Path slide set 4 Flashcards by Garrett Gilbert

3 causes of left to right shunts

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Genes for Tetrology of Fallot

JAG1 and NOTCH2

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Valve doesn’t open completely, occurs chronically and impedes forward flow

Stenosis

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age for dilated cardiomyopathy

usually between 20-50

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What congenital defect can cause shunting that causes volume overload on the right side leading to

pulmonary hypertension
right heart failure
Paradoxical embolization

Atrial septal defect

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with IV drug users, what side of the heart is often involved in infective endocarditis

right

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Valve doesn’t close completes, may occur acutely or chronically. allows reversed flow

insufficiency

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Gender and age for mitral annular calcification

Females more and >60

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What cardiotoxic drugs/substances are associated with dilated cardiomyopathy?

doxorubicin (chemotherapy)
cobalt
iron overload (repeated transfusions)

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What time frame does Acute RF occur after grp A strep infection

10 days to 6 weeks with Anti-Streptolysin O

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Apical ballooning of left ventricle with abnormal wall motion and contractile dysfunction

Takotsubo cardiomypathy

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fish mouth stenosis

RHD

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most common primary tumor of adult heart?

Myxomas

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Most common genetic cause of congenial heart disease

Trisomy 21

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What is the coarctation in relationship to the vessels that supply the upper body?

After

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Bizarre Enlarged myocytes with a cytoplasm appearance of “spider cells”

Rhabdomyoma

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Genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to reduced stroke volume and often ventricular outflow obstruction

Hypertrophic cardiomyopathy

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Excess catecholamines following extreme emotional or psychological stress

Takotsubo cardiomyopathy

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Most are asymptomatic but a miniority might have pain mimicking angina and dyspnea

mitral valve prolapse

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SUBACUTE infective endocarditis vegetations may have what component

granulation tissue

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the vegetations in infective endocarditis are mixtures of what?

fibrin, inflammatory cells (neutrophils) and organisms

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Nonbacterial thrombotic endocarditis may be a source of what?

associated with what?

emboli

malignancies (especially mucinous adenocarcinomas)

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Extracellular deposition of proteins which from an insoluble beta-pleated sheet

Amyloid

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decrease in ventricular compliance leading to diastolic dysfunction

Restrictive cardiomyopathy

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What valvular problem comes about from mitral annular calcification?

mitral valve prolapse

what type of hypertrophy does Calcific aortic stenosis cause?

pressure overload hypertrophy

What do affected valves with calcific aortic stenosis contain?

osteoblast-like cells, which deposit an osteoid-like substance-->ossifies

This is generally seen with a PDA if found in infancy and without a PDA in adults form

Coarctation of the Aorta

Mutations for Myxomas

- GNAS (McCune-Albright syndrome) | - PRKAR1A (null mutation in Carney complex)

immune response to streptococcal M protein cross reacts with cardiac self antigens

Pathogeneis of Rheumatic heart disease

Collapse of young athlete and sudden death

Hypertrophic cardiomyopathy

What organism causes infective endocarditis in really acute setting or with IV drug user

S. aureus

if you have mitral valve stenosis what is most likely the cause?

Rheumatic heart disease

Where do calcific deposits occur in Mitral annular calcification?

the fibrous annulus - NOT leaflets like in aortic - doesn't affect valve function

what organism causes infective endocarditis in someone with a prosthetic valve

S. epidermidis

a slower progressing infection of a PREVIOUSLY deformed valve (such as in chronic RHD)

SUBACUTE infective endocarditis

What valve abnormality shows accelerated course for calcific aortic stenosis?

bicuspid valve

Progressive cardiac dilation and systolic dysfunction, usually with dilated hypertrophy

dilated cardiomyopathy

Binucleated macrophages and T cells (Aschoff Bodies)

Rheumatic Heart Disease

What is the most common valve abnormality

calcific aortic stenosis

What type of morphology is seen with dilated cardiomyopathy

interstitial fibrosis

Right ventricle hypertrophies and left atrophied

Transposition of Great vessels

Describe the size of the chambers in restrictive cardiomyopathy

Ventricles are usually of normal size, but both atria can be enlarged

describe the immune response in rheumatic heart disease

both humoral and T cell

What's the most common mutation involved in hypertrophic cardiomyopathy

B-myosin heavy chain | -usually involving sarcomeric protein

What has a STONG association with dilated cardiomyopathy

alcohol abuse

Gene for Bicuspid Aortic valve

NOTCH1

gender preference for mitral valve prolapse

7:1 f:m

Inflammation of the MYOCARDIUM is most commonly due to what?

Viruses (coxsackie A and B)

Familial, usually autosomal dominant Usually gene involved with desmosome and cardiomyocyte connections - intercalated disc

Arrhythmogenic right ventricular cardiomyopathy

rapidly progressing, destructive infection of a previously normal valve

ACUTE infective endocarditis (Staph Aureus)

The clinical severity of Tetrology of Fallot depends on what?

