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Cardiology > Congenital Heart Defects > Flashcards

Congenital Heart Defects Flashcards

1
Q

What are tet spells?

A

This is when there is increased cyanosis due to crying. Crying increases pulmonary resistance → increase in RV pressure → increased blood flow from R to L ventricle via VSD shunting → worsens cyanosis.

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2
Q

What is infantile coarctation of aorta associated with?

A

Turner syndrome

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3
Q

When does cyanosis manifest in R→L shunts and why?

A

Early childhood or even at birth because they cause early hypoxia. This is beacuse deoxygenated blood from the left side of the heart crosses over to the right side of the heart, entering the systemic circulation.

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4
Q

What is the heart sound herad in congenital aortic stenosis?

A

Crescendo-decrescendo murmur loudest at the base

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5
Q

What type of heard sound best characterizes atrial septal defect? Where can it be heard?

A

Loud S1 with a wide, fixed split S2; best heard in upper left sternal border

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6
Q

What causes closure of ductus arteriosus?

A

1) Increase in O2 (from respiration)
2) Decrease in prostaglandins (from placental separation)

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7
Q

In what cases may prostaglandin infusion to maintain patency of ductus arteriosus be warranted? (2)

A

1) Infants with severe coarctation of aorta- patency of ductus arteriosus maintains perfusion to the lower extremities.
2) Infants with transposition of great arteries- initial tx can be maintaining patent DA for mixing of blood to keep them alive until defintive surgical correction is made.

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8
Q

What are the sx of pulmonic stenosis?

A

Dyspnea on exertion

Exercise intolerance

Signs and sx of right sided HF (leg edema, abdominal fullness)

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9
Q

What does a “step up” in oxygen saturation on catheterization between SVC/IVC to RA indicate?

A

ASD- blood coming from SVC or IVC is deoxygenated but if there is a sudden increase in O2 content in RA, the oxygen has to be coming from the left atrium via ASD.

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10
Q

How do babies with persistent truncus arteriosus present?

A

Cyanotic

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11
Q

How does the heart respond in ASD after birth?

A

In ASD, blood goes from left to right. The right side of the heart isn’t used to all the volume and dilates as a response (eccentric hypertrophy). In addition, pulmonary HTN results because the right ventricle can’t pump all the fluid it receives.

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12
Q

What type of shunts results from high pulmonary venous resistance and low systemic vascular resistance? What can this do to the shunt?

A

R to L shunts; this will increase shunting and worsen hypoxia

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13
Q

Common congenital heart disease leading to L to R shunt (3)

A

1) VSD
2) ASD
3) PDA

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14
Q

What are the sx of congenital aortic stenosis in infants?

A

In infants, if stenosis is bad, can present as poor feeding, tachycardia, tachypnea, failure to thrive

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15
Q

why does foramen ovale close after birth?

A

At birth, infant cries (inflates lungs), decreasing resistance to pulmonary vasculature. As a result, there is an increase in left atrial pressure vs. right atrial pressure, leading to closure.

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16
Q

What is unique in children with down syndrome in regards to heart rate?

A

They usually have a low baseline heart rate

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17
Q

What changes occur in the heart in congenital aortic stenosis?

A

Due to increased pressure by LV (since LVP must increase to pump blood across the valve of aorta), LV hypertrophies (concentric hypertrophy)

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18
Q

What type of defects are common in children with down syndrome?

A

Endocardial cushion defect with VSD, ASD (Esp. ostium primum type), or AV septal defects as well as Tetralogy of Fallot

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19
Q

What type of cyanosis (early vs. late) does Tetrology of Fallot present with?

A

Early cyanosis

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20
Q

What other condition besides aortic stenosis is associated with bicuspid aortic valve?

A

Coarctation of aorta

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21
Q

Which chamber of heart is affected most initially by VSD and why?

A

While LA, LV, RV, and pulmonary circulation are all affected, initially, increased blood return to the LV causes volume overload. This leads to dilitation of the LV.

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22
Q

What step in cardiac development forms the primitive heart tube?

A

Fusion of endocardial heart tubes

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23
Q

What causes tetralogy of fallot?

A

Anterosuperior displacement of infundibular portion of IV septum, which creates a subvalvular pulmonic stenosis (stenosis of RV outflow tract)

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24
Q

What are the two types of ASDs? Which of them are more common?

A

1) Ostium secundum (most common)
2) Ostium primum

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25
Q

What is the jugular venous pressure curve finding in pulmonic stenosis?

A

Prominant “a wave”

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26
Q

What causes the heart sound in ASD?

A

Heart sound is loud S1 with a wide, fixed split S2. This is because the RV has to pump more blood during systole due to increased blood flow from high pressure LA ⇒ low pressure RA, delaying closing of the pulmonic valve both during inspiration + expiration (fixed). The delaying of closing of pulmonic valve compared to aortic valve cause split S2.

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27
Q

What changes to the heart occur due to pulmonic stenosis?

