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Valvular and Congenital heart Disease Flashcards

1
Q

4 cardinal features of Tetralogy of Fallot

A
  1. VSD
  2. Pulmonary stenosis (obstruction of flow from RV to pulmonary artery)
  3. Right ventricular hypertrophy
  4. overriding aorta (aortic valve is enlarged and appears to arise from both ventricles)

-also shows a “boot-shaped” heart on CXR

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2
Q

What is the most common cause of aortic stenosis?

A

Calcific aortic degeneration

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3
Q

What is Eisenmernger Syndrome?

A

The conversion of Left-to-right sided shunting to right-to-left

  • due to volume and pressure overloads that eventually cause pulmonary hypertension and right-sided pressures that exceed those on the left resulting in flow reversal
  • becomes a cyanotic defect
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4
Q

Pathogenesis of Rheumatic Heart Disease?

A

Molecular mimicry

  1. Abs against streptococcal M proteins bind the myocardium and valves
  2. Abs activate complement and Fc receptor-bearing cells (including macrophages)
  3. CD4+ T cells that recognize streptococcal peptides also cross react with host antigens elicit cytokine-mediated inflammatory responses

-characteristic 2-3 week delay (after infection) is due to the time needed to mount the immune response

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5
Q

How does Rheumatic valvular disease effect the heart

A

It causes inflammation of all parts of the heart

**Valvular inflammation and scarring are most important clinically

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6
Q

T or F: Streptococci are found in the lesion of the heart

A

F

They are completely absent from the lesions

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7
Q

Disease characterized by

- deposits of small thrombotic masses composed of mainly fibrin and platelets on cardiac valves

A

Noninfected vegitations (Marantic endocarditis)

  • sterile masses (i.e. no infectious organisms)
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8
Q

Which form of aortic coarctation typically has PDA?

A

Infantile

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9
Q

What are the Left-to-Right shunts?

A
  1. Atrial Septal Defect (ASD)
  2. Ventricular Septal Defect (VSD)
  3. Patent Ductus Arteriosus (PDA)
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10
Q

Cardiac defect associated with Turner syndrome?

A

Coarctation of the aorta

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11
Q

What is a paradoxical embolus? When does it occur?

A

A venous emboli that enters systemic arterial circulation

  • occurs if right-sided atrial pressures increase, such as with pulmonary HTN or a valsalva maneuver
  • may enter the the arterial circulation through a patent foramen ovale
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12
Q

Gross characteristics of Chronic Rheumatic heart disease

A
    1. Fibrous bridging across the valve and calcification create a Fishmouth or Buttonhole stenosis. (may also be insufficiency)
      1. Chordae tendineae become thick and FUSE!
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13
Q

Cardiac defect associated with fetal alcohol syndrome?

A

VSD/ASD

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14
Q

Where does calcification of the valve normally occur

A

The leaflet

- not the edges (So no fusion - differentiates from Rheumatic heart disease)

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15
Q

Failure of a heart valve to open completely is called? What causes it?

A

Stenosis

- Primary cuspal abnormality
Calcification or Valve Scarring
- usually a chronic process

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16
Q

What is the most common Left-to-right congenital heart defect?

A

Ventricular Septal defect (VSD)

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17
Q

Patients with SLE or antiphospholipid Ab syndrome have what type of endocarditis?

A

Libman-Sacks endocarditis (LSE)

- a type of noninfectious vegitation

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18
Q

The congenital heart defect associated with the failure of the primitive truncus to divide into the pulmonary artery and aorta?

What genetic abnormality (and disease) is it associated with?

A

Persistent truncus arteriosus

  • Del 22q which includes DiGeorge
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19
Q

Morphological features of Rheumatic fever

A

Pancarditis (Affect all layers of heart)
1. Pericardium - fibrinous exudate

  1. Myocardium - myocarditis takes form of scattered Aschoff bodies and Anitschkow cells
  2. Valve (endocardium) - Fibrinoid necrosis
    - fibrin along lines of closure
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20
Q

Causes of Myxomatous degeneration?

A
  1. Most primary cases are unknown

2. Marfan patients are at more risk

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21
Q

Cardiac defect associated with Maternal DM type I and II

A

Transposotion of the great vessels

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22
Q

Characteristics of Libman-Sacks Endocarditis?

