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EOR - Pediatrics (Alice copy + Seth's) > Congenital heart defects (CV) (Seth's Additional info) (10%) > Flashcards

Congenital heart defects (CV) (Seth's Additional info) (10%) Flashcards

Contains all CV obj except: rheumatic fever, kawasaki, and syncope

1
Q

MC congenital heart disease in childhood

A

Ventricular Septal Defect

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2
Q

Which congenital cardiac defect is associated with a “3 sign” on CXR? Boot-shaped heart on CXR?

A

3 sign = Coarctation of the aorta (notching)
Boot-shaped heart = TOF (d/t a prominent RV that looks like the heel of a boot)

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3
Q

What is a VSD and what does it lead to overall?

A

Hole in the ventricular septum leading to a left-to-right shunt

left to right shunt for mild/mod

high pressure to low pressure

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4
Q

While mild/moderate VSD is associated with a left to right shunt, large (unrestricted) VSD defects may lead to a ____ shunt, which is called _____.

A

Right to left shunt

Eisenmenger syndrome

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5
Q

What is the MC type of VSD?

A

Perimembranous

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6
Q

Where is the hole of a perimembranous VSD?

A

LV outflow tract near the tricuspid valve

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7
Q

which congenital heart defect is associated with a “swiss cheese” pattern?

A

VSD muscular type

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8
Q

Where is an inlet VSD located? What is it aka?

A

posterior to the septal leaflet of the tricuspid valve

aka posterior VSD

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9
Q

Where is an outlet VSD located? What is it aka?

A

beneath the pulmonic valve, may have aortic valve insufficiency

aka supracristal VSD

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10
Q

Small VSD is typically asymptomatic (may have mild symptoms), but a moderate VSD is associated with these symptoms

A

Excessive sweating or fatigue especially during feeds
lack of adequate growth (not getting enough blood)
frequent respiratory infxs (due to congested pulmonary vasculature

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11
Q

what type of VSD is associated with no pressure difference between the ventricles?

A

Large (unrestricted)

further develops into right-to-left shunt = Eisenmenger syndrome

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12
Q

describe the murmur associated with VSD

A

High-pitched harsh holosystolic murmur best heard @ lower left sternal border

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13
Q

what part of the PE often differs a small vs larger VSD?

A

small is louder and often has a palpable thrill

diastolic rumble at MV sometimes

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14
Q

Echo dx VSD showing size and extent
EKG often shows LVH because of left to right shunt
Sometimes EKG shows RVH + LVH in more severe disease, and this is called ______.

A

Katz-Wachtel phenomenon

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15
Q

you choose to observe a VSD because it is small, but it must close by ____

A

12 months

otherwise surgery

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16
Q

Surgery for VSD and when to do it

A

Patch closure

symptomatic infants or uncontrolled CHF, growth delay, recurrent respiratory infections, or no closure by 12 months

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17
Q

2nd MC congenital heart disease

A

Atrial septal defect (ASD)

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18
Q

What fetal heart component is a feature of an ASD?

A

Associated with a PFO

foramen ovale allows blood to flow from the RA to the LA

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19
Q

Because there is a PFO associated with ASD, what type of shunt develops

A

left to right

acyanotic heart defect

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20
Q

MC type of ASD and the others

A

Ostium secundum

there is also ostium primum, sinus venosis, and coronary sinus

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21
Q

how does the age of first noticing symptoms differ in ASD vs VSD

A

ASD typically develops symptoms much later in life (like 3rd decade) as opposed to VSD (3rd week)

the atrium is not as big and it is easier to compensate early on

just as a rule of thumb, but any age can have symptoms

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22
Q

How do the symptoms of ASD differ in infants and young children vs adolescence and adults?

A

Infants & young children: recurrent respiratory infx, failure to thrive, DOE
Adolescents & young adults: exertional dyspnea, easy fatigability, palpitations, atrial
arrhythmias, syncope, heart failure

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23
Q

One of the main worries of ASD is the development of ____

A

paradoxical emboli

stroke from venous clots

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24
Q

Explain the murmur associated with ASD

A

Systolic ejection crescendo-decrescendo flow murmur @ pulmonic area (LUSB – 2nd or 3rd intercostal space)
* Wide, fixed split S2 that does not vary with respirations, loud S1 & hyperdynamic right ventricle

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25
Q

definitive diagnosis of ASD and VSD

A

Cardiac cath

rarely indicated though

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26
Q

small ASD less than < () may be observed and often close by ()

A

<5mm
12 months (like VSD)

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27
Q

Symptomatic treatment of ASD

A

Diuretics
ACEI
Digoxin

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28
Q

What is the surgical correction for ASD and when is it typically done?

