9. Heart 2 Flashcards
Arrhythmia from atrium = supraventricular
What is choatic depolarization without functional ventricular contraction?
Ventricular fibirillation
What is the most common cause of arrhythmias?
Ischemic injury
What occurs when the SA node is damaged leading to bradycardia?
Sick sinus syndrome
What occurs when myocytes depolarize independently and sporadically (atrial dilation) with variable transmission through AV node w/ irregular irregular HR?
Atrial fibrillation
When the AV node is dysfunctional, it is known as heart block
First degree: prolonged PR interval
Second degree:
Third degree: ?
Second: intermittent transmission
Third Degree heart block: complete failure
The following can be due to what?
Ischemic heart dz, dilated cardiomyopathy, myocyte hypertrophy, inflammation (myocarditis) and amyloid…
Gap junction abnormalities
Most hereditary heart conditions are autosomal dominant. What are mutations in genes that are required for normal ion channel function? (sometimes assoc w skeletal muscle DOs and diabetes)
Channelopathies
What is the most common arrythmogenic syndrome manifests as arryhthmias associated with excessive prolongation of the cardiac repolarization, presenting with stress induced syncope, SCD, assoc w swimming?
Long QT syndrome
What syndrome presents with patients having arrhythmias associated with abbreviated repolarization intervals, have palpitations, syncope and SCD?
Short QT syndrome
What syndrome manifests as ECG abnormalities (St segment elevations and right bundle branch block) in the absence of a heart DO?
Brugada Syndrome
Sudden cardiac death SCD is unexpected deeath from cardiac cause, either without symtpoms or within 1-24 hours of symptom onset. 80-90% of successively resuscitated patients have no?
lab or ECG changes
What is the most common underlying etiology, causing 80-90% of SCD?
Coronary artery disease CAD
Pt usually have >75% stenosis of 1+ of the coronary As, prior MIs are seen in 40% of the cases… WHat is the first manifestation of Ischemic heart dz? IHD
SCD
SCD is typically due to what, which is most frequently a consequence of CAD/ ischemia induce myocardial irritability?
fatal arryhtmia/ ventricular fibrillation
Systemic left sided hypertensive heart disease (HHD) occurs when?
there is a pressure overload resulting in left ventricular hypertrophy (LVH)
In left sided HHD, the LV wall is concentrically (septum and wall same) thickened (>1.5cm, weight >500gm). The earliest morphologic change of system HHD is?
that myocytes show and increase tranverse diameter
What dysnfunction can result in left atrial enlargement leading to atrial fibirillation?
diastolic dysfunction
Systemic or left sided HHD may lead to CHF and is a risk factor for?
SCD
Pulmonary right sided HHD is isolated right sided HHD that arises in the setting of?
pulmonary hypertension
Acute cor pulmonale may arise from a large pulmonary embolus, what can be seen in the heart in this case?
marked dilation of RV without hypertrophy (if chronic cor pulomonale will have hypetrophy)
What is the most common cause of pulmonary hypertension?
Left sided heart disease
What are two ways one can tell the difference grossly between left sided and right sided hypertensive heart disease?
Right sided wall thickened and will be the new apex of the heart in some cases
Left sided wall thickened concentrically
PAthologic changes of cadiac valves are largely of 3 types…
1) damage to collagen weakens leaflets (MVP)
2) Nodular calcification beginning in interstitial cells (calcific aortic stenosis)
3)
Fibrotic thickening as seen in rheumatic heart dz (mitral stenosis)
Because cardiac valves are thin enough to be nourished by diffusion from the blood, normal leaflets and cusps have only scant blood vessels limited to?
the proximal portion of the valve
When does valvular disease come to clinical attention? 2
due to stenosis and insufficiency/ regurgiation / incompetence
What is used to describe the incompetence of a valve stemming from an abnormality in one of its support structures as opposed to a primary valve defect?
function (mitral valve) regurgitation
*common in IHD and dilated cardiomyopathy
Stenosis is when a valve doesnt open completely occuring chronically, impeding forward flow, sometimes resulting in?
pressure overload hypertrophy (CHF)
Insufficiency is when a valve doesnt close completely, occuring acutely and chronically, allowing reversed flow or regurgitation, chornically causing?
volume overload hypertrophy and CHF
What is the only cause of mitral stenosis?
