Lecture 3: Cardiac Pathology Part 2 (Hillard) Flashcards
1
Q
What is the most common cause of arrythmias?
A
Ischemic Heart Disease
- also caused by cardiomyopathies, myocarditis, valvular disease, congenital disorders
almost ANY structural change can cause an arrhythmia
2
Q
What is Sick Sinus Syndrome and Atrial Fibrillation?
A
SSS: SA node damage causing bradycardia
- AV node takes over –> < 50-60 BPM
AF: myocytes depolarize independently and sporadically w/variable transmission in AV node (can be seen with atrial dilation)
- irregular HR = atrial fibrillation
- can cause thrombus formation/thromboembolism
3
Q
What is Heart Block and what is the difference between first, second, and third degree?
A
- dysfunctional AV node
First Degree: prolonged PR interval
Second Degree: intermittent transmission
Third Degree: complete failure
4
Q
Hereditary Channelopathies
What is Long QT Syndrome?
What is the classical presentation of disease?
A
- due to hereditary abnormal ion channels which cause arrhythmogenic disease (K+ LOF/Na+ GOF)
- Long QT is most common form
- cause of SUDDEN DEATH after EXERTION
Classic Pt: strong swimmer that drowns due to arrhythmia
- shows Torsades de Pointes on ECG
5
Q
What is Hypertensive Heart Disease?
What can it lead to the development of and what is another organ finding common in patients with disease?
A
- left ventricular hypertrophy (typically concentric, > 500 grams) with clinical history or evidence of hypertension in other organs (myocytes are larger and thicker)
- diastolic dysfunction leading to left atrial enlargement –> atrial fibrillation (can cause CHF or Sudden Cardiac Death)
look for granular kidneys with hyaline atherosclerosis
6
Q
What is Cor Pulmonale?
A
- isolated Right-sided Pulmonary hypertensive heart disease in the setting of Pulmonary Hypertension
- can arise due to disease of pulmonary parenchyma, vessels, or disorders of chest movements; also large pulmonary embolus
Left Heart Failure is MOST common cause of Right-sided Heart Failure
7
Q
What is Calcific Aortic Stenosis?
What are three common conditions it is associated with? (H/H/I)
A
- MOST COMMON VALVULAR ABNORMALITY (inc. risk with age - 60-80 yo)
- due to “wear and tear” associated with chronic HTN, hyperlipidemia, and inflammation
- see mounded calcifications in CUSPS that prevent complete opening of valve
8
Q
What is a Bicuspid Aortic Valve?
What is a complication that is frequently found with this disease?
A
- a congenital condition that shows accelerated course of aortic stenosis (shows symptoms 1-2 decades earlier)
- can become incompetent leading to valve dilation and prolapse
- bacterial endocarditis is commonly seen with this condition
9
Q
How does Calcific Aortic Stenosis commonly present?
A
- leads to inc. LV pressure and concentric LV hypertrophy (muscle becomes ischemic –> angina)
signs: systolic murmur, angina (die within 5 yrs), syncope (die within 3 yrs), and CHF (die within 2 yrs) - treat with SURGICAL REPLACEMENT
10
Q
What is Mitral Annular Calcification?
Who is it commonly seen in?
A
- calcific deposits in fibrous annulus (base of leaflets) of mitral valve that is usually asymptomatic
- can lead to regurgitation, stenosis, arrhythmias with deep calcifications; acts as nidus for thrombus and infective endocarditis
- commonly seen in WOMEN > 60 yo
11
Q
What is Mitral Valve Prolapse?
What genetic disease is it commonly seen in?
What do the leaflets look like in this disease?
A
- valve prolapse back into left atrium during systole (“floppy valve”) –> more common in WOMEN
- can be seen in MARFAN SYNDROME; also MI and rheumatic fever
- leaflets are thick and rubbery (Myxomatous Degeneration due to inc. proteoglycan deposits) and “hooding” (interchordal ballooning)
12
Q
How does Mitral Valve Prolapse present clinically?
A
- most asymptomatic with mid-systolic CLICK and is the most common cause of mitral regurgitation in developed countries; chronic can cause dyspnea
- can cause (rare): infective endocarditis, mitral insufficiency, arrhythmias (atrial), and thromboembolism
13
Q
What is Rheumatic Fever and what is used to confirm diagnosis?