A
  • Affects small to medium-sized inflammatory vegetations
  • Can be attached on either or both sides of valve leaflets
  • heal with scarring
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23
Q

How does a patent foramen ovale form?

A

Caused by the failure of the septum primum and secundum to fuse against the foramen ovale

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24
Q

The functional outcome of Transposition of the great vessels?

A

Separation of the systemic and pulmonary

- incompatible with life unless a VSD exists

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25
Chronic rheumatic heart disease is characterized by..
organization of the acute inflammation and subsequent scarring
26
Congenital Heart disease characterized by: | - Abnormal fixed opening in the atrial septum that allows unrestricted blood flow between atrial chambers
Atrial septal defect
27
Diagnostic features of Rheumatic Heart disease
1. Serological evidence of previous streptococcal infection (serum titers to one or more strep anitgens - Streptolysin O or DNAase) 2. Erythema Marginatum (rash) 3. Sydenham Chorea (Neurological disorder characterized by involuntary rapid movements)
28
What is the number 1 cause of cyanotic congenital heart disease?
Tetralogy of Fallot
29
Aortic coarctation with Right ventricular hypertrophy?
Infantile
30
T or F: Most VSDs close spontaneously in childhood
T
31
Causes/Factors contributing to congenital heart defects
1. Most are sporadic/ Unknown 2. Infection, rubella 3. Alcohol 4. Radition 5. Teratogenic drugs 6. Genetic (Down syndrom increases risk)
32
Cardiac defect associated with Rubella?
PDA
33
Types of degenerative valve changes
1. Calcifications (Cuspal or annular) 2. Alterations in the ECM 3. Changes w/ Metalloprteinases
34
What type of shunt is a patent ductus arteriousus (PDA)?
Left-to-right - the ductus remains open allowing blood to flow from the aorta (left) into the pulmonary artery (right) - contributes to congestive heart failure
35
Two types of Prosthetic valves
1. Mechanical 2. Bioprosthetic valve Both susceptible to infection and infection involves suture line
36
T or F Congenital valve abnormalities (i.e. Bicuspid aortic valve) are more likely to undergo pathological processes such as calcification and infection
T
37
What are Aschoff Bodies?
Aschoff Bodies - Myocardial inflammatory lesions consisting of a collection of macrophages called Anitschkow cells - found in the myocardium - about a week after infection
38
Systemic manifestations of Carcinoid heart disease?
Flushing, diarrhea, dermatitis and bronchospasm
39
What valve is involved with Infective endocarditis?
Tricuspid (especially in IV drugs users via staff Aureus)
40
In the infantile form of aortic coarctation, where does the narrowing occur in respect to the ductus arteriosus?
PROXIMAL
41
When do cardiac lesion from Carcinoid syndrome occur?
After there is a massive hepatic metastatic burden | - because the liver normally catabolizes circulating mediators before they can affect the heart
42
Failure of a heart valve to close completely is called?
Insufficiency | - allows regurgitation of blood
43
Which form of aortic coarctation typically has inferior rib notching? what causes it?
Adult - caused secondary to dilated intercostal collateral vessels which form as a way to bypass the coarctation and supply the descending aorta
44
Aortic coarctation with left ventricular hypertrophy?
Adult
45
What pathological process leads to mitral valve prolapse?
1. Myxomatous degeneration | 2. Marfans syndrome
46
Cardiac defect associated with del 22q
Tetralogy of Fallot -associated with DiGeorge
47
What is Degenerative valve disease?
pathological changes that affect the integrity of the valvular extracellular matrix (ECM)
48
Risk factors for Infective endocarditis
1. Pre-existing structural heart disease (Rheumatic Heart disease, Hypertrophic cardiomyopathy. sunaortic stenosis, ventricular aneurysm. 2. Degenerative valve disease 3. Cardiac interventions (prosthetic valve, pacemaker, or defibrillator) 4. Congenital Heart disease
49