Posinflammatory scarring due to rheumatic heart disease***
What are two causes of aortic valve disease?
postinflammatory scarring d/t rheumatic heart dz
Calcification of congenitally deformed valve
What are two causes of aortic regurgitation?
Rheumatic heart dz
Aortic insufficiency: dilation of the ascending aorta secondary to HTN/aging
Syphilitic aortitis and marfan syndrome cause what valvle disease?
Aortic valve disease
Abnormalities of tensor apparatus and of the left ventricle and or annulus can lead to?
mitral valve disease
What are the two MCC of mitral regurgitation?
Abnormalities of leaflets and commissures
Mitral valve prolapse (myxomatous degen)
Calcific aortic stenosis is the most common of all valvular problems whose prevalence increases with age (60-80) and is caused by wear and tear associated with what? 3
chronic HTN
hyperlipidemia
inflammation
Calcific aortic stenosis has a HUGE association with what, which shows an accelerated course due to mechanical stress?
Bicuspid arotic valve (BAV)
If someone has a bicuspid aortic valve, there will be clinical sx 1-2 decades earlier.. Affected valves contain osteoblast like cells which deposit?
an osteoid-like substance and ossifies
**no commisural fusion (as seen in rheumatic and congential aortic stenosis)
Calcific aortic stenosis has mounded calcification in cusps which prevent complete opening of the valve. What are 3 common sx?
angina CHF and syncope
LVH develops due to increase pressure of the valve not opening, most pts die in 5 years of devloping angina, in 3 of developing syncope and 2 in developing?
CHF
*tx with surgical replacement
Biscuspid valves are prone to calcification. Patients can remain asymptomatic until stenosis reaches a critical point when?
CHF ensues.
In mitral annular calcification calcific deposits occurs in the?
fibrous annulus-at the base of the leaflets
annular calcification normally does not affect valve function however there are expections including regurg, stenosis and?
**arrhythmias and occasionally sudden death by penetration of calcium deposits to a depth sufficient to impinge on the atrioventricular conduction system
patients with mitral annular calcification are at an increased risk for thrombus formation or infective endocarditis.. Where is this most commonly seen? 3
Females more than Males
greater than 60 y/o
WITH mitral valve prolapse
Myxomatous degeneration of the mitral valve, OR mitral valve prolapse MVP is where the valve leaflets prolapse BACK into the left atrium during systole. Affects 2-3% of adults affecting M/F more? and what type of murmur?
7Females to 1 Male
*mid systolic click
Marfan syndrome (FBN1) causes loss of CT support in the MV leaflets making them floppy. The leaflets in MVP become thickened and rubbery due to?
proteoglycan deposits (myxomatous degeneration) in spongiosa layer and elastic fiber disruption
What is the characteristic anatomic change in MVP?
interchordal ballooning of the leaflets
Most MVPs are asymptomatic but a minority may experience what two sx?
chest pain mimicking angina
dyspnea
MVP has rare complications including infective endocarditis, mitral insufficiency, thromboembolism and?
arrhythmias=SCD
*tx with valve replacement
Rheumatic fever is a multisystem inflammatory disorder following pharyngeal infection with?
group A streptococcus
Incidence of rheumatic fever has decreased with more rapid dx and tx of strep. Acute rheumatic fever may include carditis component and overtime may evolve into?
chronic rheumatic heart disease
The pathogenesis of rheumatic fever include immune response to streptococcall M proteins that cross react with?
cardiac self antigens (among others)
Acute RF occurs 10 day to 6 weeks after group a strep infection and can be determined by what two titers?
Anti-streptolysin O
Anti-DNAse B
acute RF symptoms include pancarditis, migratory polyarthritis, subcutaneous nodules and erythema marginatum (rash). It also involves a neuro DO with involuntary rapid purposeless movements known as?
Sydenham chorea
Cardiac features of RF include pancarditis (inflamm of entire heart) with Aschoff bodies contain T cells, plasma cells and plump activated macrophages known as?