A
- multisystem inflammatory disorder following GROUP A STREP pharyngitis (“SCARLET FEVER”)
- typically occurs 10 days-6 wks AFTER infection
- cannot culture active infection from blood
- caused by Abs and CD4+ T cell rxn against M Streptococcal Ag that causes rxn to heart/joint/skin/soft tissue/nervous system antigens (MIMICRY)
Confirm: Abs to STREPTOLYSIN O and DNase B
14
Q
What are 6 common signs of Acute Rheumatic Fever? (F/MP/P/SN/EM/SC)
A
- Fever (101 F+)
- migratory polyarthritis (large joints)
- pancarditis
- subQ nodules
- erythema marginatum (curved, ring-shaped macules)
- Sydenham Chorea (basal ganglia damage)
- hopping, halting gait, grimacing, asymmetric jerking
15
Q
What are 4 common heart findings in pts. with Acute Rheumatic Fever? (P/VV/MP/AB)
What valves does Rheumatic Heart Disease affect?
A
- pericarditis (fibrinous), myocarditis, endocarditis
- verrucae formation (valvulitis w/vegetations)
- MacCallum Plaques
- irregular thickening in Left Atrium due to regurg.
Histo: Aschoff Bodies w/Anitschkow cells
- PATHOMNEUMONIC
affects: mitral > aortic > tricuspid valves
- mitral valves have “fish mouth”/”buttonhole” stenosis
16
Q
How does Chronic Rheumatic Heart Disease present?
A
- valvular leaflet thickening, short chordae tendinae, fusion, and regurg. –> VALVULAR STENOSIS causing DIASTOLIC “rumbling” MURMUR
- structural changes predisposes valve to infective endocarditis
- can cause left atrial enlargement –> atrial fibrillation and thromboembolic events
17
Q
What are the most likely causes of infective endocarditis in patients with:
- native, structurally abnormal valves
- poor dentition/invasive dental procedures
- prosthetic valves
- IV drug use
A
- Streptococcus viridans
- HACEK organisms
- Staph epidermidis
- Stap. aureus
- causes RIGHT-SIDED endocarditis if injected
most infective endocarditis is LEF-SIDED
18
Q
What are the HACEK organisms associated with infective endocarditis in patients with poor dentition?
A
H - hemophilus A - actinobacillus C - cardiobacterium E - eikenella K - kingella
19
Q
What are 4 physical findings of infective endocarditis?
What are they all caused by?
A
- Subungal Splinter Hemorrhages
- Janeway Lesions (spots on palms/soles)
- Osler Nodes (tender nodules w/pale center)
- Roth Spots (retinal hemorrhage)
all are caused by small septic emboli that flake off of left-sided endocarditis
20
Q
What are 4 common causes of Nonbacterial Thrombotic Endocarditis? (S/C/AS/SLE)
A
- sepsis, cancer (procoagulant release), antiphospholipid syndrome (APS), and systemic lupus erythematous (LIBMAN-SACKS ENDOCARDITIS)
- all cause sterile, non-inflammatory valvular thrombi and are asymptomatic until embolization occurs
21
Q
What are Antiphospholipid Syndrome and Libman-Sacks Endocarditis?
A
APS: autoantibodies to endothelial membrane (ANTICARDIOLIPIN, lupus anticoagulant)
- manifests as fetal loss during pregnancy
- can also be associated with lupus
LSE: endocarditis caused by systemic lupus
- nonbacterial
22
Q
What is Carcinoid Heart Disease?
What side of the heart does it occur on and what are common signs of disease?
A
- bioactive compounds (SEROTONIN) from carcinoid tumors induce plaque-like endocardial/valvular thickening
- lesions are usually seen during massive metastatic liver burden and ONLY on RIGHT-SIDE of heart (lung vasculature degrades the mediators)
Signs: flushing, diarrhea, dermatitis, bronchoconstriction
23
Q
What is the difference between use of Mechanical Prosthetic valves and Tissue Prosthetic valves?
A
M: usually long lasting (>25 yrs)
- inc. risk of thromboembolism
- pts. on life-long anticoagulant therapy
T: made from cow or pig valves
- inc. risk of mechanical failure (usually 10-15 yrs)
- only on anticoagulants for 1st 3-6 months
both types have INCREASED risk of infective endocarditis
24
Q
What conditions are associated with:
- crescendo-decrescendo systolic murmurs
- holosystolic murmurs (2)
- diastolic decrescendo murmur
- diastolic rumbling murmur
- continuous “machine-like” murmur
A
- calcific aortic stenosis
- mitral regurgitation and VSD
- aortic regurgitation
- Rheumatic Heart Disease (mitral prolapse)
- patent ductus arteriosus
25
What is Cardiomyopathy and what are the three major types?
