Cardiac Path Flashcards
1
Q
What is the predominant coronary artery in most people?
A
90% RCA dominant
2
Q
How many people have patent foramen oval?
A
20-25%
3
Q
What are specialized end-end junctions of adjoining cardiac cells?
A
Intercalated discs
4
Q
What are 6 basic causes of cardiac dysfunction?
A
1) Pump failure
2) Obstruction to Blood Flow through heart
3) Regurgitant flow
4) Shunted Flow
5) Disorders of Cardiac Conduction
6) Disruption of continuity of circulatory system
5
Q
Cardiac hypertrophy
A
Increase in ventricular thickness
6
Q
Cardiomegaly
A
Increase in heart size/weight
7
Q
What is a normal heart weight?
A
Male 300-350gm
Female 250-300 gm
8
Q
Pressure Overload Hypertrophy
A
Concentric hypertrophy with increase in wall thickness of stressed ventricle
9
Q
Volume Overload Hypertrophy
A
Eccentric hypertrophy with chamber dilation/increased ventricular diameter
Ventricle wall normal or minimally thickened
Due to increase in chamber size overall cardiac muscle mass is increased
(Heart getting larger not thicker)
10
Q
CHF
A
Insufficient pump rate to meet demands
Pump can only meet demands with elevated filling pressure
11
Q
Systolic Heart Failure
A
Decreased LV contraction
Low EF
12
Q
Diastolic Heart Failure
A
Decreased LV compliance with impaired relaxation
Normal EF at rest
S4 atrial gallop due to increased resistance to filling in late diastole
Usually accompanied by pulmonary congestion
13
Q
When might isolated RHF occur?
A
With severe chronic pulmonary HTN = cor pulmonale
14
Q
What level of BNP is most consistent with CHF?
A
BNP >500
15
Q
When do congenital heart defects occur?
A
Between 3-8 weeks gestational age
16
Q
NKX2.5 gene mutation
A
Non-Syndromic
ASD or conduction defects
Congenital Heart Disease
17
Q
Holt-Oram Syndrome
A
TBX5
ASD, VSD, Conduction defects
18
Q
DiGeorge Syndrome
A
TBX1 del 22q11.2
Cardiac outflow tract obstruction
ASD/VSD
19
Q
Left to right shunt develops Pulm HTN and then…
A
Eisenmenger syndrome as the right heart has increased pressure over the left heart and the shunt reverse form right to left
Now we have RV volume and pressure hypertrophy
Cyanosis may occurs months to years after birth
20
Q
Types of ASD
A
1) Secundum: most common a patent foramen ovale
2) Primum: adjacent to AV valve
3) Sinus venosus: near SVC
21
Q
How often do ASD pt’s present with Pulm HTN?
A
only 10% it is generally well tolerated and pt tend to be asymptomatic
22
Q
When does the ductus arteriosus usually close and what causes it to close?
A
Increased O2
Decreased pulmonary resistance
Decreased prostaglandin E2
Usually closes 1-2 days
23
Q
How does a PDA present and how do you treat it?
A
Presents as a continuous harsh machine like murmur
If chronic develop pulmonary HTN and cyanosis
Tx NSAIDs to close
Sets up Left to Right shunt which is why pulmonary HTN develops
24
Q
What population most commonly has AVSD?
A
Down Syndrome Pt >1/3
40-60% have CHD with AVSD leading the list
25
What are the four defects in Tetralogy of Fallot?