Anitschkow cells ***** pathognomonic for RF
central wavy ribbon of chromatin- looks like catepillar
Inflammation and fibrinoid necrosis of endocardium on left sided valves seen with what?*
verrucae (vegetations) is seen in RF.
What causes mitral leaflet thickening, fusion of commissures with shortenin, fusion and thickening of cords?
Chronic Rhematic Heart disease
What is the ONLY cause of mitral stenosis?
chronic RHD
RHD causes left atrial enlargement leading to Afib and thromembolic events. It also causes right heart failure leading to?
right ventricular hypertrophy
*infective endocarditis as well
What is marked by a form of granulomatous inflammation “aschoff nodules’ centered around vessels?
acute rheumatic myocarditis
Where can one find maccallum plaques as seen in the heart in RHD?
Left atrium
Neovascularization is seen in RHD as well as what? which occurs due to calcification and fibrous bridging across the valvular commissures
Fish mouth or button hole stenosis
what is an infection of valves or endocardium, characterized by vegetations consisting of microbes and debris associated with underlying tissue destruction?
Infective endocarditis (IE) (acute and subacute)
What type of infectious endocarditis is a RAPIDLY progessing destructive infection of a previously normal valve?
ACUTE Infective endocarditis
*requries surgery and abx
What type of infectious endocarditis is a slower-progressing infection of a previously DEFORMED valve (such as in chronic RHD)
SUBACTE infective endocarditis
*tx abx alone
Predisposing conditions to IE including valvular abnormalities (RHD, prosthetic valves, MV prolapse) and bacteremia - including?4
***dental work/surgery
site infection
contaminated needle (IVDU)
Compromised epithelium
Classic features of IE are friable, bulky, destructive valvular vegetations with what side valves being more common?
Left sided valves EXCEPT in IV drug users- right side more common
The friability of the vegetations lead to septic emboli or what if they are on the right side valve?
pulmonary embolism
The vegetations are mixtures of fibrin, inflammatory cells and organism, note subacte veges may have?
granulation tissue component
Patients with IE present with fever weightloss fatigue and flu-like symptoms. What are the sx for acute endocarditis?
fever, chills, weakness, lassitude
What organism affect previouslt damaged or abnormal valves *dental organisms?
Strep Viridans- subacute-not as bad
What organism is highly virulent and seen in IV drug users and normal valves?
Staph Aureus ***acute-bad
What organism is commonly infecting prothestic valves?
S. Epidermidis
What is the significance of the following? haemophilus actinobacillus cardiobacterium kingella eikenalla
HACEK organisms = commensal bacteria in the oral cavity
What are the different forms of vegetative endocarditis (warty lesions) in the following? Rheumatic Heart disease Infective endocarditis Nonbacterial thrombotic Endocarditis Libman-Sacks disease
Rheumatic Heart disease: warty line along line of closure
Infective endocarditis: large and irregular masses on the valve cusps
Nonbacterial thrombotic Endocarditis: small, bland vegetations along line of clousre
Libman-Sacks disease: small/medium sized on either or BOTH sides of the valve leaflet
What is present in 90% of patients with left sided infective endocarditis?
Heart murmur
acute IE can cause mortality in less than 6 weeks (50%) and necrotizing ulcerative lesions. Its emboli can cause mycotic aneurysms and what is an outcome grossly?
RING ABSCESS : vegetation erode into underlying myocardium
The duke criteria is used for dianosing infective endocarditis which include?
system of labs, clinical, echo and cultures that identify IE
What are 4 COMMON important signs of infective endocarditis?
subungal splinter hemorrhages (d/t emobli) janeway lesions (red lesions hands/feet) osler nodes (subq painful nodules hands/feet) roth spots (retinal hemorrhaging)
What is characterized by small, STERILE thrombi on cardiac valve leaflets along the line of closure; loosely attached, not invasive, do NOT illicit inflam reaction?
nonbacterial thrombotic endocarditis (NBTE)
NBTE may be a source of emboli (produces infarcts in brain/heart). What is the IMP* malignancy it is associated with? along with spesis or catheter induced endocardial trauma
MUCINOUS ADENOCARCINOMAS
nonbacterial thrombotic endocarditis occurs on previously normal valves due to?
a hypercoaguable state
Libman sacks endocarditis is due to SLE, mitral valve is more commonly involved causing regurgitation. The cause is due to antiphospholipid syndrome associated with lupus
Not on the test
What is a systemic disorder marked by flushing, diarrhea, dermatitis and bronchoconstriction?
