Hillard Cardiac Pathology Part 2 Flashcards
(91 cards)
1
Q
What is the most common structural birth defect?
A
Congenital heart defect, specifically VSD
2
Q
What causes congenital heart disease?
A
- most common due to sporadic genetic mutations
- fetal alcohol syndrome
3
Q
Describe left to right shunts and the symptoms.
A
- High pressure left heart or aorta (ox blood) moves to the lower pressure right heart or pulmonary trunk
- Initially asymptomatic, not cyanotic
4
Q
Describe right to left shunts.
A
- Blood bypasses pulmonary circulation
- Typically symptomatic and cyanotic
5
Q
What are the three L to R shunts?
A
- ASD
- VSD
- PDA
6
Q
What is the main type of ASD?
A
- Ostium Secundum (insufficient formation of septum secundum)
- 90% of all ASD’s are this and located in the center of the atrial septum
7
Q
Clinical features of ASD? Prognosis?
A
- usually asymptomatic until adulthood
- hear systolic ejection murmur
- Mortality is low and small defects spontaneously close
8
Q
What are the majority of VSDs? Clinical features? Prognosis?
A
- 80-90% are membranous VSD
- Asymptomatic until adulthood
- Holosystolic murmur
- ~50-70% small VSDs close spontaneously
- Prognosis depend on presence of other heart defects, those that have symptoms as kids are probably associated with other cardiac anomalies
9
Q
What is PDA?
A
- Ductus arteriosus fails to close when infants have increased pulmonary vascualr pressure
- Sx include hypoxia and other heart defects such as VSD
10
Q
Clinical features of PDA? (what is heard, symptoms, when do you close vs keep open)
A
- Typically asymptomatic
- Hear harsh machinery like murmmur peaks during systole but throughout diastole as well
- Initally L to R shunt so no cyanosis
- Isolated PDA should be closed with Indomethacin
- Preservation of patency with prostaglandin E1 can be life saving with certain congeintal malformations
11
Q
What is Eisenmenger syndrome?
A
- Occurs in long term L to R shunt results in:
- Increased pulmonary blood flow
- Endothelial dysfunction and pulmonary vascular remodeling ( irreversible)
- Increases backflow pressure (so we have increase in Pulmonary vascular resistance, blood doesn’t like to go in anymore)
- Results in inverted shunt, now it is right to left
12
Q
What are the threee R to L shunts? Cyanotic or not?
A
- Tetralogy of fallot
- Transposition of great arteries
- Tricuspid atresia
- Cyanosis at birth
13
Q
What are the symptoms in general of R to L shunts?
A
- “blue baby” cyanosis
- Clubbing of fingers (long term)
14
Q
What are the 4 features of tetralogy of fallot?
A
- VSD
- R. vent hypertrophy
- Subpulmonic stenosis (severity of this abnormality contributes to severity of overall disease)
- Overriding aorta (aorta is located in center of heart over the VSD)
15
Q
What is the most common Cyanotic congenital heart defect?
A
Tetralogy of fallot
16
Q
Clinical characteristics of tetralogy of fallot?
A
- Infants are cyanotic from birth in most cases
- VSD causes holosystolic murmur
- Squatting to increase systemic pressure
- Tet spell=cyanosis syncope during emotional distress, exercise
17
Q
What is a case of preserving a PDA?
A
Transposition of great arteries to allow mixing of blood
18
Q
Describe transposition of great arteries
A
- Aorta and pulmonary artery are switched
- Incompatible with life unless shunt is present allowing for mixing of blood
19
Q
Describe tricuspid atresia.
A
- Complete absence of triscupid valve
- Oxygenation maintained by ASD/PFFO and VSD
- Severe immediate cyanosis after birth
- High mortality- need surgery
20
Q
Congenital obstructive cardiac conditions?
A
- Coarctation of aorta
- Congenital aortic and pulmonary stenosis/atresia
21
Q
Coarctation of aorta?
A
- Focal narrowing of aorta
- Infantile form: coarctation with PDA
- Adult form coarctation without PDA
22
Q
Epidemiology of Coarctation of aorta?
A
- More common in males 2x
- Females assoc with turner syndrome
- Bicuspid aortic valve
23
Q
Describe the adult form of coarctation of aorta?