A

1cm+/symptomatic (between 2-4 y/o)→perQ transcatheter closure v. surgical intervention

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29
Q

A patent ductus arteriosus (PDA) allows persistent communication between the ____ & ______ after birth

A

Persistent communication between the descending thoracic aorta & main pulmonary artery after birth

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30
Q

t/f, like ASD and VSD, PDA is associated with a left to right shunt

A

True

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31
Q

Use of ___ keeps the ductus arteriosus open

A

prostaglandin E1

continued production of this leads to a PDA

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32
Q

What gender is MC for PDA?

A

female 2x risk

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33
Q

Like VSD and ASD, most PDAs are asymptomatic, but infants may develop ___ but the characteristic difference in severe disease is ___

A

poor feeding
weight loss
Lower respiratory tract infections
Pulm congestion
Endocarditis
Eisenmenger syndrome
Cyanotic lower extremities

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34
Q

Murmur of PDA

A
  • Continuous machine-like or “to and fro” murmur loudest @ pulmonic area (LUSB – 2nd
    intercostal space)
  • Wide pulse pressure (bounding peripheral pulses) with low DB, loud S2

P = pulmonic

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35
Q

Best test to dx PDA

A

ECHOOOOO

like everything

cardiac cath is definitive but rarely needed

36
Q

ECG and CXR findings of PDA

A

ECG: LVH, left atrial enlargement
CXR: normal or cardiomegaly

37
Q

Treatment of PDA

A

NSAIDs

IV indomethacin or ibuprofen

inhibits PGE1

38
Q

MCC of sudden cardiac death in young athletes in the US

A

Hypertrophic cardiomyopathy

HCM

39
Q

Inheritance pattern of HCM

A

Autosomal dominant

Gotta screen FHx in athletes

40
Q
A
41
Q

Overall, what worsens the murmur of HCM?

A

Anything that causes decreased preload

42
Q

Affect of squatting vs standing/valsava for HCM murmur

A
  1. Squatting decreases the murmur
  2. Standing/valsava increases the murmur
43
Q

Why does exercise lead to a louder murmur and worse obstruction with HCM?

A

Higher demand increases contractility

anytime your body requires more effort to pump blood = worse murmur and more dangerous

44
Q

What does dehydration do to HCM murmur?

A

louder murmur

less preload and more effort to pump blood

45
Q

What drug (1) and class (1) are associated with increased contractility of the heart and therefore a worsened HCM murmur?

A
  1. Digoxin
  2. Beta antagonists

Makes sense why BBs would be a treatment as a result!

46
Q

What drug worsens the murmur of HCM by decreasing AFTERload

A

Amyl nitrate

47
Q

Most common presenting symptom of HCM

and some others

A

Dyspnea on exertion

fatigue, angina, dizziness, etc

48
Q

What arrythmia is most associated with sudden death with extreme exertion and HCM

A

V fib

49
Q

Murmur of HCM
() pitched () murmur
() (systolic/diastolic)
(cresendo/decrendo/cresendo)
loudest at ()
extra heart sound () with () lift

A

Medium-pitched harsh mid-systolic crescendo-decrescendo murmur heard @ left sternal border often with a loud S4 gallop w/ apical lift.

50
Q

Like all murmurs, you should get an echo with HCM, which will show ()
EKG shows ()

A

Echo = asymmetric ventricular wall thickness (especially septal thickness)
EKG: LVH

51
Q

1st line medical mng of HCM

A

BBs to lessen contractility of the heart

Early detection is KEY

52
Q

Your young patient still has HCM that is not controlled with a BB, so you consider ___.

A

Myomectomy

remove the hypertrophy

53
Q

The cardiologists deems that your patient is not a candidate for a myomectomy for HCM refractory to a BB, so the cardiologist may consider this procedure () or this invasive prevention () for syncope or sudden cardiac arrest

A

Alcohol septal ablation
ICD

54
Q

What is the pathophys / description of coarctation of the aorta

A

Congenital narrowing of the aortic lumen at the distal arch &/ descending aorta

55
Q

Coarctation of the aorta is MC in ___
PDA is MC in ___

gender/sex

A

Coarc = male
PDA = female

think CAM (CoArc Male)
think PDF (PDFemale)

56
Q

50% of patients with Coarctation of the aorta also have this cardiac abnormality

A

Bicuspid aortic valve

COA
BOA

57
Q

What is the overall difference between adult type and infantile type coarctation of the aorta

A

Adult = postductal
Infantile = preductal

classified by WHERE the narrowing occurs

58
Q

Explain the anatomic abnormality associated with POSTductal coarctation of the aorta

adult type

A

narrowing occurs distal to the ductus arteriosus

59
Q

Explain the anatomic abnormality associated with PREductal coarctation of the aorta

infantile type

A

narrowing occurs proximal to the ductus arteriosus

60
Q

what is the function of the ductus arteriosus

A

a short blood vessel that connects the pulmonary artery to the aorta in a fetus

normally closes after birth

61
Q

Neonatal presentation of coarctation of the aorta is similar to most noncyanotic heart defects and is associated with ().

