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Medical Pathology and Genetics
PPT 10: The Heart
133
Other
Graduate
02/23/2012

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Term
-Heart disease accounts for _____% of postnatal deaths in the US
-Atherosclerosis causes _____% of heart disease
-________ is an important complication of atherosclerosis
-HTN can occur independent of atherosclerosis and accounts for ______% of heart disease
Definition
40
80
HTN
9
Term
Heart action is critically dependent on a constant supply of ________ and _________
Definition
nutrients and oxygen
Term
_______ blood pressure, which depends primarily on the pumping of the heart, is regulated by __________ and _________/_________
Definition
arterial
hormones
biogenic amines
Term
THe large vol. of blood that passes through the heart makes it susceptible to what?
Definition
blood borne infections
Term
Immunoglobulins in the blood and circulating immune complexes may be deposited in the heart and cause ________ and ________
Definition
inflammation lesions
Term
________/_______ diseases often affect the heart and BVs, what is the most common?
Definition
systemic metabolic
DM
Term
What is the common endpoint of many cardiac diseases? How many are affected each year? How many fatalities?
Definition
CHF
5 million
300,000
Term
What is Systolic CHF?
Definition
Most CHF caused by deterioration of myocardial contractile function, resulting mainly from ischemic heart disease or HTN
Term
What is Diastolic CHF?
Definition
contraction is normal, but relaxation is abnormal; mainly older women with HTN or DM
Term
What is an example of something that causes valve failure?
Definition
endocarditis
Term
WHat is an abnormal load?
Definition
volume or pressure overload
Term
In CHF, how do our bodies respond: by activation of what (3)?
Definition
-Neurohumoral systems (NE, renin angiotensin, and ANP), frank-starling mech, hypertrophy
Term
What eventually happens in CHF, after the system responds.
Definition
myocytes degenerate
heart needs more oxygen
myocardium becomes vulnerable to ischemia
Term
In left sided heart failure, the left _______ fails and blood backs up into the _________
Definition
ventricle
lungs
Term
What are the 4 most common causes of left sided heart failure
Definition
-ischemic heart disease
-systemic hypertension
-mitral or aortic valve disease
-primary disease of the myocardium
Term
In left sided heart failure, the left ventricle is usually __________ and _____________
Definition
hypertrophied
dilated
Term
In left-sided heart failure, secondary enlargement of the left atrium with a-fib may lead to what 3 things?
Definition
-reduced stroke volume
-blood stasis
-thrombus formation
Term
In right sided heart failure the right _________ fails and blood backs up in the ___________
Definition
ventricle
body
Term
What is the most common cause of R sided heart failure?
Definition
-left sided heart failure: pressure increase in the pulmonary circulation produces increased burden on the R side of the heart
Term
Isolated R sided heart failure is less common, what are 3 common causes other than L sided heart failure?
Definition
-disease of the lung parenchyma a/o lung vasculature; cor pulmonale
-pulmonic or tricuspid valve disease
-congenital heart diseases with a left-to-right shunt
Term
In R sided heart failure the R ventricle and atrium become ___________ and ______________
Definition
hypertrophied
dilated
Term
Congenital heart disease (CHD) is due to faulty ___________, at week _____-_____
Definition
embryogenesis
weeks 3-8
Term
What are the three major groups of CHD?
Definition
-L to R shunts (most common; non-cyanotic)
-R to L shunts (cyanotic)
-Obstructions
Term
What is a shunt?
Definition
an abnormal communication between cardiac chamber or BVs, a shunt permits blood to flow between chambers or vessels that are normally isolated from each other
Term
What is a R to L cardiac shunt?
Definition
pulmonary circulation is bypassed and poorly oxygenated blood enters the systemic circulation causing blueness of the skin (cyanosis)
Term
What is a L to R cardiac shunt?
Definition
increases pulmonary blood flow, increasing pressure and volume of pulmonary circulation, causing R ventricular hypertrophy
Term
What is obstructive CHD?
Definition
decreased vascular flow caused by narrowing (stenosis) or complete blockage (atresia) of the heart chambers, valves, and major BVs
Term
What are more common, atrial sepatal defects (ASDs) or ventricular septal defects (VSDs)
Definition
ventricular septal defects
Term
ASDs are _____ likely than VSDs to close spontaneously, what does this mean when it comes to diagnosing them?
Definition
-less likely
-they are the most common CHDs to first be diagnosed in adults
Term
ASDs are ____ to _____ shunts
Definition
Left to right
Term
In ASDs, pulmonary vascular resistance may _______ , leading to a _______ to _______ shunt
Definition
increase (pulmonary HTN)
R to L shunt (Eisenmenger syndrome)
Term
What can be done about ASDs?
