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ICM Exam 3 Cardiology > Congenital Heart Defects - Bell > Flashcards

Congenital Heart Defects - Bell Flashcards

1
Q

Changes at birth:

  1. First breath
  2. Clamped placental vessel
  3. Others
A
  1. Decrease in pulmonary resistance
  2. Sys. vascular resistance increases
  3. FO closes​​
    1. PG dehydrogenase destoys PGE2 from placenta
    2. Decreased m coat of pulmonary vascular resistance over the next 6 weeks
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2
Q

Cyanosis can be ___ or ____

A

acrocyanosis, central cyanosis

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3
Q

_____ is a sign of serious abnormality

A

Central cyanosis

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4
Q

Difference in limbs that is cause for evaluation

A

3%

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5
Q

Central cyanosis ______ Hb

A

3-5g/dL desaturated

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6
Q

Abnormality cardiac physiology that causes hypoxia without hypercarbia

A

5 terrible T’s with PS

(TAVPR, Pulm atresia, Single ventricle)

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7
Q

Cardiac defects show this level even on 100% oxygen

A

Usually less than 50

PCO2 doest change (still low, normal level)

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8
Q

CHF sx in infants

A
  • tachypnea
  • poor feeding
  • tachycardia, diaphoresis
  • Hyperdynamic precordium, tachy/tachy
  • Hepatomegaly
  • Edema
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9
Q

Younger children with CHF may appear to have _______

A

gastroenteritis, with N/V/D

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10
Q

Older children with CHF may present with

A

exercise intolerance, cough, anorexia, fatigue

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11
Q

Pathophys. causes of chf

A
  1. decreased C.O.
  2. Increased SY tone and increased mineralocorticoids due to high RAAS
    1. Also Increased ANP, BNP, IGF1 ,GH
  3. Both lead to cardiac remodeling
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12
Q

4 physiologic mechanisms that reult in HF

A
  1. Increased fluid load to heart (Increased preload)
  2. Obstructed ventricular emptying (increased afterload)
  3. Decreased contractility
  4. Abnormal rhythms
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13
Q

Cyanotic lesions that lead to increased volume load

A

Decreased pulmonary blood flow

obstruction to pulm blood flow and R>L shunt

Tricuspid atresia, Single ventrical with pulmonary stenosis, tetrology

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14
Q

Cyanotic lesions with increased pressure load

A

Increased pulmonary blood flow

intracardiac mixing or abnormal ventricular-arterial connection

Transposition, TAPVR, TA, Common atria or ventricle

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15
Q

Top 3 Congenital HDs’

A

VSD (25-30)

ASD (Secundum) (6-8)

PDA (6-8)

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16
Q

___________ associated with VSD

A

NKx2.5, GATA4, TBX5

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17
Q

Two others assoc’d with VSD

A

22q11 deletion, Holt-Oram syndrome

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18
Q

Gender ratio of VSD

A

equal

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19
Q

4 types of VSD

A

Supracristal

Peri-membranous

Posterior

Muscular

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20
Q

Large VSD can progress to…(6)

A
  • CHF
  • Holosystolic murmur (2 weeks) with diastolic rumble at apex
  • Prominent L precurdium
  • Palpable Sternal lift
  • Systolic thrill
  • Apical Thrust
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21
Q

Small VSD’s…

A

close spontaneouslyl

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22
Q

Complications of large VSD

A

Growth fail, pulm infctns (untreated CHF)

Eisenmonger’s physiology

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23
Q

Long-term VSD risk

A

bacterial endocarditis

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24
Q

Eisenmonger’s physiology

A

Dilated pulmonary artery secondary to pulmonary HTN

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25
Q

VSD Treatment

A

Diuretics, ACEi, ARB, nitrate, milrinone (or CCB)

If refractory, pulmHTN, or A regurg = close surgically

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26
Q

Complications of Percutaneous VSD closure

A

late (1 week) AV block

hemolysis

sepsis

device embolization

arrhythmia

thrombosis

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27
Q

Atrial septal defect gender ratio

A

Female to male 3:1

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28
Q

___ is one third of the CHD detected in adults

A

ASD

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29
Q

____is most common defect in Holt Oram syndrome

A

ASD

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30
Q

TF’s involved in ASD

A

NKx,

TBX5, GATA4 (activate MYH6)

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31
Q

ASD associated with

A

PAPVR, MVP

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32
Q

Ausculation of ASD

A

First heart sound is loud (possible pulmonic ej. click)

Soft systolic ejection murumur at the 2nd interspace

fixed widely split second heart sound

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33
Q

ASD on ECG

A

RVH, R axis deviation

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34
Q

Echo in ASD

A

septal motion with increased RV will move anterior in systole rather than posterior in systole/anterior in diastole

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35
Q

____ may trigger ASD complications

A

pregnancy

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36
Q

___ rare with ASD

A

bact. endocarditis

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37
Q

Can delineate lesion and shunt in ASD

A

Cardiac cath

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38
Q

Surgical closure of ASD indicated with

A

2:1 shunt (regardless of sx)

39
Q

Most ASD closures done when?

A

1-5

40
Q

PDA female to male

A

2:1

41
Q

TF associated with PDA

A

TFAP2B (6p12)

neural crest TF associated with Char syndrome and PDA

42
Q

______ have high incidence of PDA

A

preterm infants (

43
Q

PDA - what arch artery?

