Cardiac Embryology and congenital heart disease III Flashcards

1
Q

Define

tetralogy of Fallot

A

cyanotic heart disease

1) RV outflow tract obstruction
2) RVH
3) dextraposition of aorta (aorta overrides VSD)
4) VSD

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2
Q

monology of Fallot

A

anterior and superior deviation of infundibular portion of ventricular septum

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3
Q

most common cyanotic defect

A

tetralogy of fallot

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4
Q

physiology of Tetralogy of fallot

A

1) VSD is large so RV and LV pressures equal)

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5
Q

magnitude of pulmonary blood flow in tetralogy of fallot determined by (4) things

A

1) source of pulm blood flow
2) severity of RV outflow obstruction
3) balanced RV and LV pressure
4) ductus arteriosus

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6
Q

source of pulm blood flow

A

1) antegrade RV output to pulm arteries

2) ducts arteriosus flow

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7
Q

if outflow obstruction is severe, most PBF derived from ___

A

ductus arteriosus

size of ducts primary determinant of PBF magnitude

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8
Q

What happens in RV outflow obstruction

A

1) narrowing of infundibular region

2) stenosis of pulm valve

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9
Q

what determines R–> L shunt with RV outflow obstruction

A

if RV outflow resistance HIGHER THAN SYSTEMIC VASCULAR RESISTANCE –> cyanosis

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10
Q

what determines L –> R shunt with RV outflow obstruction

A

if RV outflow resistance LESS THAN SYSTEMIC VASCULAR RESISTANCE –> no cyanosis

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11
Q

which shunt has cyanosis

A

R–> L shunt

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12
Q

which shunt occurs if RV outflow resistance greater than systemic vascular resistance

A

R–> L shunt

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13
Q

what are tet spells

A

hypoxic or hypercyanotic spells

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14
Q

when do tet spells occur

A

2-6 y/o

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15
Q

Different mechanisms of Tet spells

A

1) infundibular spasm –> decr PBF
2) decr venous return to heart –> decr RV pressure –> decr PBF
3) decr SV –> incr R to L shunt –> decr PBF

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16
Q

what can precipitate tet spells

A

1) prolonged crying

2 anemia

3) dehydration

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17
Q

exam of tet spells

A

1) BLUE
2) decr murmur intensity
3) altered consciousness/seizures

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18
Q

treatment of tet spells

A

INCREASE PULM BLOOD FLOW

1) knee chest position
2) phenylephrine
3) morphine for sedation
4) volume expansion with IV fluids

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19
Q

prevention of tet spells

A

beta blockers (propranolol) –> decr infundibular obstruction

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20
Q

what does clinical presentation of tetralogy of fallot depend on (3)

A

1) size of VSD
2) severity of RV outflow obstruction
3) SVR level

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21
Q

how does tetralogy of fallot present

A

BLUE BABY WITH LOUD MURMUR

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22
Q

HOW CAN CYANOSIS IN tetralogy of fallot worsen?

A

as ductus arteriosus closes

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23
Q

if a patient with tetralogy of fallot had severe RV outflow tract obstruction how do you treat

A

pt may have ductal dependent pulm blood flow

need prostaglandins or early surgical repair

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24
Q

how to diagnosis tetralogy of fallot

A

1) tachycardic and cyanotic if blue tet
2) diaphoretic and tachypneic if pink tet
3) precordial impulse displaced to left lower sternal border –> RV dominance

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25
Q

murmur of tetralogy of fallot

A

2-3/6 short systolic murmur of pulmonary stenosis

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26
Q

ecg of tetralogy of fallot

A

Right axis deviation

RVH

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27
Q

prevention of tetralogy of fallot

A

elective surgical repair at 2-4 month before tet spell risk inca

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28
Q

progression of tetralogy of fallot

if no intervention

A

1) if RV outflow tract obstruction severe and pt has ductal dependent pulm blood flow
- -> DEATH AT TIME OF DUCTAL CLOSURE w/o intervention

29
Q

progression of tetralogy of fallot

if yes intevention in adulthood

A

1) cyanotic
2) clubbing of fingers
3) poorly develop enamel/teeth
4) incr bleeding
5) decr exercise tolerance (squat with exercise)
6) arrhythmia

30
Q

why do tetralogy of fallot patients squat

A

to incr SVR

and incr pulm blood flow

31
Q

known complication of unrepaired cyanotic congenital heart disease

A

cerebral abscess

after 1.5-2 y/o

32
Q

symptoms of cerebal abscesses

A

persistent unexplained fever

behavioral changes

33
Q

define coarctation of aorta

A

narrowing of aortic lumen

34
Q

theories of coarctation of aorta

A

1) extension of ductal tissue into aortic arch
1) disturbance of subclavian artery migration
1) altered blood flow in fetus –> decr blood thru isthmus