A
- upper body htn
- Lower body hypotension, weak pulses, arteiral insufficiency
- Long standing rib notching
- due to collateral intercostal vessels oxygenating causing a pressure erosion
24
Q
Infantile form of coarctation of aorta?
A
- Presents with cyanosis at birth on lower half of body only
- Severity depends on narrowing
25
What is congenital aortic stenosis/atresia?
* mild stenosis or sub aortic stenosis causing left ventricular hypertrophy
* hypertrophy of ventricle causes larger muscle amount but no increase in vasculature
26
What is a paradoxical emobolism? Where would an embolism normally go?
* Emboli arising in the venous system passes through a PFO and travels into arterial system causing a stroke
* Normally go to lungs only
27
What is a PFO?
* 80% close permanently by 2 years
* 20% can remain open if the right side pressure is increased
* Temporary increase in pressure can produce brief periods of R to L shunting
* valsalva
* Bowel movement
* Cough/sneeze
* Resulting in possible paradoxical emboli
28
3 Most common congenital heart defects with down syndrome?
* Atrioventricular\> Ventricular\> atrial
29
What is marfan syndrome? What cardiac risks do they have?
* Fibrillin 1 mutation, excessive TFG-B causing increase in MMP's which degrade elastin
* talll thin build, flexible joints, pectus excavatum, long arms/legs/fingeers
* Increase risk of **aortic aneurysm and aortic dissection**
* mitral or aortic valve prolapse
30
What is DiGeorge? What are the associated cardiac anomalies?
* CATCH-22
* cardiac anomalies, abnormal facies, thymic aplasia, cleft palate, hypocalcemia, deletion 22q11
* **Conotruncal heart** abnormalities **tetralogy of fallot,** transposition arteries, ASD VSD
31
What cardiac anomaly is turner syndrome associated with?
* Coarctation of aorta
32
What is the hypertensive heart disease criteria?
* Concentric left ventricular hypertrophy
* Other evidence of htn
33
How does hypertensive heart disease impact diastolic function and what can that lead to?
* Dysfunction of diastole (cant relax), left atrial enlargement, atrial fibriliation
* can lead to CHF and atrial fib can lead to thrombus
34
How does hypertensive heart disease impact systole?
* Systolic dysfunction, due to increase oxygen demand without vasculature to handle it leads to congestive heat failure
35
Describe the mechanism of right sided hypertensive disease? (Cor pulmonale)
* isolated right sided pulmonary hypertensive disease
* diseases of pulmonary parenchyma, vessels, or chest movement can increase the pulmonary pressure by constricting pulmonary small vessels
* this leads to increased resistance so high pressure in the pulmonary artery and therefore right side of heart
* eventually induces right side heart failure
36
What is the most common valve abnormality? Prevelance, risk factors?
* Calcific aortic stenosis
* increase with age around 60-80 yo
* “wear and tear” assoc. with chronic htn hyperlipidemia
37
What is a bicuspid aortic valve? What can it lead to?
* accelerated course of calcific aortic stenosis showing symptoms 1-2 decades earlier
* May become incompetent leading to aortic valve prolapse
38
What can a bicuspid aortic valve predispose an individual to?
* Infective endocarditis
39
How does a calcific aortic stenosis change the cardiac system and what are the signs and sx?
* Increases LV pressure leading to concentric left ventricular hypertrophy
* Signs and sx:
* systolic murmur, syncope, angina, CHF
40
Prognosis of aortic stenosis with angina, syncope and CHF?
* Angina die w/n 5 yrs
* die w/n 3 years with syncope
* Die w/n 2 years with CHF onset
41
What are mitral annular calcifications?
* calcific deposits in the fibrous annulus (base of leaflets)
* Females \>60yo
* Can cause arrhythmia
* Can lead to infective endocarditis
42
What is MVP? Prevelance? Causes?
* Valve leaflets prolapse back into left atrium during systole
* F:M 7:1
* Caused by marfan syndrome or complication of MI or rheumatic fever
43
Clinical findings of MVP?
* Mid systolic click but asymptomatic
* However chronic MVP can lead to dyspnea
* Complications include:
* infective endocarditis
* Arrhythmia
* Find:
* Leaflets thick and rubbery (myxomatous degeneration)
* Interchordal ballooning (hooding) of leaflets
44
Rheumatic fever signs and symptoms?