In the adult form of aortic coarctation, where does the narrowing occur in respect to the ductus arteriosus
Post ductal
50
What type of congenital heart defect is more common, Left-to-right, or Right-to-Left?
Left-to-right (non-cyanotic)
51
Causes of Insufficiency?
1. intrinsic disease of the valve cusps 2. Disruption of the supporting structures w/o primary cuspal injury 3. abrupt (Chordal rupture) 4. Insidiously (Leaflet scarring and retraction)
52
Which genetic syndrome is associated with aortic coarctation?
Turner's syndrome (45 X/- )
53
Cause of Carcinoid Heart Disease?
Caused by the bioactive compounds (serotonin) released by carcinoid tumors
54
congenital defect associated with "machinery-like" murmer
PDA | continuous murmer - occurring during systole and diastole
55
Complications of Infective Endocarditis
1. Cardiac Failure (volume overload due to regurgitant lesions and infarcts) 2. Systemic infarcts (emboli) 3. Systemic abscess (infected emboli) 4. Aneurysms (infected emboli) 5. Renal failure (emboli and Immune-mediated glomerulonephritis
56
What is Rheumatic valvular disease?
Acute, immuno-mediated multisystem inflammatory disease (cardiac manifestation of Rheumatic fever) - occurs after Group A Beta-hemolytic Strepococcal infections!!!
57
Cardiac defect associated with Down syndrome?
septal defect
58
Name 5 Right-to-Left shunts (cyanotic shunts)
1. Truncus arteriosus 2. Transposition of the great vessels 3. Tricuspid Atresia 4. Tetralogy of Fallot 5. TAPVC
59
Complications of Rheumatic heart disease
1. Cardiac murmurs 2. Cardiac hypertrophy and dilation 3. CHF 4. Scarred Valves (more susceptible to infective endocarditis) 5. Arrhythmias (a-fib in the setting of mitral stenosis) 6. Thromboembolic complications due to atrial mural thrombi
60
What is aortic coarctation?
Narrowing or constricting of the aorta - form of obstructive congenital heart disease - affects men>women - 50% also have bicuspid valve
61
Most commonly affected valves in Rheumatic heart disease
Aortic and Mitotic | - really for any heart disease (except IV drug use)
62
Disease characterized by microbial invasion of the heart valves or mural endocardium often with DESTRUCTION of the underlying cardiac tissue -results in in bulky, friable vegitations composed of necrotic debris, thrombus, and organisms
Infective endocarditis - bacteria eat through the valves
63
Causative organisms of Infective endocarditis
1. S. Viridans (streptococci) 2. S. Aureus (Staphylococci) 3. Enterococci (the HACEK organisms) - Haemophilus parainfluenza - Haemophilus aphrophilus - Actinobacillus (Haemophilus) - Actinomyecetemcomitans - Cardiobacterium hominis - Eikenella species - Kingella species
64
What gross and microscopic findings will you find on the heart in carcinoid syndrome?
Gross: Distinctive glistening white intimal plaque-like thickening on he endocardial surfaces of the chambers and leaflets Micro: Mucopolysaccarides
65
What are predisposing conditions for Noninfectious vegitations?
1. Hypercoaguable states | 2. Mucinous Adenocarcinomas!!
66
Complications of PDA
1. Ischemia (commonly intestinal) 2. Irreversible pulmonary hypertension 3. Risk for endocarditis
67
Clinical signs of PDA
1. Pulses are bounding 2. Tachycardia 3. Tachypnea - helps get more gas exchange 4. Congested "wet" lungs
68
Clinical signs of Pulmonary hypertension of the newborn
1. meconium aspiration | 2. Holosystolic mumer along mid right sternum (Tricuspid regurg)
69
Which congenital defects cause systolic murmers?
1. Pulmonary Stenosis 2. Aortic Stenosis 3. Tricuspid Regurgitaion 4. Mitral Regurgitation 5. Ventricular Septal defect
70
Which congenital defects cause diastolic murmurs?
1. Aortic insufficiency 2. Pulmonary insufficiency 3. Mitral Stenosis 4. Tricuspid stenosis
71
Congenital defect associated with "egg on a string" heart?
Transposition of the great vessels
72
Murmur associated with mid systolic click
Mitral valve prolapse
73
Most common cardiac-cause of palpatations?
Mitral valve prolapse
74
Genetic mutation associated with tetralogy of fallot?
NOTCH2

Cardiology (15 decks)

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