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LMCC Study - Peds > Acyanotic Congenital Heart Disease > Flashcards

Acyanotic Congenital Heart Disease Flashcards

1
Q

Congenitial heart disease occurs in how many births per 1000?

A
  • 8/1000
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2
Q

What are the three pathophysiological groups that congenital heart disease can fall into?

A

1) Left-Right shunt
2) Right-Left shunt
3) Obstructive, stenotic lesions

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3
Q

Which pathophysiological groups can cause acyanotic heart disease?

A

1) Left-Right shunt (increased pulmonary blood flow)
- PDA
- VSD
- ASD
2) Obstructive lesions
- Aortic stenosis
- Pulmonary stenosis
- Coarctation of aorta

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4
Q

What is the most common congenital heart disease?

A
  • Ventricular septal defect

- Accounts for 25% of all

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5
Q

Large VSDs are typically asymptomatic at birth. Explain why. When will this be picked up?

A

Large VSDs:

  • Less resistance to flow so murmur may be absent
  • Pulmonary vascular resistant is high at birth resulting in less of a left to right shunt
  • First 6-8 weeks of life this pulmonary vascular resistance decreases and shunting increases
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6
Q

How would you describe the murmur associated with a small VSD?

A
  • Loud pansystolic murmur
  • usually loudest over L sternal border
  • May have Thrill
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7
Q

How would you describe the murmur associated with a large VSD?

A
  • Increased flow across the mitral valve

- Mid-diastolic murmur at the apex

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8
Q

What investigations can be done to look for VSD?

A

1) ECG
- Findings of L atrial and ventricular enlargement
2) CXR
- Cardiomegaly
- Enlarged L ventricle
- Pulmonary HTN may lead to R heart enlargement

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9
Q

How are VSDs managed?

A
  • 1/3 will close spontaneously (small more often)
    2) Surgical closure
    3) Initial tx for mod-sev VSD includes:
  • Diuretics +/- digoxin
  • Afterload reduction
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10
Q

What is the name of the most common type of atrial septal defect? Where is the defect?

A
  • Secundum defect

- Defect in region of the foramen ovale

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11
Q

What clinical findings are associated with a VSD?

A
  • Small: Assymptomatic

- Mod-severe: Pulmonary overcirculation, heart failure

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12
Q

What are the clinical manifestations of an atrial septal defect?

A
  • Rarely symptomatic
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13
Q

What may you see on ECG and CXR with Atrial septal defect?

A

1) ECG
- Right axis deviation
- Right ventricular enlargement
2) CXR
- Cardiomegaly
- Right atrial enlargement
- Prominant pulmonary artery

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14
Q

What is the treatment for a ASD?

A
  • Rarely indication

- If significant shunt still present around age 3 can be closed surgically or by cath lab

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15
Q

What is the ductus arteriosis?

A
  • A blood vessel in the developing fetus that connects the pulmonary artery to the descending aorta
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16
Q

What is the function of the ductus arteriosus?

A
  • Allows blood from the right ventricle to bypass the fetuses fluid filled lungs
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17
Q

What happens to the ductus arteriosus after birth?

A
  • It becomes the ligamentum arteriosum

- Ductus arteriosus typically closes within a few days after birth

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18
Q

What happens if the ductus arteriosis remains patent?

A
  • Left to Right shunting

- Higher pressure in aorta shunts blood (left to right) to the pulmonary artery resulting in higher pulmonary blood flow

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19
Q

What are the clinical manifestations of a PDA?

A
  • Can be asymptomatic
  • Larger shunts can produce symptoms of heart failure
  • Resp distress
  • poor feeding
  • irritability
  • poor growth
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20
Q

What physical exam findings might you see with a PDA?

A
  • Widened pulse pressure (due to runoff of blood into pulmonary circulation during diastole)
  • Continuous machine like murmur in L intraclavicular area (often heard well over L side of the back)
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21
Q

What might be seen on imaging studies in a patient with a PDA?

A

1) ECG
- Full pulmonary artery silhouette
- Increased pulmonary vascularity
2) CXR
- Normal or evidence of L ventricular hypertrophy (compensating for poor output)
3) Doppler Echo
- showing blood flow from Aorta to L pulmonary artery (L-R shunt)

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22
Q

What is the medical treatment for PDA?

A
  • NSAID (indomethacin I.v)

- Cannot use with renal failure, necrotizing enterocolitis, or overt bleeding.

23
Q

If NSAIDs fail or are contraindication what is your next step in managing a PDA?

A
  • Surgical occlusion
  • If PDA too small for occlusion, diuretics and ACE inhibitors can be used to treat congestive symptoms until it grows larger
24
Q

What is an endocardial cushon defect?

A
  • AKA: Atrioventricular canal defect

- Failure of the septum to fuse with endocardial cushion

25
Q

What are the symptoms of an endocardial cushion defect?

A
  • Symptoms of heart failure as pulmonary vascular resistance decreases
    • poor feeding
    • tachypnea
    • poor growth
    • irritability
  • Pulmonary HTN from increased pulmonary circulation
26
Q

Complete endocardial cushion defects are most commonly seen in children with this genetic condition?

A
  • Trisomy 21
27
Q

A complete endocardial cushion defect is typically diagnosed how?

A
  • Echocardiogram
  • Other things that may be seen:
    • enlargement of all chambers of heart and increased vascularity on CXR
  • L axis deviation, and combined ventricular enlargement on ECG
28
Q

How is an endocardial cushion defect managed?

A
  • Initial management with diuretics +/- digoxin

- Surgical repair is ultimately needed

29
Q

What is pulmonary stenosis?