A

Cocnentric hypertrophy of RV due to increased RVP needed to pass blood across stenosis.

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28
Q

What is the difference between patent foramen ovale and ASD?

A

ASD is a pathological defect while patent foramen ovale is a normal variant.

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29
Q

How does infantile coarctation of aorta commonly present as?

A

Lower extremity cyanosis (differential cyanosis)

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30
Q

What heart sounds are heard in pulmonic stenosis?

A

Pulmonic valve click (high pithced click) that follows S1 followed by late peaking crescendo-decrscendo systolic murmur at left upper sternal border

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31
Q

What condition is associated with “notching of ribs” on x-ray and why?

A

Adult coarctation of aorta; this leads to increased collateral circulation over intercostal arteries to get to the descending aorta, which enlarges them, leading to notching of the ribs.

32
Q

What is the most common congenital cardiac defect?

A

Ventricular septal defect

33
Q

What are the similarities/ differences in presentation between congenital aortic stenosis caused by bicuspid aortic valve in adults vs. degenerative/calcific aortic stenosis in people with a normal aortic valve?

A

The sx are the same regardless of their etiology. Adults who have congenital AS vs. calcific AS include general aortic stenosis sx including fatigue, exertional dyspnea, angina pectoris, and syncope.

The difference is the age at which they present. Patients with bicuspic aortic valve (congenital AS) present earlier (40-60) while calcific aortic stenosis usually presents around 70-80.

34
Q

How does ostium primum ASD occur?

A

Normally, septum primum grows towards the endocardial cushions of the AV canal. Ostium primum ASD occurs when there is incomplete growth of septum primum to the endocardial cushions.

35
Q

Where in the heart can pulmonic stenosis be seen?

A

Valve itself (90%), within RV, or pulmonary artery

36
Q

What type of shunt is caused by persistent truncus arteriosus and why?

A

A right to left shunt; this is because one large vessel leaves both right and left ventricles. As a result, deoxygenated blood from right side of the heart and oxygenated blood from left side of the heart mix in the vessel.

37
Q

What infection is associated with PDA?

A

Congenital rubella

38
Q

What are the sx of VSD?

A

Pts with small VSDs typically remain symptom free while pts with large VSDs have early heart failure sx (tachypnea, poor feeding, failure to thrive, freqeuent lower respiratory tracti nfections).

39
Q

Why is correction of original defect contraindicated once Eisenmenger has developed?

A

Because it relieves right ventricular pressure. With correction, it has to pump against pulmonary HTN, leading to right heart failure.

40
Q

Physical exam findings of tetralogy of fallot

A

1) Cyanosis
2) Clubbing (for chronic)
3) Pulmonic systolic ejection murmur

41
Q

What chamber(s) of the heart is/are affected by PDA?

A

LA and LV will dilitate because it results in volume overload (sees more volume).

42
Q

What condition is associated with congenital rubella?

A

PDA

43
Q

What is persistent truncus arteriosus caused by?

A

Abnormal neural crest migration , which leads to incomplete fusion of the aorticopulmonary septum and failure of the truncus arteriosus to divide.

44
Q

What congenital condition is associated with maternal diabetes?

A

Transposition of great arteries

45
Q

What is a late manifestation of VSD?

A

Pulmonary HTN and Eisenmenger’s syndrome

46
Q

What are the sx of PDA?

A

Small PDAs are asympomatic. Those with large L to R shunts develop early congestive heart failure with tachycardia, poor feeding, slow growth, and recurrent respiratory tract infections.

47
Q

What are the common congenital heart diseases leading to R⇒L shunts?

A

1) Truncus arteriosus (1 vessel)
2) Transposition of great vessels (2 vessels switched)
3) Tricuspid atresia (Tri=3)
4) Tetralogy of Fallot (Tetra=4)
5) Total anomalous pulmonary venous return (TAPVR)

48
Q

What condition is associated with a boot shaped herat on x-ray?

A

Tetraloy of Fallot because this leads to RVH

49
Q

Why do kids with Tetrology of Fallot learn to crouch down?

A

During a tet spell in which there is increased cyanosis, crouching down dincreases systemic vascular resistance → increased LVP → lessens effect of R→L shunt, which decreases cyanosis.

50
Q

What is ostium secundum ASD and how is it formed?

A

It is a shunt that ccurs when the septum secundum fails to grow fully, unable to cover the ostium secundum.

51
Q

What does CXR of congenital aortic stenosis show?

A

Enlarged left ventricle with dilated ascending aorta (When valve is tight, aorta responds by dilating)

52
Q

What type of murmur is heard with ventricular septal defect and where on the chest can this be heard?

A

Harsh holosystolic murmur; can be heard at left lower sternal border

53
Q

What type of murmur is heard in ASD and why?

A

Systolic ejection murmur- due to increased blood flow across the pulmonary valve due to L→R shunt.

54
Q

How does adult coarctation present?