* Fever
* Migratory polyarthritis
* Pancarditis
* Subcutaneous nodules
* Erythema marginatum
* Syndham chorea
45
Rheumatic fever: Acute Rheumatic heart disease
* Pericarditis myocarditis and or endocarditis
* Valvulitis with vegetation
* MacCallum plaques
* Aschoff bodies with Anitschkow cells (acute rheumatic heart disease indicator)
46
What valves are affected with rheumatic heart disease?
* Mitral\>aortic\>tricuspid
47
Describe chronic rheumatic heart disease
* Valvular leaflet thickening short chordae tendinae, fusion, regurgitation
* Valvular stenosis Mitral\>Aortic\>Tricuspid
48
What causes infective endocarditis?
* infectious organism, inflammation,fibribnous debris
* Occur on abnormal or prosthetic valves
* Vast majority are bacterial
49
Risk factors for infective endocarditis?
* **IV drug use**
* piercings, males
* **Poor dentition, invasive dental procedures**
50
Preexisting conditions with infective endocarditis?
* **Valvular disease** such as rheumatic heart disease, MVP, calcific stenosis
* **Prosthetic heart valve**
51
Acute endocarditis symptoms?
* Rapid development of fever, chills, weakness
* diagnose pathologically by histology or clinically by duke criteria
52
Symptoms of subacute endocarditis?
* Low grade fever \<101
* Fatigue
53
Minor Duke Criteria for endocarditis?
* Subungual splinter hemorrhages
* Janeway lesions (not painful)
* Osler nodes (painful)
* Roth spots
54
With infective endocarditis is staph aureus acute or subacute, early or late prosthetic valve infection, risk factos and unique features?
55
With S. viridans describe infective endocarditis type, infection of valves, risks and unique feautres.
56
With enterococci describe infective endocarditis type, infection of valves, risks and unique feautres.
Subacute and NO and nothing for risks and features
57
with S. epidermidis describe infective endocarditis type, infection of valves, risks and unique feautres.
58
with HACEK group describe infective endocarditis type, infection of valves, risks and unique feautres.
59
Nonbacterial thrombotic endocarditis?
* valvular thrombi form made of platelet rich fibrin clots
* Due to hypercoagulable conditions
* cancers, Antiphospholipid syndrome, lupus and sepsis are causes
60
What is carcinoid heart disease?
* Bioactive compounds (**serotonin**) secreted by carcinoid tumors induces plaque like endocardial valvular htickening and carcinoid syndrome
61
What are s&s of carcinoid syndrome?
* **flushing**
* **diarrhea**
* dermatitis
* **Bronchoconstriction**
* occurs when metz to the liver
62
Reference for murmurs
63
What is cardiomyopathy?
* "heart muscle disease"
* structurally and functionally abnormal with mechanical and or electrical dysfunction in absence of coronary artery disease, htn, valvular disease, congenital heart
64
Describe type of dysfunction, cardiac abnormality and a unique feature in dilated cardiomyopathy
65
Describe type of dysfunction, cardiac abnormality and a unique feature in hypertrophic cardiomyopathy.
66
Describe type of dysfunction, cardiac abnormality and a unique feature in restrictive cardiomyopathy
67
What causes dilated cardiomyopathy?
* Familial TTN gene Titin mutation in 20% of the 30-50% of familial cases, AD
* Peripartum cardiomyopathy
* Alcohol
* Doxorubicin and daunorubicin
* Iron overload
* Takotsubo cardiomyopathy (catecholamine overload “broken heart syndrome”)
68
clinical features of dilated cardiomyopathy?
(age it presents, systolic or diastolic, what is dilated, what are the causes?)
* manifest between 20 and 50 yo
* Progressive dilation of ALL four chambers
* Systolic dysfunction
* Causes:
* Genetic 30-50% (titin mutation)
* Peripartum
* alcohol
* doxorubicin/danorubicin,
* Iron overload
69
What is takotsubo cardiomyopahy?
(associated with, what is released, what happens to L. ventricle, what does it mimic, result in?)
