Congenital Heart Disease II Flashcards

1
Q

When do symptoms of ASD usually present?

A

not in infancy- takes a while to develop symptoms

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1
Q

What are the long term risks of untreated ASDs?

A

development of pulm vascular disease atrial arrhythmias HF

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1
Q

____ is the most common cyanotic heart defect and accounts for 15% of all congenital heart defects.

A

Tetralogy of Fallot

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2
Q

In Tetrology of Fallot, the RV and LV pressures are _____.

A

equal

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3
Q

Why isn’t a murmur going away a good thing?

A

could be a large VSD with equalizing R and L pressures elevation in PVR

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4
Q

When can a VSD be a right to L shunt?

A

with pulmonary stenosis

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5
Q

What are the exam findings with a small VSD?

A

precordial activity normal normal 2nd heart sound 2-4/6 early systolic murmur NO diastolic rumble

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6
Q

DX? 2-3/6 systolic ejection murmur at L upper sternal border with possible diastolic rumble widely split 2nd heart sound

A

ASD

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6
Q

Turner’s Syndrome is associated with ______.

A

coarctation of the aorta

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6
Q

Where do coarctations of the aorta occur?

A

in the descending aorta opposite of the ductus arteriosus or lower

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6
Q

DX? 1. tachycardia 2. difference in upper extremity and lower extremity BP 3. pulmonary rales 4. hepatomegaly 5. S2 and S3 gallop 6. soft systolic murmur

A

Coarctation of the aorta

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7
Q

What happens with pulmonary vascular disease at high altitudes?

A

it’s more common and occurs at an earlier age

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8
Q

10% of VSDs are which type?

A

muscular VSDs

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8
Q

What are the exam findings with a large VSD?

A

active precordium loud 2nd heart sound 2-3/6 harsh, holosystolic murmur loudest at the LLSB diastolic murmur

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8
Q

How is VSD treated?

A

diuretics surgical close if muscular

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8
Q

How does Tetrology of Fallot present?

A
  1. blue baby 2. loud murmur
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9
Q

What are some of the s/s of VSD?

A

respiratory distress diaphoresis (esp with feeding) failure to thrive tachypnea

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10
Q

Dx? hypoxic or hypercyanotic episode, usually in the 2-6th month of life can be life threatening unk mechanism

A

Tet spell (Tetralogy of Fallot)

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11
Q

What are the steps of atrial septal embryology?

A
  1. septum primum closes 2. apoptosis of the middle primum cells to form the osteum secundum 3. septum secundum closes to enclose the foramen ovale
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12
Q

What is a fixed split S2 and what is it diagnostic of?

A

when the S2 is split at any point in the respiration cycle; ASD

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12
Q

Why isn’t ASD caught at infancy?

A

there’s usually not a loud murmur or symptoms until later in life

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13
Q

What are the phys exam findings in a large ASD?

A
  1. increased respiratory rate 2. sweating with feeding 3. hepatomegaly 4. 2-3/6 systolic ejection murmur at L upper sternal border with possible diastolic rumble 5. split 2nd heart sound
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14
Q

What is the normal pressure within the LV?

A

120

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15
Q

How is VSD treated?

A

diuretics surgical close if muscular

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16
Q

What will happen in untreated Tetrology of Fallot?

A

death at the time of ductus arteriosus closure (if severe RVOT obstruction) OR cyanosis, clubbing, poor dental enamel, bleeding tendencies, squat with exercise, arrhythmias AND MAYBE cerebral abscesses

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16
Q

Why does a baby develop symptoms of coarctation 2 days to 2 weeks post delivery after being fine at birth?

A

the ductus arteriosus closes and worsens the problem

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18
Q

How does a secundum ASD happen?

A

the osteum secundum was too large or the septum secundum inadequately develops

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19
Q

When should you decide to surgically correct a VSD?

A
  1. pulmonary vascular changes 2. persistent symptoms, failure to thrive 3. 2a complications (aortic insuff, double chambered RV)
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19
Q

What is coarctation of the aorta?

A

a narrowing of the aortic lumen

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20
Q

What does the left endocardial cushion become?