Definition
surgical repair prevents irreversible pulmonary changes and heart failure
Term
What is the most common CHD at birth
Definition
VSDs (42%)
Term
Most VSDs _______ spontaneously in childhood
Definition
close
Term
Which has a greater incidence in adults, VSDs or ASDs?
Definition
ASDs
Term
VSDs are a _______ to _______ shunt
Definition
left to right
Term
What percentage of VSDs occur with other cardiac malformations?
Definition
70%
Term
Small VSDs are __________
Definition
asymptomatic
Term
What are some possible consequences of large VSDs
Definition
-characterized by a severe L to R shunt which may lead to EIsenmenger syndrome, with cyanosis and CHF
-this would occur earlier and more often than with ASDs
Term
What is required for large VSDs?
Definition
surgical correction
Term
The ductus arteriosus permits blood to flow from pulmonary _________ to _________, bypassing _________ during fetal development
Definition
artery
aorta
lungs
Term
Ductus arteriosus closes spontaneously by day 1-2 of life, in 7% of CHD cases it fails to close, this is called?
Definition
Patent Ductus Arteriosus (PDA)
Term
What percentage of PDA are isolated?
Definition
90%
Term
How is a PDA audible?
Definition
a "machinery-like" murmur
Term
Small PDA is asymptomatic, what can a large PDA lead to?
Definition
L to R shunt, leading to Eisenmenger syndrome with cyanosis and CHF
Term
Tetralogy of Fallot is a ______ to _______ shunt
Definition
right to left
Term
What is the most common cause of cyanotic CHD?
Definition
tetralogy of fallot (R to L shunt) (5% of CHD)
Term
What are the four physiological features of tetralogy of Fallot?
Definition
-Large VSD
-Aorta overrides the VSD
-obstruction to RV outflow tract
-RV hypertrophy
-
Term
What are the 4 major signs and symptoms of tetralogy of fallot?
Definition
-cyanosis
-clubbing of the fingertips
-polycythemia
-paradoxical emboli (should lodge in the lungs, but bypasses lungs due to disorder)
Term
The clinical severity of the tetralogy of fallot depends not he degree of _________/_________/_______
Definition
pulmonary outflow obstruction
Term
What is the issue in transposition of the great arteries (TGA)?
Definition
the aorta arises from the R ventricle and the pulmonary artery arises from the L ventricle
Term
How are the atrium-ventricle connects in TGA?
Definition
normal
Term
What is the outcome of TGA?
Definition
separation of systemic and pulmonary circulations
Term
Is TGA compatible with life?
Definition
NO, unless there is a big VSD
Term
Even if there is a stable shunt, most pts with uncorrected TGA will die within the ______/_______/_______. What must happen for these pts to live?
Definition
first few months of life
correct surgery within the first few weeks of life
Term
What is the most important form of obstructive congenital heart disease?
Definition
coarctation (narrowing) of the aorta
Term
How often are males affected with coarctation of the aorta compared to females?
Definition
affected twice as often
Term
Preductile ("infantile") and postductal ("adult") coarctation of the aorta, which one is fatal?
Definition
-preductal: fatal without intervention usually
-postductal: usually asymptomatic
Term
Coarctation of the aorta often causes ________ and ______ blood pressure in the lower extremities
Definition
cyanosis
low
Term
What is a generic designation for a group of related syndromes resulting from myocardial ischemia?
Definition
Ischemic Heart Disease (IHD)
the cardiac blood supply cannot meet the myocardial oxygen demand
Term
What is IHD usually caused by?
Definition
decreased coronary artery blood flow (coronary artery disease)
Term
What is the leading cause of death in the US?
Definition
IHD
Term
What are the 4 basic clinical syndromes of IHD?
Definition
angina pectoris
Acute MI
chronic IHD
sudden cardiac death
Term
Define angina pectoris
Definition
intermittent chest pain caused by transient, reversible myocardial ischemia
Term
Define typical (stable) angina
Definition
-episodic pain on exertion
-narrowing (> or equal to 75%) of one or more coronary arteries
Term
Define prinzmental (variant) angina
Definition
paint at rest
coronary artery spasm of unknown etiology
Term
define unstable (crescendo) angina
Definition
increasing pain with less exertion, longer duration
plaque disruptions and thrombosis
Term
What is a myocardial infarction (MI)?
Definition
necrosis of the heart muscle resulting from ischemia
aka heart attack
Term
What are most MIs caused by?