1) VSD
2) Subpulmonic stenosis with obstruction of right ventricular outflow
3) Aorta overrides VSD (Dextrorotated aorta with right sided aortic arch)
4) RVH
26
Supravalvular aortic stenosis
Elastin gene mutation with aortic dysplasia thickening often presents with Williams Beuren syndrome
27
Williams Beuren Syndrome
Deletion of 28 genes on chrome 8 with elastin gene
hypercalcemia
glucose intolerance
facial and cognitive defects
28
Coarctation of aorta infantile form
cyanosis of inferior body and weak femoral pulses
29
Coarctation of aorta adult form
```
Increased UE BP
Decreased SBP and pulse in LE
Leg claudication
Renal HTN
Blood flow through collaterals
Pansystolic murmur
```
30
4 Clinical Syndromes of Ischemic Heart Disease
1) Sudden cardiac death
2) Angina pectoris
3) Chronic IHD with heart failure
4) MI
31
Most ischemic heart disease is due to
Atherosclerotic coronary arterial obstruction
32
IHD is uncommon in
Premenopausal women
33
How much of a fixed obstruction is required to cause Sx during exercise?
>75%
34
How much of a fixed obstruction is required to cause Sx during rest?
>90%
35
Acute ruptures with subsequent thrombosis occurs...
In vessels that are narrowed less than 50%
36
Acute plaque change usually occurs in...
Non-severely stenotic portions of arteries
| New plaques with thin fibrous caps are most likely to rupture
37
Prinzmetal (Variant) Angina
Sustained vasospasm causing angina
38
Sudden Cardiac Death
Unexpected death from cardiac causes early after onset of symptoms (1 to 24 hours) or sudden death from cardiac cause without antecedent acute symptoms
39
Channelopathies
Disorders of K, Na or Ca channel structures or accessory proteins involved in channel function
Mostly autosomal dominant
40
Chronic Ischemic Heart Disease
Insidious/progressive CHF resulting from long term ischemic damage to myocardial tissue via MIs or angina
Due to replacement of tissue with non-contractile scar tissue which decreases the overall contractile force
41
Unstable angina
Acute plaque change
Progressive increase in frequency and severity of attacks
Provoked by progressively less effort and may occur at rest
42
Stable angina
Decreased perfusion secondary to fixed narrowing
| Can be provoked by increased cardiac demand during emotion or exercise
43
Lipid risk factors account for how many MI's?
50-60%
44
Transmural Infarction
Ischemic necrosis involves >50% of the ventricular wall thickness
Commonly associated with acute plaque change with thrombosis
45
Subendocardial infarction
Area of ischemic necrosis limited to the inner 1/3 or at most the inner 1/2
May occur as a result of acute plaque change and thrombosis
May result from prolonged and severe reduction in systemic blood pressure ,as encountered in shock
46
MI Pathogenesis
- Initial sudden change in plaque
- Immediate formation of initial platelet plug over plaque
- Vasospasm from platelet adhesion (vasoactive substances from platelets)
- Propagation of platelet plug into stable clot via extrinsic clotting system
- Within minutes, clot occludes lumen of the involved vessel = thrombosis with coronary occlusion
47
Adrenergic Stimulation of MI
Peak incidence btw 6am-12pm
| Awakening causes intense emotional stress
48
When does complete unsalvageable necrosis occur in MI?
6 hrs
49
Which coronary artery is most commonly obstructed?
LAD
50
What are the MI modification by reperfusionn techniques?