A

posterior leaflet of the mitral valve

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20
Q

How does Tetralogy of Fallot happen?

A

abnormal development of the conal crests resulting in an infundibular septum

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20
Q

What will the phys exam findings be in Coarctation of the aorta?

A
  1. tachycardia 2. difference in upper extremity and lower extremity BP 3. pulmonary rales 4. hepatomegaly 5. S2 and S3 gallop 6. soft systolic murmur
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21
Q

Why would you want to keep the ductus arteriosus open in a Tetrology of Fallot pt?

A

to help offset the effects of the RV outflow tract obstruction

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22
Q

Why do atrial arrhythmias occur in ASD patients?

A

atrial enlargement over time

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23
Q

How does Tetralogy of Fallot happen?

A

abnormal development of the conal crests resulting in an infundibular septum that is displaced anteriorly, superiorly, and rightward

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24
Q

What are the phys exam findings in Tetrology of Fallot?

A
  1. tachycardic and cyanotic OR diaphoretic and tachypnic 2. precordial impulse at the L lower sternal border 3. 2-3/6 short systolic murmur of pulm stenosis
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25
Q

What causes the murmur in ASD?

A
  1. systolic ejection murmur = excessive blood flow across the pulmonic valve 2. diastolic rumble = excessive blood flow in diastole across the tricuspid valve
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26
Q

When should you decide to surgically correct a VSD?

A
  1. pulmonary vascular changes 2. persistent symptoms, failure to thrive 3. 2a complications (aortic insuff, double chambered RV)
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27
Q

How are Tet spells prevented (after the 1st one)?

A

beta blockers

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28
Q

DX? 2-3/6 systolic ejection murmur at L upper sternal border with possible diastolic rumble widely split 2nd heart sound

A

ASD

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29
Q

What are the components of S2 and what do they represent?

A

P2 = pulmonic valve closure A2 = atrial valve closure

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31
Q

What are the long term risks of untreated ASDs?

A

development of pulm vascular disease atrial arrhythmias HF

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32
Q

What is a swiss cheese septum?

A

multiple muscular VSDs

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33
Q

The murmur in a VSD is caused by?

A

blood flow across the defect

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34
Q

What is the most common congenital heart defect? How common is it?

A

VSD; 20% of all

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35
Q

What is considered a large ASD?

A

diameter is greater than or equal to the mitral valve size

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36
Q

How common are ASDs?

A

7-8% of all congenital heart defects

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37
Q

In Tetrology of Fallot, the RV and LV pressures are _____.

A

equal

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37
Q

How will a child present with coarctation of the aorta?

A

systemic HTN, intermittent lower extremity claudication, headaches

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38
Q

Why isn’t a louder murmur bad in VSD?

A

the VSD could be closing the PVR is low

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39
Q

How do you treat a Tet spell?

A
  1. knee-chest position 2. phenylephrine (increase BP) 3. morphine (sedative) 4. IV fluids
40
Q

Turner’s Syndrome is associated with ______.

A

coarctation of the aorta

41
Q

What is the tx for ASD?

A
  1. diuretics 2. percutaneous device closure
42
Q

What syndrome is associated with coarctation of the aorta?

A

Turner’s Syndrome

43
Q

How does a secundum ASD happen?

A

the osteum secundum was too large or the septum secundum inadequately develops

43
Q

What is the tx for coarctation of the aorta?

A

surgery for infants and young children; balloon angioplasty with stent for adolescents/adults

44
Q

What are the 4 components of Tetralogy of Fallot?

A
  1. RV outflow tract obstruction 2. RVH 3. dextraposition of the aorta 4. VSD
45
Q

What is a tet spell?

A

hypoxic or hypercyanotic episode, usually in the 2-6th month of life can be life threatening unk mechanism

46
Q

What will happen in untreated Tetrology of Fallot?

A

death at the time of ductus arteriosus closure (if severe RVOT obstruction) OR cyanosis, clubbing, poor dental enamel, bleeding tendencies, squat with exercise, arrhythmias AND MAYBE cerebral abscesses

47
Q

The murmur in a VSD is caused by?