Definition
acute coronary artery thrombosis
-sudden plaque disruption
-platelets adhere
-coagulation cascade activated
-thrombus occludes lumen within minutes
-irreversible injury/cell death in 20-40 minutes
Term
What is the therapeutic goal following an acute MI?
Definition
salvage ischemic tissue by reperfusion
Term
What are three ways reperfusion is achieved?
Definition
-thrombolysis -balloon angioplasty -coronary artery bypass graft
Term
What are two ways that reperfusion can lead to greater local injury than if no treatment was performed?
Definition
oxygen free radicals
microvascular injury and hemorrhage
Term
What is the number one symptom of an MI in most cases?
Definition
severe, crushing chest pain with or w/o radiation
typically lasts 20 min to several hours
not significantly relieved by nitroglycerin or rest
Term
What is the pulse generally like in an MI?
Definition
rapid and weak
Term
What can develop following a massive MI?
Definition
cardiogenic shock
(>40% of the LV affected)
Term
in what percentage of MI's are there no symptoms
Definition
10-15%
Term
What causes the Q wave abnormalities following an MI?
Definition
transmural infarcts
Term
What causes the ST-segment abnormalities and T-wave inversion following an MI?
Definition
abnormalities in myocardial repolarization
Term
What causes the arrhythmias seen following an MI?
Definition
electrical abnormalities of ischemic myocardium and conductance system
Term
What accounts for most deaths from MI that occur before hospitalization?
Definition
SCD (sudden cardiac death) due to lethal arrhythmias
Term
What is the laboratory evaluation of MI based on measuring?
Definition
cardaic troponins T and I, which are released from the cardiac muscle into the blood following an MI
Term
Troponins increase within what time period following an MI? How long do they remain elevated?
Definition
2-4 hours
a week or two
Term
What is the second best lab test to run following an MI?
Definition
total creatine kinase (CK) and a myocardial specific isoform (CK-MB)
Term
CKMB increases within how long after an MI? When does it return to normal?
Definition
2-4 hours
72 hours
Term
What does the prognosis of an MI depend on?
Definition
depends on the infarct size, site, and thickness of the heart wall damage
Term
What is the overall 1 year mortality for MI?
Definition
30%
3-4% mortality per year thereafter
Term
What is another name for chronic ischemic heart disease?
Definition
ischemic cardiomyopathy
Term
What is chronic ischemic heart disease?
Definition
-progressive heart failure from ischemic myocardial damage , usually there is a history of MI
-Enlarged heart from LV dilation and hypertrophy of remaining viable myocardium
Term
What account for many of the deaths due to chronic ischemic heart disease?
Definition
arrhythmias and MIs
Term
Define sudden cardiac death (SCD)
Definition
unexpected death from cardiac causes without symptoms (or within 24 hr of symptom onset)
Term
What does SCD result from?
Definition
fatal arrhythmia
Term
What is the most common cause of SCD
Definition
coronary atherosclerosis
Term
What is the most common cause of SCD in young victims?
Definition
Long-QT (LQT) syndrome caused by mutations in various cardiac ion channel genes
Term
What is hypertensive heart disease?
Definition
the heart responds to increased pressure or volume overload with myocyte hypertrophy
-can effect either left or right ventricle
Term
What is cor pulmonale?
Definition
R ventricle is enlarged due to pulmonary HTN caused by primary lung disorders
Term
What does pressure overload cause? Volume overload?
Definition
ventricular wall thickness
ventricular wall dilation
Term
What are the reasons for heart failure in HTN?
Definition
they are poorly understood
Term
What causes valvular heart disease?
Definition
stenosis (failure to open completely) and/or regurgitation (insufficiency- failure to close completely)
Term
In valvular heart disease, abnormal blood flow through the diseased valves produces _______
Definition
murmurs
Term
Stenosis of what valves accounts for 2/3 of all valve disease?
Definition
aortic and mitral valves
Term
What is the most common cause of aortic stenosis in the US?
Definition
calcific aortic stenosis
Term
Who does calcific aortic stenosis occur in?
Definition
-normal part of aging process in 70-80 yo's, OR congenitally bicuspid aortic valve (40-50 yo's)
Term
What does calcific aortic stenosis result in?
Definition
increased LV pressure
LV hypertrophy
relative ischemia
Term
What are the symptoms of calcific aortic stenosis? prognosis?
Definition
-symptoms of angina, CHF, or fainting
-50% mortality within 2 years without surgery
Term
Describe mitral valve prolapse?
Definition
the mitral leaflets are enlarged, rubbery, floppy, and prolapse back into the left atrium during systole. There is a deposition of myxomatous (mucoid) material within the leaflet
Term
How common is mitral valve prolapse?