- structurally and functionally ABNORMAL heart with mechanical and/or electrical dysfunction in ABSENCE of coronary artery disease, HTN, valvular disease, and congenital heart disease
- Dilated (90% of cases), Hypertrophic, Restrictive
26
What are 5 common causes of Dilated Cardiomyopathy? (F/P/A/D/IO)
- familial (30-50%) due to TITIN AD mutation (TTN gene)
- peripartum (multifactorial) - late in pregnancy
- volume overload
- alcohol: wet beri-beri (thiamine deficiency)
- DOXORUBICIN (chemo drug)
- IRON OVERLOAD due to Hereditary Hemochromatosis (HFE gene) or multiple transfusions
27
What is morphology of Dilated Cardiomyopathy?
How does it present clinically?
M: dilation of ALL CHAMBERS and is often hypertrophic (inc. weight without thickened ventricle walls) and functional regurgitation
C: manifests between 20-50 yo with progressive CHF
- arrhythmias and thrombi formation
**SYSTOLIC DYSFUNCTION**
28
What is Takotsubo Cardiomyopathy?
Who does it commonly affect and what is it described as looking like?
- form of Dilated Cardiomyopathy associated with emotional distress ("broken heart syndrome")
- sudden catecholamine surge causes apical ballooning of LV, as well as inc. contractility and Coronary A. constriction (acute MI)
- common in 60-75 yo FEMALES; looks like Japanese Octopus Pot
29
What is Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?
- defective cell adhesion proteins in desmosomes linking myocytes cause RIGHT ventricular failure and arrhythmias (myocytes replaced by adipose and fibrosis w/dilation)
- classically familial and Auto Dominant
- causes ventricular tachycardia or fibrillation leading to sudden death
30
What is Naxos Syndrome?
- variant of ARVC with plantar and palmar hyperkeratosis and wooly hair
- caused by mutation in gene encoding PLAKOGLOBIN (desmosome-associated protein); adipose and fibrosis of right ventricular wall (SUDDEN DEATH)
- Auto RECESSIVE; presents as heart disease after 12 yo (need defib implant)
31
What is Hypertrophic Cardiomyopathy?
What is its histological appearance?
What mutation is most commonly implicated?
- relative common genetic disorder w/MALE prominence characterized by marked myocyte hypertrophy/disarray of SEPTAL PROMINENCE (no dilation)
- mutation is commonly Beta-Myosin heavy chain (B-MHC)
**histo shows irregular and whorled myocytes**
**DIASTOLIC DYSFUNCTION**
32
How does Hypertrophic Cardiomyopathy present clinically?
What is the classic presentation?
- usually asymptomatic
- ventricular arrhythmias = sudden death, mitral valve regurg. (systolic ejection murmur), exertional dyspnea and chest pain; palpitations
Classically: young teenage athlete dies while exercising
33
What is Restrictive Cardiomyopathy and what is it caused by? (A/F)
- dec. ventricular compliance leading to DIASTOLIC DYSFUNCTION (impaired filling)
- can be caused by amyloid deposition or increased fibrosis due to radiation
- ventricles are usually NORMAL size while both atria are ENLARGED --> congestive heart failure
34
What is Amyloid-associated Restrictive Cardiomyopathy?
What staining is done to test for it?
What are two common causes of this?
- extracellular deposition of proteins forming insoluble B-pleated sheets with APPLE GREEN birefringence on CONGO RED STAIN
- can be caused by mutated Transthyretin or Senile Amyloidosis (normal transthyretin)
35
Other Forms of Restrictive Cardiomyopathy
1. Endomyocardial Fibrosis
2. Loeffler Endocarditis (two cancer association)
3. Endocardial Fibroelastosis
1. children in tropical regions (Africa)
- fibrosis of endocardium and subendocardium
2. seen in tropical regions
- EOSINOPHILIC infiltration
- associated with leukemia and lymphoma
3. fibroelastic thickening of LV endocardium in 1st 2 YEARS of LIFE
- associated w/congenital heart defects
36
What is the most common infection associated with Myocarditis?
COXSACKIEVIRUS B
**viral infection is the most common cause of myocarditis**
37
What does infectious myocarditis due to Chagas Disease look like?
What is used to visualize it?
- caused by triatomine bug or "Kissing Bug"
- can see AMASTIGOTES on lower power imaging of myocytes
- use GIEMSA-stain to visualize Trypanosoma cruzi
38
How do Trichinosis and Lyme Syndrome cause infectious myocarditis?
T: helminthic infection due to eating undercooked pork
- larvae enter bloodstream and create cysts in muscle
LS: caused by spirochetes and is associated with Erythema Migrans (Bulls Eye rash) and polyarthritis/neurological issue
- carditis with TRANSIENT HEART BLOCK
39
What is is the difference between Lymphocystic and Eosinophilic Myocarditis?