A

blood flow across the defect

48
Q

What will happen in untreated Tetrology of Fallot?

A

death at the time of ductus arteriosus closure (if severe RVOT obstruction) OR cyanosis, clubbing, poor dental enamel, bleeding tendencies, squat with exercise, arrhythmias

50
Q

What does ASD stand for?

A

atrial septal defect

50
Q

What causes the murmur in ASD?

A
  1. systolic ejection murmur = excessive blood flow across the pulmonic valve 2. diastolic rumble = excessive blood flow in diastole across the tricuspid valve
51
Q

What does the left endocardial cushion become?

A

posterior leaflet of the mitral valve

52
Q

What are some of the s/s of VSD?

A

respiratory distress diaphoresis (esp with feeding) failure to thrive tachypnea

52
Q

What is an RV outflow obstruction in Tetralogy of Fallot?

A

narrowing of the infundibular region stenosis of the pulmonary valve

54
Q

How does VSD present?

A

asymptomatic until pulmonary vascular resistance falls

55
Q

What does a deficiency or lack of the membranous portion of the interventricular septum cause?

A

a perimembranous VSD

56
Q

DX? 1. RV outflow tract obstruction 2. RVH 3. dextraposition of the aorta 4. VSD

A

Tetralogy of Fallot

56
Q

How can coarcation of the aorta present?

A
  1. necrotizing enterocolitis 2. leg muscle pain w/ exercise 3. increased RAAS activation
57
Q

What will the xray findings be in Coarcation of the Aorta?

A
  1. 3 sign along the L edge of the heart 2. rib notching from dilated intercostal a’s
58
Q

What definitively diagnoses VSD?

A

echo

59
Q

The atrial septum fuses with the endocardial cushions, which are involved in ______.

A

ventricular septation

60
Q

What is the result of abnormal development of the conal crests resulting in an infundibular septum that is displaced anteriorly, superiorly, and rightward?

A

Tetralogy of Fallot

62
Q

What is the tx for ASD?

A
  1. diuretics 2. percutaneous device closure
64
Q

Large, untreated VSDs can cause?

A
  1. Eisenmenger’s Syndrome
65
Q

The murmur of an ASD is NOT related to?

A

blood flowing across the defect

67
Q

What are the characteristics of Eisenmenger’s Syndrome?

A
  1. muscularization of the Pulm arterioles 2. pulmonary HTN 3. increased RV pressure 4. shunt reverses to R to L 5. no treatment other than transplant
68
Q

What is the result of abnormal development of the conal crests resulting in an infundibular septum that is displaced anteriorly, superiorly, and rightward?

A

Tetralogy of Fallot

70
Q

When do symptoms of ASD usually present?

A

not in infancy- takes a while to develop symptoms

72
Q

What is the most common ASD?

A

secundum ASD

73
Q

How common are coarctations of the aorta?

A

5-7% of all congenital heart disease

74
Q

How is VSD treated?

A

diuretics

76
Q

What does the superior endocardial cushion become?

A

L outlet of the interventricular septum mitral valve

78
Q

What is considered a large VSD?

A

those as big as the aortic orifice

79
Q

What are the characteristics of Eisenmenger’s Syndrome?

A
  1. muscularization of the Pulm arterioles 2. pulmonary HTN 3. increased RV pressure 4. shunt reverses to R to L 5. no treatment other than transplant
80
Q

DX? 1. tachycardia 2. difference in upper extremity and lower extremity BP 3. pulmonary rales 4. hepatomegaly 5. S2 and S3 gallop 6. soft systolic murmur

A

Coarctation of the aorta

82
Q

What are the xray findings in VSD?

A

cardiomegaly with enlarged pulm arteries

83
Q

What is the normal pressure within the RV?

A

20

84
Q

The atrial septum fuses with the endocardial cushions, which are involved in ______.

A

ventricular septation

85
Q

When can a VSD be a right to L shunt?

A

with pulmonary stenosis

86
Q

DX? precordial activity normal normal 2nd heart sound 2-4/6 early systolic murmur NO diastolic rumble

A

small VSD

87
Q

How is ASD definitively diagnosed?