Lysis of thrombus
Balloon angioplasy
Coronary a. bypass graft
51
STEMI
ST segment elevation MI transmural
52
Non-STEMI
Non ST segment elevation MI subendocardial
53
Cardiac tamponade
Pressure on the heart that occurs when blood or fluid builds up in the space between the heart muscle (myocardium) and the outer covering sac of the heart (pericardium)
Complication of acute MI
54
Systemic Hypertensive Heart Disease
1) LV hypertrophy in the absence of other cardio pathology that may cause it
2) Systemic HTN
May present clinically with CHF or atrial arrythmias
55
Pulmonary Hypertensive Heart Disease
Cor Pulmonale occurs with
1) Pulmonary parenchyma disorders (COPD, diffuse interstitial lung disease)
2) Pulmonary vessel disorders (recurrent PE, Pulm HTN)
3) Chest movement disorders (kyphoscoliosis)
4) Disorders inducing pulmonary arterial constriction (Metabolic acidosis, hypoxemia)
56
Functional regurgitation
Normal valve leaflets but problem with supporting structures
57
Stenosis Pathophys
Almost always a primary valve cusp abnormality and virtually always a chronic disease
58
Insufficiency Pathophys
From intrinsic disease of valve cusp or acquired structural abnormality of supporting anatomical structures
59
Most frequent cause of Aortic Stenosis
Calcification of anatomically normal and congenitally bicuspid aortic valves
60
Most frequent cause of Aortic Insufficiency
Dilation of ascending aorta due to HTN and aging
61
Most frequent cause of Mitral Stenosis
Rheumatic heart disease
62
Most frequent cause of Mitral Insufficiency
Myxomatous degeneration (mitral valve prolapse)
63
Most common valvular abnormality
Calcific Aortic Stenosis
64
Calcific Aortic Stenosis
Consequence of calcification due to advanced age
Nodular masses of Ca heaped up in sinuses of Valsalva
Clincial Sx present in 7th (60s) to 9th (80s) decade
LV concentric hypertrophy from flow obstruction
Tends to become ischemic leading to CHF (death w/i 2yrs) Syncope (death w/i 3yrs) Angina pectoris (death w/i 5yrs)
65
Calcific Stenosis of Congenitally Bicuspid Aortic Valve
Two cusps not equal size
More susceptible to progressive degenerative calcification earlier
Develop clinical Sx and signs of cardiac dysfunction in 5th-6th decades, earlier than tricuspid calcification
66
Mitral Annular Calcification
Occurs in
1) Women over 60yrs of age
2) Individuals with myxomatous mitral valves
3) Pt with elevated LV pressure (systemic HTN)
Generally doesn't affect valvular function
Occasionally associated with arrythmias
Most diagnosed because of large Ca deposits found on CXR
67
Myxomatous Degeneration of Mitral Valve
One or both leaflets floppy and prolapse into LA during systole
MIDSYSTOLIC CLICK +/- REGURGITANT MURMUR
Most often in young women
Seen with Marfan's
Clinically asymptomatic
68
Four Serious complications of myxomatous degeneration of mitral valve
1) infective endocardiits
2) mitral insufficiency
3) stroke/iscemic infarct
4) arrhythmias
5) atypical chest pain
69
Pathological Changes of Myxomatous Degen of Mitral Valve
Intercordal ballooning of mitral valve leaflets
Leaflets are enlarged, thick and rubbery
Thinned fibrosa layer, marked thickened spongiosa layer with mucoid deposition
Dilation of annulus, fibrosis of leaflets and endocardial surface = jet lesions
70
Acute Rheumatic Fever
Occurs 10 days- 6 weeks post strep pharyngitis
Ab against group A strep Ag (hypersensitivity rxn to M protein cross-reacting with tissue glycoproteins)
Develop fever and polyarthritis
= Acute Rheumatic carditis
Small warty verrucase along lines of closure of leaflets
71
Aschoff cells
multinucleated forms of caterpillar cells in unique linear chromatin pattern
72
Acute Rheumatic fever clinical Sx and diagnostic criteria
Affects 5 major systems
1) migratory polyarthritis of large joints (swollen, painful joints)
2) Acute carditis with cardiac enlargement and diminished function
