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Pediatrics > Lecture 5: Congenital Heart Defects > Flashcards

Lecture 5: Congenital Heart Defects Flashcards

1
Q

Acyanotic defects

A
  • ASD
  • VSD
  • PDA
  • PV Stenosis
  • Coarctation of aorta
  • Aortic stenosis
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2
Q

Cyanotic defects

A
  • Tetralogy of fallot
  • Tricuspid atresia
  • TGA
  • TAPVR
  • Hypoplastic left heart syndrome
  • Pulmonary atresia (not on PANCE)

Everything that starts with T + Pulmonary atresia and Hypo

Pulm atresia is not on PANCE

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3
Q

What kind of shunt do ASD, PDA, and VSD make?

A

Left to right shunt

Oxygenated to deoxygenated. More mild

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4
Q

What do acyanotic diseases eventually result in?

A
  • HF
  • Eisenmenger syndrome with exertion

Eisenmenger results in pulmonary arterial hypertension

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5
Q

What are the outflow obstruction congenital heart defects?

A
  • Pulmonary stenosis
  • Aortic stenosis
  • Coarctation of the aorta
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6
Q

How does HF tend to present in infants?

A
  • Poor feeding
  • Respiratory symptoms
  • Elevated JVP
  • Hepatomegaly
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7
Q

ASD S/S

A
  • Fixed, split S2 and pulmonic ejection murmur
  • Respiratory infections and failure to thrive in infants
  • Prior to age 40: palps, exercise intolerance, dyspnea, fatigue

Symptoms depend on size

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8
Q

What are the two ASD types and features?

A
  • Ostium secundum (MC), associated with fetal alcohol syndrome.
  • Ostium primum, associated with Down’s syndrome as a endocardial cushion defect

The first shall be last

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9
Q

Dx of an ASD

A
  • CXR showing R heart dilation and prominent pulmonary vascularity
  • TEE showing size and location
  • R Heart catheterization showing increased o2 sat in RA and PA.
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10
Q

Tx of an ASD

A
  • Wait and watch for small
  • Asymptomatic but large = can wait 1-3 yrs
  • Need closure if symptomatic or > 8mm

Sometimes it is better to wait since the heart will be bigger and the patient can tolerate anesthesia better.

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11
Q

Where is more blood pumped into for a VSD?

A

More blood into lungs and pulmonary artery

Higher risk of pulm HTN, arrhythmias, stroke

Rside is lower pressure, so extra fluid going towards it.

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12
Q

Which region typically is responsible for most VSDs?

A

Membranous, easier to fix and more asymptomatic

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13
Q

What are the 3 types of VSDs?

A
  1. Membranous, MC, easiest to fix
  2. Muscular (lower septum)
  3. Inlet (Posterior portion beneath the TV)
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14
Q

What is the MC congenital heart defect?

A

VSD

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15
Q

Image of VSD pathophysiology

A
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16
Q

S/S of a symptomatic VSD

A
  • Holosystolic murmur in lower left sternal border
  • Poor feeding, respiratory infections, thrill, diastolic rumble in mitral area
  • signs of CHF
  • Eventually, eisenmenger syndrome will occur
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17
Q

Best diagnostic imaging for VSD

A

Echo

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18
Q

What will EKG show usually for VSD?

A

LVH

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19
Q

Management of VSDs

A
  • Small ones close on their own
  • Diuretics + higher calorie intake
  • Surgery: for larger shunts by age 2 via patch closure ideally, or transcatheter closure
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20
Q

When is surgical closure indicated for VSD?

A
  • > 8mm with symptoms
  • Pulmonary HTN
  • Aortic insufficiency
  • LA/LV dilation

Essentially the same as an ASD

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21
Q

What does the PDA connect?

A

Pulmonary artery to aorta

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22
Q

When does a PDA normally close?

A

By week 1

Becoming the ligamentum arteriosum

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23
Q

What are the risk factors for PDA?

A
  • Female
  • Preemies
  • High altitude births (> 10k ft)
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24
Q

S/S of PDA

A
  • Holosystolic “machine-like” murmur on auscultation pathognomonic
  • Differential cyanosis
  • Widened PP
  • PMI displacement
  • Large eventually leads to HF and eisenmenger syndrome
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25
Q

What is differential cyanosis?

A

Cyanosis/clubbing of the lower extremities, seen in PDA.