Degree of subpulmonary stenosis

"broken heart syndrome"

Takotsubo cardiomyopathy

Chronic stenosis may cause what type of hypertrophy? Chronic insufficiency?

pressure overload volume overload

What organisms causes infective endocarditis if there is a preexisting valve abnormality

S. viridans

what therapy is needed for acute endocarditis

SURGERY and antibiotics

What happened to the left atrium in Rheumatic heart disease?

LA enlargement -->Afib/thrombosis, pulmonary congestion/RHF

Harsh, machinery-like murmur

PDA

multisystem inflammatory disorder following pharyngeal infection with group A streptococcus

Rheumatic fever

Friable, bulky, destructive valvular vegetations

Infective endocarditis

therapy needed for subacute endocarditis

antibiotics alone

inflammation and fibrinoid necrosis of endocardium and left-sided valves with verucae (vegetations)

Acute RF

Coarctation with PDA manifests at birth as what?

Cyanosis in lower half of body

What increases prevalence of calcific aortic stenosis

- Age (usually at 60-80) | - also same risk factors of wear and tear as atherosclerosis (HTN, hyperlipidemia, inflammation)

Septal hypertrophy and myocytes disarray

Hypertrophic cardiomyopathy

What may be secondary to deposition of material within the wall (amyloid) or increased fibrosis from radiation?

Restrictive cardiomyopathy

Describe the calcifications in calcific aortic stenosis?

mounded calcification in cusps that prevent complete opening of valve

- Progressive CHF --> dyspnea, exertional fatigue, lower EF - Arrhythmias - embolism

Dilated cardiomyopathy

Morphology for myocarditis

LYMPHOCYTIC infiltrate

Only an enlarged Atrium. Ventricle is stiff and can't expand. backup of blood into atrium may lead to Afib and mural thrombosis

Restrictive cardiomyopathy

- Right ventricular failure and arrhythmias - Myocardium of the right ventricular wall replaced by adipose and fibrosis - causes ventricular tachycardia and fibrillation, sudden death

Arrhythmogenic right ventricular cardiomyopathy

The friability of infective endocarditis leads to what?

septic emboli

4 cardinal features of Tetrology of Fallot

- VSD - Obstruction of RV outflow tract - Aorta overrides the VSD - RV hypertrophy

hypervascularity of valves

RHD

what side valves are more commonly affected with infective endocarditis

left

Pathogenesis of Dilated cardiomyopathy

- 30-50% thought to be familial (TTN gene: Titin protein | - autosomal dominan

Small, sterile thrombi on cardiac valve leaflets, along line of closure

Nonbacterial thrombotic endocarditis

what do patients usually present with when they have infective endocarditis?

- Nonspecific symptoms (fever, weight loss, fatigue) | - Murmurs usually present with left-sided lesions

Enlarged and "boot shaped" heart due to right ventricular hypertrophy

Tetralogy of Fallot

symptoms and signs similar to acute myocardial infarction. ECG changes and troponin elevated but not as much as would be in MI

Takotsubo

When a left to right shunt produces pulmonary pressure that eventually becomes a right to left shunt and introduces deoxygenated blood into systemic circulation

Eisenmenger syndrome

splinter hemorrhages on fingernails and painful nodules on digits

infective endocarditis

Which coarctation causes concentric hypertrophy?

Without PDA

What gives you a systemic Amyloid?

Myeloma

What give you amyloid restricted to the heart

Transthyretin

Most common primary tumor of pediatric heart

Rhabdomyoma

describe the leaflets in mitral valve prolapse

thickened and rubbery due to proteoglycan deposits (myxomatous degeneration) and elastic fiber disruption

What is the most common form of congenital heart disease

VSD

Nodules in mitral annular calcification may become sites for what?

thrombus formation and infective endocarditis

epidemiology for takotsubo cardiomyopathy

>90% women. ages 58-75

Pancarditis featuring Aschoff bodies

Acute RF

What stains green with a conga red stain

Amyloid

Coarctation without PDA is usually asymptomatic but when it does show symptoms what are they

- HTN in upper extremities - hypotension in lower extremities - Claudication and cold lower extremities with exercise

mid systolic click

mitral valve prolapse

Predisposing conditions for infective endocarditis

Valvular abnormalities | Bacteremia (another site of infection, DENTAL work/surgery, CONTAMINATED NEEDLE, compromised epithelium)

mitral leaflet thickeing, fusion and shortening of commisures, fusion and thicking of tendinous cords resulting in mitral stenosis

CHRONIC RHD

- Globular heart (dilation of all chambers) - Mural thombi are common - functional regurgitation of valves

Dilated cardiomyopathy