Carcinoid syndrome (eg caused by serotonin released from GI carcinoid tumor) - paraneoplastic syndrome!
Severity of the cardiac carcinoid symdrome lesions correlate with plasma levels of serotonin and urinary excretion of the serotonin metabolite..?
5-hydroxindoleacetic acid
What occurs when 50% of patients with carcinoid syndrome develop cardiac manifestations?
Carcinoid Heart disease
Carcinoid heart disease does not occur until there is a massive hepatic burden or damage since the liver normally does what?
catabolizes circulating mediatiors before they effect the heart
What heart valves are affects in carcinoid heart disease and why?
The right heart valves (and endocardium) since they are first in contact with the mediators, left side valves are protected due to pulmonary vascular bed degredation of mediators
The valvular plaques seen are similar to those in patients taking fenfluramine (appetite suppresant) or ergot alkaloids for migraines - both affect systemic serotonin metabolism. Why serotonin?
it has not been proven why this occurs or if serotonin inhibitors can fix the problem
carcinoid heart disease associated with carcinoid syndrome has intimal thickening (acid mucopolysaccharide-rich) and what other characteristic finding?
glistening white intimal plaque like thickenings of endocardial surfaces of the cardiac chambers and valve leaflet
What are the two types of valvular prosthese to replace damaged cardiac valves?
mechanical valves (nonphysologic material) tissue valves (porcine/bovine)
60% of valve recipients have serious problems within 10 years. What is a specific complication to mechanical valves?
thromboembolism due to the disruption of laminar flow (long time anticoags)
What is a specific complication to tissue valve replacements?
structural deterioration which is the most common for bioprosthetic valves (calcification and tear)
Cardiomyopathy means heart muscle disease. What are the three types of cardiomyopathies?
Dilated (MC-90%)
Hypertrophic
Restrictive
What is charcterized by progression cardiac dilation and systolic dysfunction, usually with dilated hypertrophy?
Dilated Cardiomyopathy DCM - most common
DCM is thought to be familial in 30-50% of cases (TTN titin mutations may account for 20%- AD). What is strongly linked to DCM? 2
Alcohol
Myocarditis (esp. prior evidence of Coxsackie B Virus)
Cardiotoxic drugs and substances are also known to cause DCM including doxorubicin (chemo), cobalt, and iron overload. How does iron do this?
Iron overload from hereditary hemochromatosis (HFE mutation) or multiple transfusions
What stain is used to see iron in heart cells?
Prussian blue iron stain
DCM causes a large, heavy flabby heart due to dilation of all four chambers, What is common? 2
**mural thrombi and embolism = stroke
Also, functional regurgitation of valves
DCM usually manifests between ages 20-50yo causing progressive CHF with what sx? 3
dyspnea
exertional fatigue
dec. ejection fraction by 25%(end stage)
What are two important consequences/ outcomes of dialted cardiomyopathy? 2
arrhythmias (sudden death)
embolism
Another type of dilated cardiomyopathy which is caused by excess catecholamines from extreme emotional or physiological stress?
Takotsubo cardiomyopathy OR
broken heart syndrome
Broken heart syndrome occurs in mainly women aged 58-75. Symptoms are like acut myocardial infarction. What can be seen grossly?
apical ballooning of the left ventricle with abnormal wall motion and contractile dysfunction (octopus trap like)
Histologically in DCM, there is hypertrophied muscle cells with nelard nuclei but some are stretched and attenuated, along with a variable degree of?
interstitial and endocardial fibrosis
What is the main difference between start and end point of DCM and HCM?
DCM only has 20-50% genetic causes (TITIN) while HCM is caused 100% by genetic mutations in sarcomere patients. Also in HCM, you will see myofiber disarray.
