Path slide set 4 Flashcards
3 causes of left to right shunts
- ASD
- VSD
- PDA
Genes for Tetrology of Fallot
JAG1 and NOTCH2
Valve doesn’t open completely, occurs chronically and impedes forward flow
Stenosis
age for dilated cardiomyopathy
usually between 20-50
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
with IV drug users, what side of the heart is often involved in infective endocarditis
right
Valve doesn’t close completes, may occur acutely or chronically. allows reversed flow
insufficiency
Gender and age for mitral annular calcification
Females more and >60
What cardiotoxic drugs/substances are associated with dilated cardiomyopathy?
- doxorubicin (chemotherapy)
- cobalt
- iron overload (repeated transfusions)
What time frame does Acute RF occur after grp A strep infection
10 days to 6 weeks with Anti-Streptolysin O
Apical ballooning of left ventricle with abnormal wall motion and contractile dysfunction
Takotsubo cardiomypathy
fish mouth stenosis
RHD
most common primary tumor of adult heart?
Myxomas
Most common genetic cause of congenial heart disease
Trisomy 21
What is the coarctation in relationship to the vessels that supply the upper body?
After
Bizarre Enlarged myocytes with a cytoplasm appearance of “spider cells”
Rhabdomyoma
Genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to reduced stroke volume and often ventricular outflow obstruction
Hypertrophic cardiomyopathy
Excess catecholamines following extreme emotional or psychological stress
Takotsubo cardiomyopathy
Most are asymptomatic but a miniority might have pain mimicking angina and dyspnea
mitral valve prolapse
SUBACUTE infective endocarditis vegetations may have what component
granulation tissue
the vegetations in infective endocarditis are mixtures of what?
fibrin, inflammatory cells (neutrophils) and organisms
Nonbacterial thrombotic endocarditis may be a source of what?
associated with what?
emboli
malignancies (especially mucinous adenocarcinomas)
Extracellular deposition of proteins which from an insoluble beta-pleated sheet
Amyloid
decrease in ventricular compliance leading to diastolic dysfunction
Restrictive cardiomyopathy
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