* “Broken heart syndrome”
* associated with emotional distress
* Sudden surge of catecholamines
* Apical ballooning of left ventricle
* signs and sx of acute MI
* can result in Ischemic cardiomyopathy or cardiac death
* Epidemiology is 90% women
70
What are characteristics of hypertrophic cardiomyopathy? Mutation?
* Characterized by myocyte hypertrophy and myocyte disarray
* Myocyte hypertrophy is marked with septal prominence
* Genetic disorder with male predominance
* Numerous mutations involving sarcomeric proteins most commonly, B-myosin heavy chain (B-MHC)
71
Classic presentation of hypertrophic cardiomyopathy?
* Most are asx but can present with:
* Sudden unexplained death in an athlete during exercise
* Systolic ejection murmur (mitral valve gets pushed to the septum)
72
What causes restrictive cardiomyopathy?
* Decreased ventricular compliance (increased stiffness) leading to diastolic function (impaired filling)
* amyloid
* increased fibrosis (radiation)
73
How is amyloidosis related to restrictive cardiomyopathy?
* Can involve different parts of the heart but when it invovles the myocardium restrictive cardiomyopathy is the result
74
What can amyloidosis be due to?
* myeloma
* chronic inflammatory states
* Mutated versions of transthyretin
* Senile amyloidosis
75
What is amyloid?
* deposition of proteins forming an insoluble beta pleated sheet
* can see apple green birefringence with congo red stain
76
What is endomyocardial fibrosis?
* Seen in young children young adults in tropical and subtropical regions (africa)
* Fibrosis of the endocardium and subendocardium
77
Loeffler endocarditis?
* Eosinophilic infiltration
* Part of spectrum of endomyocardial fibrosis
* Associated myeloproliferative (leukemia/lymphoma)
78
Endocardial fibroelastosos?
* Fibroelastic thickening of left ventricle endocardium in the first two years of life
* associated with congenital heart defects
79
What is arrhythmogenic right ventricular cardiomyopathy?
* Defective cell adhesion proteins in desmosomes that link adjacent cardiac myocytes
* Many cases are familial and AD
* myocardium of right ventricular wall replaced by **adipose and fibrosis with dilation**
* Causes ventricular tachycardia, premature contractions or fibrillation leading to sudden death
80
What is Naxos syndrome?
* ARVC with plantar and palmar hyperkeratosis of plantar palmar skin surfaces and wooly hair
* Mutations in the gene encoding desmosome associated protein Plakoglobin
81
Myocarditis clinical presentation? Most common cause?
* Viral infection by Coxsackie B and covid 19 are most common cause
* asymptomatic to heart failure to arrhythmia and death
* Troponin can be elevated and heart can show motion anomalies on echocardiogram
82
How does chagas disease relate to infectious myocarditis?
* amastigotes encyst in tissue cells in the heart
83
How does trichinosis cause infectious myocarditis?
* The worm larvae encysts in muscle and that can include heart muscle
84
How does Lyme disease relate to the heart?
* Carditis with heart block in early disseminated phase of lyme disease
* along with migratory polyarthritis and facial palsy and meningitis
85
Lymphocytic myocarditis?
* most common form of myocarditis
* **Viral/post viral infection**, autoimmune or idiopathic
86
Eosinophilic myocarditis?
* Characterized by marked increase of eosinophils
* Hypersensitivity myocarditis:
* due to underlying allergy/hsn rxn typically to drugs
* *need to eliminate potential parasite infectio*
87
Idiopathic Giant cell Myocarditis?
* Aggressive with poor prognosis, survival is \<3months from diagnosis
* Giant cells admixed with variable mixed inflammation
88
Myocardial Sarcoidosis?
* Idiopathic process rare cause of myocarditis with variable presentation
* Giant cells with non necrotizing granulomas
89
What congenital heart disease will not present with cyanosis immediately after birth?
* Tetralogy of Fallot
* Transposition of great arteries
* Tricuspid atresia with septal defect
* Coarctation of aorta with PDA
* VSD
VSD
90
You have a patient with metastatic cancer and bx of solid cystic ovarian mass shows mucinous cystadenocarcinoma, what type of cardiac abnormality is associated with this?
Non bacterial thrombotic endocarditis
91
Causes of prinzmetal angina?
* Cocaine abuse
* Elevated T4
* Caffeine use
* Autoantibodies
* Pheochromocytoma