Rest of the body is normal.

This + pulmonary HTN is potentially diagnostic per da google. Emailed mousatatt

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26
Q

EKG finding for PDA

A

LVH + LAE

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27
Q

Tx of PDA

A
  • Small: monitor
  • To close a PDA in a neonate over day 10, use PGE inhibitor
  • Symptomatic/large PDA: Digoxin & furosemide

Kinda similar to DCM tx

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28
Q

When is surgery indicated for PDA?

A

Failure of PGE inhibitor, done via surgical ligation

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29
Q

What are the 3 scenarios in which pulmonary stenosis presents congenitally?

A
  • MC: stenosis of the actual valve
  • Hypertrophy of the muscle below the valve
  • Hypertrophy of the muscle above the valve
30
Q

Pathognomonic finding for pulmonary stenosis

A

Schistocytes on CBC due to microangiopathic hemolytic anemia

31
Q

In severe pulmonary stenosis at birth, what can be given to keep the neonate oxygenated?

A

PGE to keep their PDA open as an alternative pathway

Severe PS will cause a R to L shunt via PFO at birth if obstructed

32
Q

S/S of pulmonary stenosis

A
  • Systolic ejection murmur that increases with inspiration
  • opening click louder with expiration
  • RV lift of palpation of precordium
33
Q

What might EKG and CXR show for pulmonary stenosis?

A
  • EKG: none to mild RVH
  • CXR: Dilation of main pulmonary artery and left PA
34
Q

What is the surgery for pulmonary stenosis?

A

Percutaneous balloon valvuloplasty

35
Q

What is coarctation of the aorta?

A

Narrowing of the aortic arch (usually proximal descending), backing blood flow up to the left ventricle.

36
Q

Although coarctation of the aorta is more common in males, what condition in females makes it more likely?

A

Turner syndrome

Monosomy X

37
Q

What is the leading cause of heart failure in neonates?

A

Coarc +/- VSD, ASD, or other cardiac abnormalities

38
Q

Why does BP increase in upper extremities for coarc but decreased BP in lower extremities? What is the danger of poor perfusion in the lower half of the body?

A
  • Blood flow increases prior to the narrowing.
  • Lower BP in lower extremities results in kidney hypoperfusion, ultimately causing secondary HTN.

Kidney hypoperfusion = RAAS activation = secondary HTN

39
Q

What is preductal coarctation MC associated with?

A
  • Turner syndrome
  • PDA
  • Presents as postductal until it worsens severely.

MC in infancy

40
Q

What structure is the marker for postductal coarctation and its common S/S?

A
  • Ligamentum arteriosum(aka ductus arteriosus)
  • Higher BP upstream of aorta, lower BP downstream
  • Often presents in adulthood.
41
Q

What are the two biggest risks from coarctation increasing upper body BP?

A
  • Berry aneurysm rupture in head
  • Aortic dissection
42
Q

How does coarctation of the aorta in an infant present?

A
  • Diamond shaped systolic
  • High-pitched crescendo murmur for diastolic
  • Lower extremity cyanosis
  • Absent/delayed femoral pulses, esp on the R side
  • Failure to thrive
  • Much higher BP in upper arms than lower body.

Coarc will not present on the left!!

43
Q

S/S of coarctation of the aorta in an adult

A
  • Secondary HTN in upper extremities, lower in lower extremities
  • CP, cold extremities, claudication on exertion
  • PMI palpable and sustained
  • Pulsations in intercostal spaces
  • Delayed/weak femoral pulses (check R!)
44
Q

CXR finding for coarctation of the aorta

A

3-sign rib notching

45
Q

How do you tx coarctation of the aorta?

A
  1. PGE to increase lower extremity flow
  2. Surgery via anastomosis
  3. Balloon angioplasty with possible stent
46
Q

What precaution should a child have if they have coarctation of their aorta?

A

Exercise testing is mandatory prior to participating in athletic activities

47
Q

What are the 3 types of aortic stenosis?

A
  • Valvular (75%)
  • Subvalvular (23%)
  • Supravalvular (2%)

MC in males

48
Q

What murmur is pathognomonic for Aortic Stenosis?

A

Crescendo-Decrescendo holosystolic murmur best heard at the upper right sternal border

Radiating to the carotids :)

Building up pressure to open it, click when it opens, then gets quieter

49
Q

How do you treat standard valvular AS?