A

echo

88
Q

What does the inferior endocardial cushion become?

A

inlet portion of the interventricular septum membranous portion of the intervent. septum tricuspid and mitral valves

89
Q

What syndrome is associated with coarctation of the aorta?

A

Turner’s Syndrome

90
Q

DX? 1. RV outflow tract obstruction 2. RVH 3. dextraposition of the aorta 4. VSD

A

Tetralogy of Fallot

92
Q

What is a fixed split S2 and what is it diagnostic of?

A

when the S2 is split at any point in the respiration cycle; ASD

93
Q

DX? 2-3/6 harsh, holosystolic murmur loudest at the LLSB diastolic murmur

A

VSD

94
Q

How does Tetrology of Fallot present?

A
  1. blue baby 2. loud murmur
95
Q

What are the 4 components of Tetralogy of Fallot?

A
  1. RV outflow tract obstruction 2. RVH 3. dextraposition of the aorta 4. VSD
96
Q

DX? 2-3/6 harsh, holosystolic murmur loudest at the LLSB diastolic murmur

A

large VSD

97
Q

What are the xray findings in VSD?

A

cardiomegaly with enlarged main pulm artery

99
Q

What is an RV outflow obstruction in Tetralogy of Fallot?

A

narrowing of the infundibular region stenosis of the pulmonary valve

100
Q

How is VSD treated?

A

diuretics surgical close if muscular

101
Q

What are the phys exam findings in Tetrology of Fallot?

A
  1. tachycardic and cyanotic OR diaphoretic and tachypnic 2. precordial impulse at the L lower sternal border 3. 2-3/6 short systolic murmur of pulm stenosis
102
Q

What does the right endocardial cushion become?

A

tricuspid valve

104
Q

What are the exam findings with a large VSD?

A

active precordium loud 2nd heart sound 2-3/6 harsh, holosystolic murmur loudest at the LLSB diastolic murmur

106
Q

DX? 1. tachycardic and cyanotic OR diaphoretic and tachypnic 2. precordial impulse at the L lower sternal border 3. 2-3/6 short systolic murmur of pulm stenosis

A

Tetrology of Fallot

107
Q

What does the left endocardial cushion become?

A

posterior leaflet of the mitral valve

108
Q

What will the xray findings be in Coarcation of the Aorta?

A
  1. 3 sign along the L edge of the heart 2. rib notching from dilated intercostal a’s
109
Q

What is considered a large VSD?

A

those as big as the aoric orifice

110
Q

What are the long term risks of untreated ASDs?

A

development of pulm vascular disease atrial arrhythmias HF

111
Q

Where is the most common place for a VSD?

A

the membranous portion

112
Q

____ is the most common cyanotic heart defect and accounts for 15% of all congenital heart defects.

A

Tetralogy of Fallot

113
Q

What are the steps of atrial septal embryology?

A
  1. septum primum closes 2. apoptosis of the middle primum cells to form the osteum secundum 3. septum secundum closes to enclose the foramen ovale
114
Q

What causes the murmur in Tetrology of Fallot?

A

pulmonary stenosis

116
Q

What is S2?

A

the 2nd heart sound

117
Q

The atrial septum fuses with the _____, which are involved in ventricular septation.

A

endocardial cushions

118
Q

What are the s/s of cerebral abscesses that happen in cyanotic heart diseases?

A

pt older than 2 years persistent, unexplained fevers behavioral changes

119
Q

In Tetrology of Fallot, the RV and LV pressures are?

A

equal

120
Q

Dx? hypoxic or hypercyanotic episode, usually in the 2-6th month of life can be life threatening unk mechanism

A

Tet spell (Tetralogy of Fallot)

122
Q

What is the most common type of VSD?

A

perimembranous VSD

123
Q

How will a baby present with coarctation of the aorta?

A

tachypnea, diaphoresis, poor feeding; cardiac shock with HF; LACK OF FEMORAL PULSES

124
Q

What are the s/s of cerebral abscesses that happen in cyanotic heart diseases?

A

pt older than 2 years persistent, unexplained fevers behavioral changes

125
Q

What is the normal pressure within the RV?

A

20