapse, calcific stenosis, bicuspid AV
-bacteremia- another site of infection, dental work, contaminated needle

Question 25

classic feature of IE

Answer 25

friable, bulky, destructive valvular vegetations!! - left-sided valves more commonly affected (right-sided valves involved in IV drug abusers) - friability- causes septic emboli - vegetations- fibrin, infl cells, organisms - subacute IE- granulation tissue

Question 26

infective endocarditis- clinical presentation

Answer 26

- nonspecific symptoms- fever, weight loss, fatigue | - murmurs- present with left-sided lesions (90%)

Question 27

infective endocarditis- complications

Answer 27

- GN - microthromboemoli (splinter/subungual hemorrhages) - erythematous/hemorrhagic nontender lesions on palms/soles (Janeway lesions) - painful subcutaneous nodules in pulp of digits (Osler nodes) - retinal hemorrhages (Roth spots)

Question 28

infective endocarditis- organisms involved?

Answer 28

- S. viridans (valve abnormalities)- 50% - S. aureus (normal/abnormal valves, IV drug abusers)- 20-30% - S. epidermis (prosthetic valves) - HACEK (Haemophilus, actinobacillus, cardiobacterium, eikenella, kingella)

Question 29

nonbacterial thrombotic endocarditis- assoc with?

Answer 29

(used to be called marantic endocarditis- malnutrition) - small, sterile thrombi on cardiac valve leaflets, along line of closure - may be a source of emboli - assoc with malignancies (mucinous adenocarcinomas), sepsis, catheter-induced endocardial trauma

Question 30

Endocarditis of Systemic Lupus Erythematosus

Answer 30

(Libman-Sacks Disease) - mitral and tricuspid valvulitis with small, sterile vegetations - use of steroids- dec the incidence of this complication! - lesions- small, single/multiple, sterile, pink vegetations with a warty (verrucous) appearance - vegetations- finely granular, fibrinous eosinophilic material - valvulitis- fibrinoid necrosis

Question 31

Carcinoid syndrome

Answer 31

-systemic disorder marked by flushing, diarrhea, dermatitis, bronchoconstriction that is caused by bioactive compounds such as serotonin released by carcinoid tumors!

Question 32

carcinoid heart disease- refers to?

Answer 32

- cardiac manifestations caused by bioactive compounds | - occurs in 1/2 of pts with the systemic carcinoid syndrome

Question 33

carcinoid heart disease- lesions

Answer 33

- dont occur until there's a massive hepatic metastatic burden (liver normally catabolizes circulating mediators) - endocardium and valves of right heart- primarily infected!!!- first cardiac tissues bathed by the mediators released by GI carcinoid tumors - left side of herat protected- pulm vascular bed degrades the mediators!

Question 34

carcinoid heart disease- pathogenesis

Answer 34

- mediators released by carcinoid tumors- serotonin, kallikrein, bradykinin, histamine, prostaglandins, tachykinins - serotonin and urinary excretion of serotonin metabolite (5-hydroxyindoleacetic acid)- correlate with severity of cardiac lesions - unknown how serotonin induces cardiac changes!

Question 35

carcinoid heart disease- morphology

Answer 35

- distinctive, glistening white intimal plaquelike thickening of endocardial surfaces of the cardiac chambers and valve leaflets - lesions- smooth m cells and sparse collagen fibers embedded in an acid mucopolysaccharide-rich matrix material - right-sided involvement- tricuspid insuff and pulm stenossis

Question 36

2 types of valvular prosthesis

Answer 36

- mechanical valves | - tissue valves (bioprostheses)

Question 37

60% of substitute valve recipients- develop what?

Answer 37

-prosthetic-related problem within 10 yrs of surgery

Question 38

complications of prosthetic valves

Answer 38

- thromboembolism- major consideration for mechanical valves!!! - structural deterioration- all bioprostheses become incompetent due to calcification and tearing!! - infective endocarditis - inadequate healing (paravalvular leak), exuberant healing (obstruction), hemolysis (due to high shear forces)