Embryology and CLD/CHD Flashcards

1
Q

3rd aortic arch develops into…

A

L common carotid and part of internal and external carotid

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2
Q

4th aortic arch develops into…

A
R = subclavian
L = arch of aorta
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3
Q

6th aortic arch develops into…

A

part of pulmonary aa and ligamentum arteriosum

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4
Q

Double aortic arch

A

Right dorsal aorta remains intact below 7th intersegmental artery > forms around esophagus and trachea > pt often present with difficulty swallowing > may present with signs of intrathoracic compression of trachea (i.e. wheezing)

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5
Q

Subclavian sling

A

Right dorsal aorta remains intact below 7th intersegmental artery but disappears more superiorly > doesn’t compress esophagus and trachea as severely > R subclavian comes off left side and crosses posterior to esophagus and trachea

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6
Q

Sinus venosus becomes

A

Smooth wall of RA

Coronary sinus

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7
Q

Primordial atrium becomes

A

R and L auricles and LA

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8
Q

Primordial ventricle becomes

A

LV

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9
Q

Bulbus cordis becomes…
Proximal 1/3
Conus cordis
Truncus arteriosus

A

Muscular RV
Smooth outflow of RV and LV
ascending aorta and pulmonary trunk

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10
Q

Aortic sac becomes…

A

Aorta and pulmonary a.

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11
Q

Endocardial cushions become…

A

Atrial septum, membranous IVS, AV and semilunar valves

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12
Q

Early pulmonary vv become…

A

Smooth wall of LA

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13
Q

Conotruncal ridges become…

A

Septum b/w aorta and pulmonary a.

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14
Q

RDS MCC of…

A

respiratory failure in newborns

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15
Q

Most common CHD lesion

A

VSD

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16
Q

Most common congenital anomaly of the upper airway

A

Laryngomalacia

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17
Q

Structural differences between infant and adult lungs

A
  • Infant airways not as complex
  • Type I alveoli not completely flat > gas exchange less efficient
  • Alveolar pores (Kuhn) and canals of Lambert not well developed
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18
Q

What phase of the fetal period is surfactant starting to be made?

A

Saccular ~26 weeks

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19
Q

Ground glass x-ray

A

RDS

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20
Q

Boot shaped heart x-ray

A

TOF

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21
Q

Egg on a string x-ray

A

TGA

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22
Q

Snowman x-ray

A

TAPVR

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23
Q

Tetralogy of Fallot Criteria

A
  • Pulmonary stenosis
  • RV hypertrophy
  • Overriding aorta
  • VSD
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24
Q

Associated with hypocalcemia, VSD

A

Truncus arteriosus

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25
Q

Associated with strong UE pulses and weak LE pulses

A

Coarctation of aorta

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26
Q

Why are infants prone to RDS?

A
  • Lower # alveoli > decreased SA, lower elastin and collagen, bronchi more collapsible
  • Ribs horizontal, not ossified, less muscle > much more compliant chest wall, much less outward recoil
27
Q

How does MAS lead to disruption of NML lung function?

A
  • Airway obstruction
  • Surfactant dysfunction
  • Direct chemical pneumonitis
  • Resultant persistent pulmonary HTN
28
Q

Septum transversum

A

Splits intraembryonic cavity into pericardial and peritoneal cavities > makes up part of diaphragm

29
Q

Space that connects pericardial and peritoneal cavities

A

Pericardioperitoneal canals

30
Q

Pleuroperitoneal folds

A

Connect with septum transversum to close pericardioperitoneal canals and form diaphragm

31
Q

CDH

A

Abdominal viscera herniate into thorax

  • Mostly L sided d/t liver
  • Ipsilateral lung hypoplastic
32
Q

Most common hernia

A

Esophageal

33
Q

CDH clinical presentation

A

Antenatally - large can be dx
Neonatal - severe resp distress
Infancy/childhood - chronic resp symptoms, feeding difficulty, can be asymptomatic and noted incidentally

34
Q

CDH Management

A

Antenatally - surgery
Neonatal - supportive care > vent, ECMO
Childhood - surgical closure

35
Q

Eventration of diaphragm

A

Similar to hernia, but no hole just weakened diaphragm

-correct by pushing back into thorax > still not functional but not impinging on lung

36
Q

Pleuropericardial fold

A

Folds anterior to lungs and posterior to heart

  • As lungs move anterior > bring folds with them > eventually form fibrous tissue of heart and parietal pleura
  • Contains phrenic n. and cardinal vv
37
Q

How is the pediatric upper airway different from adults?