L: most common form
- due to viral/post viral infection, autoimmune, idiopathic
E: marked inc. eosinophils
- due Hypersensitivity Myocarditis (allergy/hypersens)
- also idiopathic: methyl dopa and sulfonamides
- EXCLUDE PARASITIC INFECTION
40
Myocarditis with Giant Cells
What is the difference between Idiopathic Giant Cell Myocarditis and Myocardial Sarcoidosis?
IGC: aggressive with poor prognosis (< 3 months)
- admixed w/variable inflammation (eosinophils)
MS: immune-mediated granulomatous reaction
- giant cells w/non-necrotizing granulomas
41
What are two common cardiotoxic drugs (D/D) and what condition do they lead to?
What are 3 additional drugs that rarely cause serious injury? (L/P/C)
- Doxorubicin and Daunorubicin (chemo drugs)
- cause Dilated Cardiomyopathy and heart failure (dose-dependent lifetime toxicity)
also lithium (moods), phenothiazines (psychotics), and chloroquine (antimalarial) can occasionally cause reversible injury
42
What is the difference between Slow Accumulation and Acute Pericardial effusions?
SA: < 500 mL that's asymptomatic and has "heart shadowing" on CXR
A: 200-300 mL rapid accumulation (< 1 wk) that can cause symptomatic (or fatal) cardiac tamponade
**normal: < 50 mL clear, straw colored fluid
43
What is the difference between Fibrinous/Serofibrinous, Serous, and Purulent pericarditis?
F/S: MOST COMMON; "bread and butter" apperance
- Acute MI, Dressler's Syndrome
- see with uremia (chronic kidney disease, inc BUN)
S: viral or noninfectious inflammatory disease
P: ACTIVE infection via microbial invasion
44
What is Pericarditis and how does it present?
What does an ECG of a pt. with Pericarditis look like and what positions do they experience less pain?
- inflammation of pericardial sac
signs: chest pain (worse w/breathing), pericardial FRICTION RUB and effusion, fever
ECG: ST elevation (concave) and PR depression
- pt. has less pain sitting up, more pain lying down
45
What is the difference between Caseous, Hemorrhagic, and Constrictive pericarditis?
Ca: tuberculosis in origin, sometimes fungal
- looks "cheese-like" w/necrotizing granuloma
H: most commonly due to spread of malignant neoplasm; also TRAUMA
Co: dense, fibrous/fibrocalcific scar that limits diastolic expansion and cardiac output
- mimics restrictive cardiomyopathy
46
What is the most common cardiac tumor of adults and children?
Adults: myxoma
Children: rhabdomyoma
**metastatic tumors are MORE COMMON than primary tumors**
47
What is a Cardiac Myxoma and where does it typically occur?
What are common signs of Myxoma? (BV/F/P)
- stromal tumor of mesenchymal origin (gelatinous --> solid) located on LEFT ATRIUM in septal region of FOSSA OVALIS
signs: causes "Ball-Valve" obstruction that does mechanical valve damage (wrecking ball) --> embolization; fever (IL-6 inc. via tumor), and "plop" sound on ascultation
48
What are the differences between Cardiac Lipoma and Papillary Fibroelastoma?
L: localized mature lobulated fat mass
- can occur throughout the heart
PF: usually incidental; looks like "sea-anemone"; > 80% are located on the VALVES
- look like LAMBL EXCRESCENCES
- have avascular core
49
What are the differences between Cardiac Rhabdomyoma and Angiosarcoma?
What are the two genetic components of Rhabdomyoma?
R: most frequent benign tumor of pediatric heart
- hamartoma of developing cardiac myocytes
- 50% sporadic, 50% due tuberous sclerosis
- mutations in TSC1 (hamartin)/TSC2 (Tuberin)
**see large, vacuolated cells; "spider cells"**
A: malignant endothelial neoplasm that typically affects older adults
50
What is the difference between Cellular Mediated and Antibody Mediated Allograft Rejection after heart transplant?
CM: T cells; lymphocytic response
AM: antibodies; vascular neutrophilic inflammation
- reactive endothelium and macrophages
51
What is Allograft Vasculopathy?
What is a major clinical problem it leads to and why?
- late, progressive, diffusely stenosing intimal proliferation (50% in 5 yrs, all pts. within 10 yrs)
- causes concentric intimal thickening of BVs
- leads to SILENT MI; due to denervated transplanted heart (NO ANGINA)