Definition
common, 3-5% of adults in US affected
-women 7x more than men
Term
Auscultation of someone with mitral valve prolapse would reveal what?
Definition
midsystolic click
Term
What are the symptoms of mitral valve prolapse? people with this have an increased risk of what?
Definition
-most people are asymptomatic
-3% may have CHF if the chord or valve leaflets rupture
-increased risk for infective endocarditis and sudden death from ventricular arrhythmias
Term
What is rheumatic fever? What does it cause?
Definition
systemic inflammatory disease occurring a few weeks after streptococcal pharyngitis (strep throat). causes acute rheumatic heart disease (RHD)
Term
What does RHD cause?
Definition
valve deformities, especially scarring and stenosis of the mitral valve, followed by damage to both mitral valve and aortic valve
Term
What is infective endocarditis?
Definition
a bacterial invasion of heart valves and endocardium that destroys heart tissue (large, friable vegetations)
Term
What is acute endocarditis?
Definition
highly virulent pathogen attacks normal valve
half of patients dead within days to weeks
Term
What is subacute endocarditis?
Definition
low virulence pathogen colonized abnormal valve
slow onset, long course, most recover
Term
What are the symptoms and complications of infective endocarditis?
Definition
-fever and flu like symptoms
-septicemia, arrhythmias, renal failure, systemic emboli
Term
What are cardiomyopathies?
Definition
a diverse group of disorders in which there is intrinsic myocardial dysfunction
Term
What are the three classifications of cardiomyopathies?
Definition
Dilated cardiomyopathy (90%)
hypertrophic cardiomyopathy
restrictive cardiomyopathy
Term
What happens in a dilated cardiomyopathy?
Definition
heart dilates, enlarges, ineffective contraction
(90% of cardiomyopathies)
Term
What are 4 causes of dilated cardiomyopathy?
Definition
-viral
-alcohol/toxin
-peripartum
-25-35% caused by genetic mutations in genes that encode cytoskeletal proteins
Term
What is the prognosis of dilated cardiomyopathy?
Definition
slowly progressing CHF and 50% of patients are dead within 2 years
Term
What happens in hypertrophic cardiomyopathy (HCM)?
Definition
myocardial hypertrophy where the myocardium does not relax, so there is abnormal diastolic filling
-ventricle is thick-walled, heavy, and hyper contracting, but has reduced SV
Term
What causes most cases of HCM?
Definition
missense mutations in at least 12 genes that encode the sarcomeric proteins that form the contractile apparatus of striated muscle
Term
What does HCM cause? What are the symptoms? What are the treatments?
Definition
-reduced CO and secondary increase in pulmonary venous pressure
-exertional dyspnea, atrial fibrillation, CHF, arrhythmia, sudden death
-drugs to promote ventricular relaxation or surgical excision of part of the septum
Term
What is restrictive cardiomyopathy?
Definition
ventricle wall is stiffer, impairing filling during diastole
Term
WHat is the cause of restrictive cardiomyopathy?
Definition
idiopathic or secondary to systemic disease (amyloidosis, hemochromatosis, sarcoidosis)
Term
What are the symptoms of restrictive cardiomyopathy?
Definition
cough, shortness of breath, fatigue, peripheral edema
Term
What is the treatment and prognosis for restrictive cardiomyopathy?
Definition
-not often helpful, may be candidates for a heart transplant
-70% of patients die within 5 years
Term
What is pericarditis caused by?
Definition
Primary- very rare-mainly infection by viruses
Secondary, usual- acute MI, cardiac surgery, irradiation, pneumonia, uremia
Term
What are the symptoms of pericarditis?
Definition
atypical chest pain, not related to exertion, often worse when reclining
Term
What is pericarditis?
Definition
a prominent friciton rub that can cause cardiac tamponade
Term
What are the three different types of pericardial effusions and their causes?
Definition
-serous- CHF, hypoalbuminemia
-serosanguinous- blunt chest trauma, malignancy, ruptured MI, aortic dissection
-chylous- mediastinal lymphatic obstruction
Term
What is the outcome of pericardial effusions?
Definition
depends on the ability of the pericardial sac to stretch, the amount of fluid accumulated, and speed of accumulation
-slow=asymptomatic
-sudden=fatal cardiac tamponade
Term
What are the most common tumors of the heart?
Definition
-in general they are rare
-metastatic tumor from another tissue (lung, lymphoma, breast)
Term
What is the most common primary tumor of the heart?
Definition
myxoma, occurs usually in the L atrium
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