A
  • Smaller diameter
  • More anterior
  • Floppier epiglottis
  • Larger tongue
  • Larger occiput
  • Narrowest portion of airway is cricoid
38
Q

Buccopharyngeal membrane

A

Thins and ruptures posteriorly to create an opening from nasal cavity into pharynx > choana

39
Q

Choanal atresia

A

Failure in resorption of bucco-pharyngeal membrane

-suspect if unable to pass catheter through nares

40
Q

Pierre Robin

A

Micrognathis
Glossoptosis
Cleft palate
Macroglossia

41
Q

Laryngeal cleft

A

Split like opening b/w posterior larynx and esophagus > zipper
Recurrent aspiration or cyanotic spells with feeding
Developmental failure of tracheoesophageal septum

42
Q

Laryngomalacia

A

Floppy larynx > poor epiglottis cartilaginous development or abnormal muscular tone

  • Typically present with inspiratory stridor w/i first few weeks
  • Supine worse than prone
43
Q

Subglottic stenosis

A
  • Narrowing of subglottic airway at cricoid
  • Congenital results from incomplete recanalization of laryngotracheal tube
  • severe = resp distress; milder = stridor; recurrent croup in older kids
44
Q

Tracheoesophageal fistula

A

Connection between esophagus and trachea maintained

45
Q

Tracheomalacia

A
  • Weakness in tracheal wall
  • recurrent wheezing, barking cough with illness or activity
  • recurrent lower airway infections d/t impaired airway clearance
  • coarse, low-pitch, monophonic wheeze
  • beta-agonist worse
46
Q

Bronchomalacia

A
  • Monophonic wheezing
  • Typically not symptomatic past school age kids
  • Impaired mucous clearance > recurrent PNA
  • beta-agonist worsen
47
Q

Bronchial isomerism

A
  • increased risk of ciliary dyskinesia
  • associated with CHD
  • heterotaxi syndrome
48
Q

Tracheal bronchus

A
  • associated with impaired airway clearance

- increased risk of other associated anomalies

49
Q

Bronchial stenosis/atresia

A

Can both cause and result from recurrent infection

50
Q

Bronchogenic cysts

A
  • Abnormal budding of ventral foregut endoderm
  • Most mediastinal
  • Often asymptomatic
  • typically resected d/t risk of malignancy
51
Q

Cystic adenomatoid malformation

A
  • defect in development of terminal bronchioles leading to structure that resembles cystic collection of small airways
  • may cause polyhydraminos or even hydrops fetalis
52
Q

Congenital large hyperlucent lobe

A

Massive overinflation of one or more lobes > postnatally following initiation of respiration (some sort of malacia leaves bronchi to collapse when baby tries to exhale after their first inhale)
-MC in LUL

53
Q

ASD

A
  • Ostium secundum (more common, more sporadic)

- primum, or sinus venosus (less common, more associated with other defects)

54
Q

ASD/VSD presentation

A
  • Small often asymptomatic
  • Murmur may be heard
  • Large cause L-R shunt leading to R atrial enlargement, increased R pressure, and eventually HF if untreated
55
Q

ASD/VSD Tx

A
  • Small often close (for VSD location matters > membranous not as likely to close on their own)
  • Small to medium > transcatheter device
  • Large/complication > surgical patch
56
Q

AV Canal defect / endocardial cushion defect

A
  • Partial = severe low ASD or high VSD with AV valves affected but present
  • Complete = AV valves not developed and all 4 chambers contiguous (trisomy 21 association)
57
Q

LVOTO

A
  • Milder = aortic stenosis

- Severe = HLHS (underdeveloped LV > cyanotic newborn; can be caused by mitral atresia)

58
Q

RVOTO

A
  • Milder = pulmonary stenosis
  • Severe = TOF
  • Very severe = HRHS (underdeveloped RV)
59
Q

Tricuspid atresia

A
  • PFO or ASD

- VSD

60
Q

Truncus arteriosis

A

Often associated with other cardiac anomalies, DiGeorge syndrome (hypocalcemia)

61
Q

Vitelline veins

A

Deoxygenated blood from yolk sac

62
Q

Cardinal vv

A

Deoxygenated blood from embryo

63
Q

Umbilical vv

A

Oxygenated blood from placenta

64
Q

TAPVR

A

Pulmonary vv carry oxygenated blood to SVC > mixed blood