A

FTT
poor feeding

62
Q

2 buzz word symptoms/presentations of coarcation of the aorta

not talking about the murmur

A
  1. Bilateral claudication
  2. UE HTN with LE hypotension/diminished pulses

also see DOE and syncope

63
Q

Explain the murmur associated with Coarcation of the aorta.
() (systolic/diastolic) ()
(cresendo/decrendo/continuous)
radiation to ()
loudest at ()

A

Late systolic ejection murmur/continuous murmur radiating to the left back, left
scapula or chest, heard in the aortic area

64
Q

T/F, unlike most other murmurs, the diagnositc test of choice for Coarctation of the aorta is actually a CXR instead of an echo

A

FALSEEEEEE

echo is the confirmatory test

ALWAYS GET AN ECHOOOOOOO

65
Q

Echo and EKG finding associated with Coarctation of the aorta

A

Echo = narrowing of aortic lumen
EKG = LVH (has to work harder)

66
Q

CXR finding of coartaction of the aorta

A
  1. 3 sign (aortic notch looks like a 3)
  2. posterior rib notching (d/t increased intercostal artery collateral flow)
67
Q

What is the gold standard diagnostic test for coarctation of the aorta

A

Angiography

very invasive though

makes sense

68
Q

Treatment of coarctation of the aorta

A

Corrective surgery or transcatheter-based intervention (balloon angioplasty) by 2-4 yo (emergent if dangerous s/s)

69
Q

Most patients receive ___ pre-op for coarctation of the aorta to stabilize prior to surg

A

Prostaglandin E1 (Alprostadil)

maintains a patent ductus arteriosus, reducing symptoms @ improves lower extremity blood flow

70
Q

What is the life-expectancy and cause of death(s) associated with untreated coarctation of the aorta?

A

Most adults die by 50 y/o d/t aortic rupture, CVA, or aortic dissection

71
Q

You should r/o coarctation of the aorta in a young, healthy adult with ___

A

HTN

72
Q

MC cyanotic congenital heart disease

A

TOF

73
Q

What is the shunt associated with TOF and what causes is

A

Right to left

d/t pulmonary stenosis

74
Q

What is the genetic abnormality sometimes associated with TOF

A

chromosome 22 deletion

Think tet = twenty-two

75
Q

Four abnormalities associated with TOF

A

PROV
1. Pulmonary stenosis (RV outflow obstruction)
2. RVH
3. Overriding aorta
4. VSD

76
Q

MC associated symptom with TOF

A

Cyanosis (blue-baby syndrome) especially with crying/feeding/increased energy requirement

can see exertional dyspnea and progressive cyanosis in older patients

77
Q

How to relieve a tet spell in an infant vs child

A

Infant = flex knees to chest
Child = squatting

kinda like HCM

both increase preload and allow the heart to receive more blood, therefore decreasing right-to-left shunt

78
Q

Explain the murmur associated with TOF
() (systolic/diastolic)
loudest at ()

A

harsh systolic murmur @ left mid to upper sternal border (VSD), right ventricular heave (RVH)

79
Q

All tests of choices for congenital heart defects in these objectives involve

A

ECHOOOO

also for TOF

80
Q

EKG finding of TOF

A

RVH
RAE

sometimes increased QRS = risk for sudden death

81
Q

CXR of TOF and why

A

Boot-shaped heart

d/t prominent right ventricle (RV almost looks like the heel of a boot)

82
Q

Treatment of choice for TOF and when it should be done

A

Surgical repair

ideally in first 4-12 months of life

83
Q

t/f, PGE1 is used prior to surgery for TOF in order to maintain the PDA and allow blood to flow from the pulmonary artery to the aorta

A

TRUE

also do prophylatic ABX for endocarditis

84
Q

Complications of surgery of TOF

A

arrhythmias, pulmonary regurgitation, residual outflow obstruction, heart failure

85
Q

MCC of death of TOF

A

Sudden cardiac death and HF :(

EOR - Pediatrics (Alice copy + Seth's) (22 decks)

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