3) SubQ nodules
4) Erythema marginatum of skin
5) Sydenham chorea (involuntary purposeless movements of extremities)
Jones criteria requires evidence of prior group A strep infection plus either: 2 major system findings or 1 major system finding plus 2 minor manifestations such as fever, arthralgia, or evidence of acute phase reactants (elevated see rate or elevated C-reactive protein)
73
Acute Rheumatic Fever Tx
Pt should receive long term antibiotic PCN prophylaxis into adulthood and perhaps for life
74
Rheumatic Heart Disease
| Which valves are most commonly affected
Term used for chronic valvular disease that results
| Most commonly affects the mitral and aortic valves
75
Infective Endocarditis
Serious destructive infection of heart valves or mural endocardium by organisms
Involves left valves except in IV drug users
76
Infective Endocarditis Morphologic Changes
Bulk friable vegetations of fibrin-platelet clot and organisms
Large irregular masses on valve cusps that can extend onto chordae
Destruction of underlying cardiac organisms especially valves
Risk of systemic microemboli leading to
77
Infective Endocarditis Organisms
S.viridans, S. aureus, HACEK
| S.epidermidis #1 in artificial valves
78
Acute Bacterial Endocarditis
Rapidly progressive destruction of infected valve which was normal prior to infection
Highly virulent bacteria infection such as S. aureus in IV drug abusers
Even with aggressive Ab therapy 50% of pt die
79
Subacute Bacterial Endocarditis
Insidious onset and lengthy clinical course
Deformed/defected valve prior to infection (mitral valve prolapse)
Low virulence bacterial infection (S. viridians MCC)
Pt recover with Abi therapy
80
Diagnostic Criteria for Infective Endocarditis
GET 3 BLOOD CULTURES IN 24 HRS
New valvular regurg MURMUR and ECHO findings all MAJOR along with a + blood test
*Diagnosis by these guidelines, often called the Duke Criteria, requires either pathologic or clinical criteria; if clinical criteria are used, 2 major, 1 major + 3 minor, or 5 minor criteria are required for diagnosis.
81
Tetralogoy of Fallot Morphology
Boot shaped heart
82
Nonbacterial Thrombotic Endocarditis
Small 1-5mm along line of closure of valve leaflets or cusps, small bland vegetations on line of closure
Lesions are sterile & non-destructive
Major risk factor is hyper-coagulable state
May fragment and produce system emboli
"Marantic endocarditis"
Paraneoplastic syndrome
83
Libman-Sacks Disease
= Endocarditis of SLE
Small 1-4mm sterile verrucous vegetations on any surface of Av leaflets, valvular endocardium, chord tendinae
Vegetations on either or both sides of valve leaflets or elsewhere
SLE pt with antiphoshpholipid Ab syndrome
May be due to IC deposition with inflammation
84
What is the most consistent clinical sign for infective endocarditis?
Fever! usually of unknown origin
85
Carcinoid Heart Disease
Result of tumor of the small intestine that has metastasized to the liver and is producing serotonin there which gains access to the hepatic vv. IVC and then right heart causing increased fibrosis which results in TV regurg and PV stenosis
Fibrous intimal thickening of endocardial surfaces of right heart
Thickening is composed of muscle proliferation and increased acid mucopolysaccharide matrix
86
Cardiomyopathy
Heart disease resulting from primary abnormality in the myocardium
Either dilated, hypertrophic or restrictive
87
Myocarditis
Global enlargement of heart and dilation of all chambers
Lymphocyte infiltrate with focal necrosis highly predictive of viral myocarditis
Major cause of sudden death
Presents with dyspnea, fever, chest pain, arrhythmias
88
Causes of myocarditis
```
Coxsackievirus A & B
Borrelia burgorferi
Rickettsiae rickettsi
Hypersensitivity
Tricihnella spiralis
Trypanosoma cruzi causes Chagas Disease in South America
```
89
What is the most common indication for cardiac transplantation?