What is an inherited disease of myocardium causing right ventricular failure and rhythym distrubances (ventricular tachycardia/ fibrillation) with SUDDEN DEATH?
arrhythmogenic right ventricular cardiomyopathy ARVC
in ARVC, myocardium of the right ventricular wall is replaced by adipose and fibrosis no muscle left. How do you get this disease?
familial, autosomal dominant - defective cell adhesion proteins in the desmosomes that link adjacent cardiac myocytes
What syndrome is ARVC with hyperkeratosis of plantar and palmar skin surfaces?
Naxos Syndrome
Naxos syndromes is specifically associated with mutations in the gene encoding the desmosome-associated protein ______?
plakoglobin
What is a genetic disorder leading to myocardial hypertrophy and diastolic dysfunction, leading to dec. stroke volume and often ventricular outflow obstruction?
Hypertrophic cardiomyopathy HCM
There are numerous mutations known involving sarcomeric proteins, the most common one is?
B-MHC : b-myosin heavy chain
HCM presents with massive myocardial hypertrophy often with marked septal hypertrophy, without dilation. What is important of this hypertrophy?
it is not concentric, instead eccentric asymetric septal hypertrophy (ventricular septum more thick than ventricular wall)
Microscopically, HCM shows myocyte/fiber disarray (haphazard disarray). What else can be seen?
interstitial and replacement fibrosis and fibrous endocardial plaques
What is said about the lumen in HCM, which is due to disproportionate septal and wall thickening without ventricular dilation?
Banana Lumen
What symptom of HCM is caused by the ventricular outflow obstruction as the anterior mitral leaflet moves toward the ventricular septum during systole?
HARSH systolic ejection murmur
hypertrophied ventricular septum BLOCKs aorta
Consequences of HCM include foci of myocardial ischemia, dec CO and inc pulomnary congestion leading to exertional dyspnea, arrhythmias and what other two important things?
SUDDEN DEATH (MC cause of sudden unexplained death in athletes) left atrial dilation & mural thrombus
What is caused by decreased ventricular compliance (inc stiffness), leading to diastolic dysfunction (impaired filling), while systolic function of the LV remains normal?
Restrictive Cardiomyopathy
Restrictive Cardiomyopathy may be secondary to deposition of material within the wall or increased fibrosis, each due to? 2
amyloid (deposition in wall)
radiation (increase fibrosis)
Restrictive Cardiomyopathy has patchy diffuse interstitial fibrosis and has normal sized ventricles but?
both atria can be enlarged
What is extracellular deposition of proteins which form an insoluble B pleated sheet, sometimes systemic (myeloma) or restricted to the heart (transthyretin)?
Amyloid
Certain mutations of transthyretin are more amyloidogenic, and it can involve different parts of the heart but when deposits are in the interstitium of the myocardium….?
resctrictive cardiomyopathy occurs
Amyloid can be seen in myocardial interstitium using what stain and light?
congo red stain and using polarized light which shows the amyloid as apple green color
Myocarditis is inflammation of the myocardium most commonly due to a virus in the US. Whats the most common virus?
Coxsackie A and B viruses
note viral=lymphocytes/bacterial=neutrophil
What disease causes 10% to die during acute attacks, progressing to cardiac insufficiency in 10-20yrs, and causing myocarditis?
Trypanosoma Cruzi (chagas dz)
Chagas can be seen with mixed inflammatory cell infiltrate (PMN, lymps, mø, and eosinophils), what can be seen in the myofibers?
parasitization of scattered myofibers (amastigotes to trypomastigotes in the heart muscle)
Some noninfectious causes of myocarditis include immune mediated reactions like RH, drug hypersensitivity and?
SLE
Active myocarditis is characterized by interstitial inflammatory infiltrate with focal myocyte necrosis, commonly lymphocytic. What can be seen histologically in hypersensitivity myocarditis?
interstitial infiltrates, principally perivascular composed of lymphocytes mø and eosinophils
What type of myocarditis is characterized by widespread inflammatory cellular infilitrate containing multinucleate giant cells with lympocytes, eosinophils, mø and plasma cells?