A

VI pharyngeal arch artery

44
Q

PDA caused by either

A

Deficiency of the endothelial and musclar layer of vessel, regulatory proteins that direct fibrosis

OR

Another cardiac lesion that requires this to remain open

45
Q

Flow through PDA is initially..

A

in systole, then in both systole and diastole

46
Q

PDA presentation at birth

A

normal but with increased peripheral pulses

47
Q

Large PDA presentation

A

2-6 weeks will be signs of CHF

LV thrust

Continuous murmur at ULSB, radiating to the back

Hepatomegaly

Peripheral bounding pulse

48
Q

Small PDA will have normal _______

A

CXR and EKG

49
Q

PDA Tretment

A

surgical closure for ALL lesions

50
Q

Indomethacin is used when?

A

Premature infants with PDA

-> everyone else gets surgical closure

51
Q

Most common Coarc

A

juxtaductal

(below the origin of the subclavian artery at the ductus)

52
Q

Gender ratio of Coarc

A

males to female 2-5:1

53
Q

Coarc associated with

A

Turner’s

(bicuspid AV, VSD, PDA, Mitral stenosis or regurg, CoW aneurysms)

54
Q

LVOTO association

A

Coarc

55
Q

HTN in coarc due to

A

decreased renal perfusion

56
Q

Adult presentation of coarc

A

HTn

HA

epistaxis

dizziness

palpitation

57
Q

What makes the Diagnosis in Coarc

A

higher BP in arms with bounding pulse, while LE pulses distant

58
Q

Ausc. of coarc

A

thrill or systolic ejection click from bicuspid AV

May be systsolic murmur of AS at 3rd R interspace

OR

systolic murmur of collateral circulation over R and L sides of the chest

59
Q

BA esophagram in coarc

A

Displacement of the esophagus

60
Q

Unoperated coarc may cause

A

early death from UE HTN

Intracranial hemorrhage

Aneurysms in brain, descending aorta, collat. circ

Hypertensive encephalopathy

61
Q

PGE1 infusion for whom?

A

coarc

repair after

62
Q

____ is major problem in coarc

A

bacterial endocarditis

63
Q

____ stents may decrease the recoarctation rate

A

Endovascular stents

64
Q

Coarc need what?

A

SBE prophylaxis

65
Q

coarc complications

A

postcoarctecctomy synd.

HTN and abdominal pain from inability of mesenteric arteries to regulate BP with new perfusion

66
Q

Most common cyanotic HD diagnosed in adults

A

tetrology

67
Q

22q11 testing indicated for TOF if…

A

associated with absent pulmonic valve, arch abnormalities, or aorto-pulmonary collaerals

68
Q

Short TOF presentation

A

sleep and muscle weakness

69
Q

Prolonged TOF presentation

A

unconciousness and convulsions

70
Q

TOF older child presentation

A

Progressive cyanosis, dyspnea, cyanosis

Squatting episodese (helps compress LE arteries and increase aortic resistance so pulmonary flow is increased)

71
Q

Murmur in TOF

A

systolic ej. murmur of pulmonic stenosis

holosystolic murmur at LLSB of VSD

72
Q

CXR of TOF

A

Boot shaped heart

73
Q

TOF tx

A

PGE1, O2, hypoglycemia and anemia Tx

Corrective surgery

If underdev. pulm arteries, palliative procedures like Blalock-Taussig shunt or palliative balloon valvuloplasty

74
Q

Administer ___ if O2 doesn’t help with tet spell

A

Morphine

Causes vagal stimulation, increased PSy tone

75
Q

Other Tet spell treatments

A

Acidosis Tx

Phenylephrine

Propranolol

76
Q

If TOF patient is febrile before surgery, consider

A

brain abscess or endocarditis

77
Q

40-50% of TOF have

A

ventricular arrhythmias

78
Q

____________ occurs when aorta arises from RV and is anterior to the pulmonary artery

A

D transposition (Complete transposition)

79
Q

TGA requires

A

PDA and PFO

80
Q

TGA clinical presentation

A

Cyanotic

Loud single second heart sound

81
Q

CXR for TGA

A

Egg shaped wih narrow mediastinum, cardiomegaly,

increased pulmonary vascular markings if VSD present

82
Q

Mortality of untreated TGA

A

90% by 6 months

83
Q

Jatene =

A

Arerial switch procedure

performed within 1-2 weeks

84
Q

TGA treatment - After 2 weeks

A

Two stage switch can be completed

85
Q

TGA infants with VSD may have

A

switch and VSD closure after 2 weeks

because LV pressures are high with flow through VSD

86
Q

Arterial treatment for TGA

Morbidity?

A

Jatene switch

Morbidity: Supravalvular stenosis at reanastomosis sites, dilatation of aortic root with valcular insufficiency

87
Q

Atrial repair of TGA

Morbidity?

A

Atrial switch (Mustard/Sennig)

Morbidity: arrhythmias, baffle obstruction of flow

88
Q

LTGA

A

present in HF and cyanosis

89
Q

TGA gene

A

ZIC3

90
Q

Gene for PDA

A

TFAP2B

91
Q

Pulmonic stenosis gene

A

GATA4, ZIC3

92
Q

TOF gene

A

NKX, TX5, FOG2

93
Q

_______ can be normal in ped exam

A

early systolic vibratory ej. murmur

ICM Exam 3 Cardiology (8 decks)

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