A

HTN in upper ex and hypotesion with weak pulses in lower extremities

55
Q

What medication can tx PDA?

A

COX inhibitors such as NSAIDs (e.g. indomethacin)

56
Q

Which ASD occurs as an isolated defect, and which is associated with other cardiac defects?

A

Ostium secundum occurs as an isolated defect, while ostium primum is associated with other cardiac defects.

57
Q

What is the tx for Tetrology of Fallot?

A

Surgical repair– VSD patch closure and RV outflow tract reconstruction

58
Q

What are some signs associated with Eisenmenger’s syndrome?

A

Late cyanosis, clubbing, polycythemia

59
Q

Why is closure of ASD contraindicated if Eisenmenger has developed?

A

Closure is contraindicated bc ASD serves as a pressure release for RV. If ASD is closed, RV has to pump against pulmonary HTN (increased afterload), which will lead to right sided heart failure.

60
Q

What is anomalous coronaries and what is the most lethal?

A

Anomalous coronaries is when coronary artery comes off somewhere other than where it should come out of. For instance, LCA should come off the right cusp while RCA should come off right cusp of aorta.

The most lethal is when left main comes off right cusp and it courses between pulmonary artery and aorta. This is associated with sudden death because when pt exercises, pulmonary artery and aorta gets bigger, which squeezes the left main. Always fixed with bypass surgery.

61
Q

What are the 4 congenital abnormalities associated with Tetrology of Fallot?

A

1) Pulmonic stenosis
2) RVH
3) Overriding aorta
4) Ventricular septal defect

(PROVe)

62
Q

How does transposition of grat vessels present and why?

A

Cyanosis at birth. This condition is incompatible with life.

63
Q

What condition usually causes congenital aortic stenosis in adults?

A

Bicuspid aortic valves. The sx present during adulthood because the valves become progressively stenotic over many years due to calcification.

64
Q

What is unique about pulmonic valve clicks compared to other right sided heart murmurs?

A

Usually, right sided heart murmurs increase in intensity with inspiration because there is more blood flow to the right side. However, in pulmonic valve clicks decrease in intensity with inspiration because as more blood volume is brought into right side of the heart, it’s already stretching the pulmonic valve. As a result, when pulmonic valve opens, snapping motion is less pronounced.

65
Q

What is the defect in transposition of great vessels?

A

Aorta originates from RV while pulmonar artery originates from LV. This leads to two parellel circulations, in which deoxygenated blood from systemic circulation that enter right side of the herat is pumped immediately back out into the periphery by the aorta which originates from RV.

66
Q

What conditino is assocaited with adult coarctation?

A

Bicuspid aortic valve

67
Q

What is Eisenmenger’s syndrome?

A

Condition in which a left to right shunt reverses to become a right to left shunt in pressence of progressive pulmonary HTN due to increased pulmonary circulation.

68
Q

What does cardiac looping of primitive heart tube establish?

A

Left to right polarity

69
Q

Describe the process of atrial septation.

A

1) Septum primum grows towards endocardial cushions in AV canal, narrowing foramen (ostium) primum.
2) As septum primum grows, septations occur in the middle of septum primum, forming foramen secundum. This maintains the right to left shunt in fetus. Septum primum grows toward endocardial cushion and foramen primum disappears.
3) Septum secundum grows right next to septum primum, covering foramen secundum.
4) A one way valve (foramen ovale) is created, maintaining right to left shunt.

70
Q

Why is foramen ovale a one way shunt?

A

In fetal circulation, pressure on right side of the herat is higher than the left.

71
Q

What condition is associated with a continuous machine-like murmur? Where can this be heard?

A

PDA; can be heard over the left infraclavicular region

72
Q

Where do majority of ventricular septal defects occur (muscular or membranous)?

A

Membranous interventricular septum

73
Q

What determines the severity of tetrology of Fallot?

A

Degree of pulmonic stenosis

74
Q

In which direction does blood flow after birth in PDA relative to aorta and pulmonary artery?

A

Blood flows from aorta to pulmonary artery via the PDA because after birth, the systemic pressure increases so the aortic pressure > pulmonary circulation pressure.

75
Q

Why do pts with uncorrected ASD or patent foramen ovale have increased risk of stroke?

A

Due to paradoxical embolism (embolism of venous origin)

76
Q

How is transposition of great vessels managed initially vs. long-term?

A

Initially, IV prostagladins are given to maintain patency of PDA. This allows oxygenated blood in pulmonary trunk and deoxygenated blood from aorta to mix so that some oxygenated blood can go into the aorta for systemic circulation.

Long term treatment involves surgical correction of the transposition.

77
Q

What determines the degree of caynosis in Tetrology of Fallot and why?

A

Pulmonic stenosis. If pulmonic stenosis is tight, there will be more R→L shunting and more blood will go into the overriding aorta. If it’s loose, some deoxygenated blood can still go into the pulmonary trunk and less will go into aorta.

Cardiology (21 decks)

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