A
  • Obstruction to blood flow from the right ventricle to the pulmonary artery
  • The stenosis can be valvular, subvalvular, or supravalvular
30
Q

What is valvular pulmonary stenosis?

A
  • Stenosis in the pulmonary valve
  • Can’t open wide enough limiting the flood flow from the right ventricle to the lungs
    Due to:
    • Thick leaflets
    • Narrow fusion of leaflets (leaves narrow hole in center)
31
Q

What is subvalvular pulmonary stenosis?

A
  • The muscle under the valve area is thickened which creates a smaller outflow tract from the right ventricle into the pulmonary artery
32
Q

What is supravalvular pulmonary stenosis?

A
  • Pulmonary artery, just above the pulmonary valve, is narrow
33
Q

What are the clinical manifestations of pulmonary stenosis

A

1) Mild: Asymptomatic
2) Mod-Severe:
- Tachypnea
- Fatigue/Fainting
- Tachycardia
- Swelling in feet, face, eyelids, and/or abdomen
- cyanosis in end stages

34
Q

True or False: Spontaneous closure of a PDA after a few weeks of age in a full term baby is common?

A
  • False uncommon after a few weeks of age in full term baby
  • High oxygen and decreasing levels of prostaglandins associated with fully developed lungs and distance from maternal prostaglandins should have closed it by a few weeks of age
35
Q

What helps to keep the ductus arteriosus open? What can help close it?

A

1) Open: Prostaglandin, low O2 environment

2) Close: High O2 environment, removal of prostaglandins (NSAIDS - indomethacin)

36
Q

What ECG and CXR findings may you expect in a patient with pulmonic stenosis?

A
  • Mild: None
  • CXR: No cardiomegaly but dilation of the main pulmonary artery may be seen
  • ECG: Right axis deviation and right ventricular hypertrophy
37
Q

What is the treatment for pulmonic stenosis?

A
  • Note that pulmonic stenosis typically is not progressive and does not worsen
  • Balloon valvuloplasty is usually sucessful
  • Otherwise surgical repair
38
Q

What is aortic stenosis?

A
  • Obstruction to blood flow from the left ventricle to the aorta
  • The stenosis can be valvular, subvalvular, or supravalvular
39
Q

describe the murmur associated with pulmonic stenosis?

A
  • systolic ejection murmur
  • Second left intercostal space
  • Radiation to back
40
Q

Describe the murmur associated with aortic stenosis?

A
  • Systolic ejection murmur
  • Second right intercostal space
  • Radiation to neck
41
Q

What are the clinical manifestations of aortic stenosis?

A
  • Mild to moderate : Asymptomatic
  • Severe: easy fatigue, exertional chest pain, syncope
  • Symptoms of heart failure when critical
42
Q

What can be seen on CXR, ECG and Echo with aortic stenosis?

A

CXR: LV hypertrophy, dilation of ascending aortic knob due to intrinstic aortopathy may be seen

ECG: LV hypertrophy

Echo: Site of stenosis, valve morphology, presence of LVH and can estimate pressure gradient

43
Q

Is aortic stenosis typically a progressive disease?

A
  • Yes, frequently progresses with growth and with age
44
Q

How is aortic stenosis managed?

A
  • Serial follow-up with echo
  • Balloon valvuloplasty usually first interventional procedure for severe stenosis
  • Surgical management is another option is previous methods insufficient
45
Q

What is coarctation of the aorta? Where does it typically occur?

A
  • A congenital condition where there is narrowing of the aorta
  • Typically occurs in the area where the ductus arteriosus inserts
46
Q

What other abnormalities are commonly seen in a patient with coarctation of the aorta?

A
  • Hypoplastic (small) aortic arch
  • Abnormal aortic valve
  • VSDs
47
Q

Is a patient with coarctation of the aorta affected by the patency of the ductus arteriosus? explain?

A
  • Yes (Sort of)
    Depends on two things:
    1) Degree of coarctation (how narrow is the vessel), the more severe the more likely that a patient DA is needed
    2) Location of coarctation, if coarctation is preductal (above the DA) then a patient DA allows additional blood to perfuse the lower extremities
48
Q

20% of patients with a 45 X,0 karyotype will have this congenital cardiac malformation? Also what is the genetic condition?

A
  • Coarctation of the aorta

- Turner syndrome

49
Q

True or False: Coarctation of the aorta occurs equally in male and female patients

A
  • False

- 2:1 male to female ratio

50
Q

What are the clinical manifestations of coarctation of the aorta?

A
  • Femoral pulses weaker and delayed compared to right radial
  • Blood pressure in lower extremities lower than upper
  • Poor feeding
  • Respiratory distress
  • Older children presenting are typically asymptomatic, may have leg discomfort with exertion *
51
Q

What is rib notching? What causes it and what is it associated with?

A
  • Notching of the upper ribs seen on CXR
  • Collateral intercostal arteries have increased blood volume causing them to erode the ribs
  • Seen in coarctation of the aorta in children over 8
52
Q

How would you manage an infant with coarctation of the aorta?

A

1) Prostaglandin E1 (keep ductus arteriosus open)
2) Inotropic medication or diuretics for BP control
3) Surgical repair ultimately needed

53
Q

Which ventrical is enlarged in an infant presenting with coartation of the aorta? What about in a young adult presenting with the same?

A

1) Infant: Right (right heart pumping in womb against narrow artery)
2) Adult: Left (left ventricle pumping against narrow artery)

LMCC Study - Peds (12 decks)

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