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Cardiology > Valvular and Congenital heart Disease > Flashcards

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
Q

Chronic rheumatic heart disease is characterized by..

A

organization of the acute inflammation and subsequent scarring

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26
Q

Congenital Heart disease characterized by:

- Abnormal fixed opening in the atrial septum that allows unrestricted blood flow between atrial chambers

A

Atrial septal defect

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27
Q

Diagnostic features of Rheumatic Heart disease

A
  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)
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28
Q

What is the number 1 cause of cyanotic congenital heart disease?

A

Tetralogy of Fallot

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29
Q

Aortic coarctation with Right ventricular hypertrophy?

A

Infantile

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30
Q

T or F: Most VSDs close spontaneously in childhood

A

T

31
Q

Causes/Factors contributing to congenital heart defects

A
  1. Most are sporadic/ Unknown
  2. Infection, rubella
  3. Alcohol
  4. Radition
  5. Teratogenic drugs
  6. Genetic (Down syndrom increases risk)
32
Q

Cardiac defect associated with Rubella?

A

PDA

33
Q

Types of degenerative valve changes

A
  1. Calcifications (Cuspal or annular)
  2. Alterations in the ECM
  3. Changes w/ Metalloprteinases
34
Q

What type of shunt is a patent ductus arteriousus (PDA)?

A

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
Q

Two types of Prosthetic valves

A
  1. Mechanical
  2. Bioprosthetic valve

Both susceptible to infection and infection involves suture line

36
Q

T or F Congenital valve abnormalities (i.e. Bicuspid aortic valve) are more likely to undergo pathological processes such as calcification and infection

A

T

37
Q

What are Aschoff Bodies?

A

Aschoff Bodies - Myocardial inflammatory lesions consisting of a collection of macrophages called Anitschkow cells

  • found in the myocardium
  • about a week after infection
38
Q

Systemic manifestations of Carcinoid heart disease?

A

Flushing, diarrhea, dermatitis and bronchospasm

39
Q

What valve is involved with Infective endocarditis?

A

Tricuspid (especially in IV drugs users via staff Aureus)

40
Q

In the infantile form of aortic coarctation, where does the narrowing occur in respect to the ductus arteriosus?

A

PROXIMAL

41
Q

When do cardiac lesion from Carcinoid syndrome occur?

A

After there is a massive hepatic metastatic burden

- because the liver normally catabolizes circulating mediators before they can affect the heart

42
Q

Failure of a heart valve to close completely is called?

A

Insufficiency

- allows regurgitation of blood

43
Q

Which form of aortic coarctation typically has inferior rib notching? what causes it?

A

Adult

  • caused secondary to dilated intercostal collateral vessels which form as a way to bypass the coarctation and supply the descending aorta
44
Q

Aortic coarctation with left ventricular hypertrophy?

A

Adult

45
Q

What pathological process leads to mitral valve prolapse?

A
  1. Myxomatous degeneration

2. Marfans syndrome

46
Q

Cardiac defect associated with del 22q

A

Tetralogy of Fallot

-associated with DiGeorge

47
Q

What is Degenerative valve disease?

A

pathological changes that affect the integrity of the valvular extracellular matrix (ECM)

48
Q

Risk factors for Infective endocarditis

A
  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
Q

In the adult form of aortic coarctation, where does the narrowing occur in respect to the ductus arteriosus

A

Post ductal

50
Q

What type of congenital heart defect is more common, Left-to-right, or Right-to-Left?

A

Left-to-right (non-cyanotic)

51
Q

Causes of Insufficiency?

A
  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
Q

Which genetic syndrome is associated with aortic coarctation?

A

Turner’s syndrome (45 X/- )

53
Q

Cause of Carcinoid Heart Disease?

A

Caused by the bioactive compounds (serotonin) released by carcinoid tumors

54
Q

congenital defect associated with “machinery-like” murmer

A

PDA

continuous murmer - occurring during systole and diastole

55
Q

Complications of Infective Endocarditis

A
  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
Q

What is Rheumatic valvular disease?

A

Acute, immuno-mediated multisystem inflammatory disease (cardiac manifestation of Rheumatic fever)

  • occurs after Group A Beta-hemolytic Strepococcal infections!!!
57
Q

Cardiac defect associated with Down syndrome?

A

septal defect

58
Q

Name 5 Right-to-Left shunts (cyanotic shunts)

A
  1. Truncus arteriosus
  2. Transposition of the great vessels
  3. Tricuspid Atresia
  4. Tetralogy of Fallot
  5. TAPVC
59
Q

Complications of Rheumatic heart disease

A
  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
Q

What is aortic coarctation?

A

Narrowing or constricting of the aorta

  • form of obstructive congenital heart disease
  • affects men>women
  • 50% also have bicuspid valve
61
Q

Most commonly affected valves in Rheumatic heart disease

A

Aortic and Mitotic

- really for any heart disease (except IV drug use)

62
Q

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

A

Infective endocarditis

  • bacteria eat through the valves
63
Q

Causative organisms of Infective endocarditis

A
  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
Q

What gross and microscopic findings will you find on the heart in carcinoid syndrome?

A

Gross: Distinctive glistening white intimal plaque-like thickening on he endocardial surfaces of the chambers and leaflets

Micro: Mucopolysaccarides

65
Q

What are predisposing conditions for Noninfectious vegitations?

A
  1. Hypercoaguable states

2. Mucinous Adenocarcinomas!!

66
Q

Complications of PDA

A
  1. Ischemia (commonly intestinal)
  2. Irreversible pulmonary hypertension
  3. Risk for endocarditis
67
Q

Clinical signs of PDA

A
  1. Pulses are bounding
  2. Tachycardia
  3. Tachypnea - helps get more gas exchange
  4. Congested “wet” lungs
68
Q

Clinical signs of Pulmonary hypertension of the newborn

A
  1. meconium aspiration

2. Holosystolic mumer along mid right sternum (Tricuspid regurg)

69
Q

Which congenital defects cause systolic murmers?

A
  1. Pulmonary Stenosis
  2. Aortic Stenosis
  3. Tricuspid Regurgitaion
  4. Mitral Regurgitation
  5. Ventricular Septal defect
70
Q

Which congenital defects cause diastolic murmurs?

A
  1. Aortic insufficiency
  2. Pulmonary insufficiency
  3. Mitral Stenosis
  4. Tricuspid stenosis
71
Q

Congenital defect associated with “egg on a string” heart?

A

Transposition of the great vessels

72
Q

Murmur associated with mid systolic click

A

Mitral valve prolapse

73
Q

Most common cardiac-cause of palpatations?

A

Mitral valve prolapse

74
Q

Genetic mutation associated with tetralogy of fallot?

A

NOTCH2

Cardiology (15 decks)

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