Dilated Cardiomyopathy
90
Dilated Cardiomyopathy
Insidious slowly progressive CHF w/
1) Progressive cardiac dilation
2) Contractile (Systolic) dysfunction (decrease in contractility leads to global heart enlargement)
3)Cardiomegaly with increased cardiac mass (hypertrophy)
Sx of CHF but poorly responsive to CHF treatments
91
Causes of Dilated Cardiomyopathy
1) Myocarditis (Coxsackie B virus)
2) Alcohol
2) Peripartum cardiomyopathy
3) Iron overload
4) Familial (presents in children with autosomal dominant pattern)
5) Supraphysiologic stress
92
Morphology of DCM
```
Increased size (2-3x)
Large and flabby
Dilation of all chambers
Ventricular wall thickness may be normal, thin or thick
Mural thrombi common
Normal valves and aa. free of narrowing
```
93
Arrythmogenic RV Cardiomyopathy
Right sided heart failure and rhythm disturbances
Causes of unexpected death
RV severely thinned with myocytes being replaced with adipocytes and interstitial fibrous tissue
Autosomal dominant mutation in plakoglobin, desmoplakin genes
Naxos syndrome
94
Naxos Syndrome
Palmoplantar keratoderma with arrythmogenic RV cardiomyopathy and wooly hair (recessive plakoglobin mutation)
95
Carvajal Syndrome
Palmoplantar keratoderma with arrythmogenic LV cardiomyopathy and wooly hair (recessive desmoplakin mutation)
96
What is the most common mutation in HCM?
Mutation of B-myosin heavy chain gene on Chr. 14
97
Hypertrophic Cardiomyopathy
Features myocardial hypertrophy, abnormal diastolic filling, intermittent ventricular outflow obstruction with systolic ejection murmur
98
HCM Clinical Features
Major cause of SCD in young people including basketball and football athletes
Most pt have stable non-progrssive course
Major Sx: exertional dyspnea
May exhibit Afib
99
HCM vs. DCM
Ventricle/Force/Dysfunction
/Clinical/Mutations
HCM: firm small lumen with thick wall due to hypertrophic IVS
Hypercontracting, Distolic dysfunction, Exertional dyspnea, Sarcomere protein mutation
DCM: dilated and flabby, hypocontracting, systolic dysfunction, CHF, cytoskeleton protein mutation
100
Restrictive Cardiomyopathy
Mild increase in heart size/mass without increase in volume of LV
Characterized by decreased ventricular compliance resulting in impaired filling during diastole
Bilateral atrial enlargement
Associated with amyloidosis, endomycoardial fibrosis, loeffler endomyocardiits, endocardial fibroelastosis
101
Endomyocardial fibrosis
endocardial and subendocardial fibrosis with mural thrombi, occurs in African children/young adults and in other tropical areas (most common worldwide)
102
Loeffler endomyocarditis
endomyocardial fibrosis with large mural thrombi that occurs worldwide and is associated with eosinophilic leukemia [some have platelet derived growth factor receptor abnormalities (Rx tyrosine kinase inhibitor imatinib) or may be secondary to basic protein release]
103
Endoardial fibroelastosis
Multifactoral LV endocardial fibrosis that usually occurs in first 2 years of life
104
Amyloidosis
Interstitial deposition of amyloid protein results in restrictive cardiomyopathy complicated by arrhythmias
Amyloidosis primariliy in heart in systemic senile in pt >60
Amyloid composed of transthyretin product and more common in AA due to transthyretin mutation
105
Direct Cardiac Toxicity: Adriamycin
Lipid peroxidation of myocyte membranes
106
Direct Cardiac Toxicity: Cyclophosphamide
Vascular lesion with myocardial hemorrhage
107
Direct Cardiac Toxicity: High dose Catechoalmines
Vasopressors toxicity via calcium overload or vasoconstriction and increased cardiac load
108
Direct Cardiac Toxicity: Iron-Overload
Interferes with metal dependent enzyme systems
109
Myxoma
Can cause fever and malaise via interleukin 6
Carney complex includes myxomas, pigmented skin lesions and overactive endocrine organs
Large pedunculated lesion arising from fossa ovalis
110
Rhabdomyoma
#1 in children and 50% associated with tuberous sclerosis (TSC1/TSC2 genes)
111
Chylous Pericardial effusion
Lymphatic obstruction
112
Serosanginous pericardial effusion
Malignancy, trauma, rupture MI, aortic dissection
113
Serous pericardial effusion
CHF, hypoalbuminemia