Giant cell myocarditis
Normal pericardial fluid is less than 50mL of clear, yellow colored fluid. Slow accumulation of fluid will allow how much and still be asymptomatic?
less than 500mL if slow enough = no sx
*globular enlargement of heart on CXR
Acute accumulation of 200-300mL rapidly fast is clinically devastating and can cause?
Cardiac tamponade (pericardial effusion)
What are the two most common types of pericarditis?
fibrinous and serofibrinous
Both sero and fibrinous pericarditis can be due to AMI, post infarction (dresslers), chest irradiation, RF, SLE, trauma and most importantly?
UREMIA
Serofibrinous pericarditis is a yellow-brown turbid fluid (chunky) with WBC, RBC and fibrin while fibrinous pericarditis is?
dry, finely granular/rough
Some sx of sero/fibrinous pericarditis includ pain (sharp, pleuritic and position dependent), fever, +/- CHF. what is the MOST COMMON feature seen?
** LOUD pericardial friction rub **
What pericarditis is characteristically produced by noninfectious inflammatory diseases including rheumatic fever, SLE, sleroderma, tumors and uremia?
Serous pericarditis
What pericarditis is an active infection caused by microbial invasion of the pericadial space (direct extension, seeding from the blood, lympathic extension, direct introduction)?
Purulent or suppurative pericarditis
What pericarditis has an exudate composed of blood mixed with a fibrinous or suppurative effusion?
Hemorrhagic pericarditis
MC d/t spread of malignant neoplasm
What pericarditis is tuberculous in origin and infrequently fungal infections evoke the same reaction?
Caseous pericarditis
What pericarditis occurs when the heart is encased in a dense, fibrous or fibrocalcific scar that limits diastolic expansion and cardiac output, features that mimic a restrictive cardiomyopathy?
constrictive pericarditis (limits diastole relaxation)
Primary cardiac tumors are rare, the top 5 most common are all benign. What are the two most important?
Myxoma
Angiosarcoma
Metastatic tumors occur only in 5% of people. What is the most common primary, pedunculated (sessile (on stalk) ) tumor usually in the fossa ovalis (foramen ovale)?
Myxoma (left atrium MC)
Familial syndromes associated with myxomas have activing mutations in the GNAS1 gene, encoding what?
subunit of G protein Gsa (associated with mccune albright syndrome)
Myxomas range from a globular hard mass mottled with hemorrhade to soft, translucent, papillary, or villous lesion with a ?
gelatinous appearance ***UNIQUE
Sx of myxomas include constitutional symptoms (fever malaise d/t IL6), embolization and?
Ball-valve obstruction
Pedunculated form of myxoma can cause obstruction during systole of AV valve, which can do what ?
‘wrecking ball’ does damage to the leaflets
ausculation = tumor “plop”
On histology, myxomas are composed of stellate or globular myxoma cells embedded with an abundant acid mucopolysacchride groud substance.. Whats the buzz work for histo?
Multinucleated myxoma cells / giant cells
What cardiac tumor is usually incidental, sea-anemone-like lesions, most often identified at autopsy and 80% are located on valves?
Papillary fibroelastoma (look like laml excresecences)
What is a tumor of the pediatric heart, 50% due to sporadic mutations and 50% associated with tuberous sclerosis (mut in TSC1 hamartin or TSC2 tuberin)?
Rhabdomyoma
What is the only malignant endothelial neoplasm that primarily affects older adult hearts?
Angiosarcoma
Cardiac transplants occur about 3000/yr, mostly due to dilated cardiomyopathy and ischemic heart disease. What is the major complication?
Allograft rejection (check via endomyocardial bx)
Allograft arteriopathy is the most important long term limitation for cardiac transplant because there is late…?
progressive, diffusely stenosing intimal proliferation
Allograft arteriopathy develops in 50% of people in 5 years, and virtually all patients in 10 years. This leads to what?
silent myocardial infarctions (because of the denervated transplanted heart (no angina) )
Along with arteriopathy, what is common due to chronic T cell immunosupression?
EBV associated B cell lymphomas