A

Percutaneous balloon valvuloplasty

Surgery in other types or very high resting gradient

50
Q

What happens as AS progresses in a child?

A
  • Reduced exercise capacity
  • Predisposed to ventricular dysrhythmias
  • Avoid all isometric exercise (like planking)
51
Q

What level of deoxy Hgb is likely to have cyanosis?

A

3g/dl, which is usually 80-85% spo2

52
Q

What test differentiates cardiac and non cardiac causes of cyanosis?

A

Hyperoxia test

Greater than PaO2 of 250 = no structural cyanotic disease

Give neonate 100% O2

53
Q

What are the 5 T’s of cyanotic heart disease?

A
  • Truncus Arteriosus
  • TGA
  • Tricuspid atresia
  • Tetralogy of Fallot
  • TAPVR
54
Q

Tetralogy of Fallot components

A
  1. Pulmonary stenosis
  2. RVH
  3. Overriding Aorta
  4. VSD

PROVe

55
Q

What is the pathogonomic finding for tetralogy of fallot on CXR?

A

Boot shaped heart

56
Q

What is a tet spell?

A
  • Hypercyanotic episode
  • Anything that decreases SVR will leads to a worse R to L shunt
  • Bluish skin seen during crying/feeding

Lside of the heart pumps against SVR, so if SVR is low, L side can relax

57
Q

Management of a tet spell

A
  • Keep them calm
  • Give O2
  • IVF
  • Meds to improve pulmonary blood flow
  • Squat or flex knees to increase SVR
  • Morphine (Decrease agitation and RVOT obstruction)
  • Bicarbonate (metabolic acidosis causes pulmonary vasoconstriction)
  • Phenylpephrine (increases SVR)
  • BB (Decrease RVOT obstruction)

Increasing SVR will increase LV pressure, so the shunt will reverse back to L to R.

58
Q

How soon do ToF babies need surgery?

A

Prior to age 2, they need open heart surgery for a good prognosis

59
Q

What is TGA?

A
  • Aorta coming off RV
  • PA coming off LV

Forms two separate circuits, so blood will never be oxygenated

Parallel circulation occurs.

60
Q

What are the two types of TGA?

A
  • d-TGA: dextro/complete TGA where aorta is on the right.
  • l-TGA: levo/congenitally corrected TGA, aorta on left, ventricles and valves switched instead.

d-TGA is lethal, L-TGA is fine

61
Q

What are the two MC CCHD?

Critical congenital heart defect

A
  1. ToF
  2. d-TGA (males)
62
Q

What connection must be made in order for d-TGA to be viable?

A

ASD or PFO to allow mixing of blood.

63
Q

Risk factors for TGA

A
  • Diabetes
  • Rubella
  • Poor nutrition
  • Alcohol
  • > 40y
64
Q

Classic triad of CXR for d-TGA

A
  1. Egg on a string (heart)
  2. Lung congestion
  3. Cardiomegaly
65
Q

Initial tx for d-TGA

A

PGE to keep PDA open

66
Q

Surgery for d-TGA

A
  • Balloon atrial septostomy
  • Surgically switch great vessels
67
Q

What is hypoplastic left heart syndrome?

A
  • Poorly developed LV and ascending aorta
  • Valves may be narrow or absent
  • Ultimately leading to left sided HF and cardiogenic shock

CANNOT DO ANYTHING VIGOROUS

68
Q

What is required for an infant with hypoplastic left heart syndrome to survive?

A

An ASD or PDA, since their left side cannot sustain life.

69
Q

Initial tx for practically all cyanotic defects

A

PGE

Keeps PDA open so you can shunt

70
Q

Surgical protocol for HLHS

A
  1. Norwood (new aorta to RV and tube to RA) (1-2 wk old)
  2. Bidirectional Glenn (PA & SVC connection) (4-6 months)
  3. Fontan (PA & IVC connection) (2y)

3 stage surgery

71
Q

What are the 6 innocent murmurs of childhood?

A
  • Newborn murmur
  • Peripheral pulmonary artery stenosis (PPS)
  • Still’s murmur (MC)
  • Pulmonary ejection murmur
  • Venous hum
  • Neck/supraclavicular bruit
72
Q

What makes Still’s murmur louder?

A
  • Loud when supine, gone when inspiring or sitting
  • Loud with anemia and fever

Pediatrics (23 decks)

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