35
Q

15% of ____ patients have coarctation

A

TURNER’S SYNDROME

36
Q

15% OF TURNER’S PATIENTS HAVE ___

A

coarctation

37
Q

anatomy of coarctation

A

1) intraluminal projection of a “shelf” from lateral, posterior, anterior wall of aorta in area of ductus arteriosus

38
Q

consequences of poor descending aorta perfusion

A

1) decr blood to bowel –> necrotizing enterocolitis
2) decr blood to leg muscles –> claudication with exercise
3) decr blood to kidneys –> incr RAAS activ, rebound hypertension after repair

39
Q

clinical presentation of coarctation

general

A

1) asymptomatic as newborn (PDA allows adeq post-coarct flow)

2) as ductus closes –> tachypnea, diaphoresis, poor feeding
- -> cardiac failure

40
Q

clinical presentation of coarctation

infancy

A

lack femoral pulses

cardiac failure/shock

41
Q

clinical presentation of coarctation

childhood

A

asymptomatic
or

systemic HTN
intermittent lower extrem claudication and HA

42
Q

clinical presentation of coarctation

adult

A

systemic HTN

43
Q

diagnosis of coarctation

A

1) tachycardia
2) BP differential btwn Lower and Upper extrem
3) rales +/- hepatomegaly

44
Q

heart sounds with coarctation

A

1) accentuated S2 or S3
2) soft systolic murmur
3) systolic click over apex if assoc bicuspid aortic valve

45
Q

diagnosis of coarctation

A

1) absent or weak femoral pulses
2) ecg
3) CXR

46
Q

ecg with coarctation

infants

A

Right axis deviation

RVH

47
Q

ecg with coarctation

children

A

incr in LV forces

48
Q

ecg with coarctation

adults

A

ST depression

T wave flattening/inversion

49
Q

CXR with coarctation

A

normal after birth

signs of cardiac failure

1) cardiomegaly
2) prominent pulm arterial markings
3) pulm edema

50
Q

echo with coarctation

A

site and severity of obstruction

presence of ductus (difficult to dx with large ductus

assoc bicuspid
VSD
aortic/subaortic senosis

51
Q

management of coarctation

infants

A

1) maintain on prostaglandins until surgery
2) end to end anastomosis surgical repair
3) risk of recoarctation and aneurysm long term

52
Q

management with coarctation

young children

A

balloon angioplasty vs surgery

53
Q

management with coarctation

adults

A

surgery vs stent placement

54
Q

progression of coarctation

A

1) develop collaterals with marked obstruction
- weak delayed femoral pulses compared to arm pulses

2) BP differential variable
- usually arm BP more increased
- young children slightly lower leg BP

55
Q

consequences of coarctation (cause of death)

A

1) heart failure
2) aortic rupture/dissection
3) infective endocarditis
4) cerebral hemorrhage

56
Q

monology of Fallot

“simplified tetrology of Fallot”

A

anterior and superior deviation of infundibular portion of ventricular septum

–> forms subpulmonary outflow tract

57
Q

what causes a pink baby

A

blood left to right across VSD

depends on amt of blood thru RV outflow tract

58
Q

what causes a blue baby

A

blood right to left across VSD

59
Q

when do tet spells occur

A

2-6 months

60
Q

Different mechanisms of Tet spells

A

1) infundibular spasm –> incr blood across VSD –> decr PBF
2) decr venous return to heart –> decr RV pressure –> decr PBF
3) decr SV –> incr R to L shunt –> decr PBF

61
Q

what can precipitate tet spells

A

1) prolonged crying

2 anemia

3) dehydration

62
Q

exam of tet spells

A

1) BLUE
2) decr murmur intensity (b/c less blood across pulm valve and go across VSD)
3) altered consciousness/seizures

63
Q

treatment of tet spells

A

INCREASE PULM BLOOD FLOW

1) knee chest position (incr SVR)
2) phenylephrine (incr SVR)
3) morphine for sedation
4) volume expansion with IV fluids

64
Q

prevention of tet spells

A

beta blockers (propranolol) –> decr infundibular obstruction

65
Q

what does clinical presentation of tetralogy of fallot depend on (3)

A

1) size of VSD
2) severity of RV outflow obstruction
3) SVR level

66
Q

how does tetralogy of fallot present

A

BLUE BABY WITH LOUD MURMUR

67
Q

HOW CAN CYANOSIS IN tetralogy of fallot worsen?

A

as ductus arteriosus closes

68
Q

if a patient with tetralogy of fallot had severe RV outflow tract obstruction how do you treat

A

pt may have ductal dependent pulm blood flow

need prostaglandins or early surgical repair

69
Q

what is the 3 sign on CXR

when do you see?

why not in infants

A

SEEN IN ADULTS
1) dilated aortic knob

2) coarctation
3) post-stenotic dilation

rib notching from dilated intercostal arteries

not in infants b/c thymus so large