Acyanotic Congenital Heart Defects Flashcards

1
Q

What is cyanosis? 2

A
  1. A blue, gray, or dark purple discolouration of the mucous membranes caused by low blood oxygen (hypoxia)
  2. Blood with low oxygen levels circulating to the body
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2
Q

What is cyanosis caused by?

A

Oxygenated blood mixing in with unoxygenated blood

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3
Q

Cyanosis defects does not allow for what?

A

Adequate oxygenation, therefore is more serious

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4
Q

With acyanotic, babies still receive adequate oxygen despite what?

A

The defect therefore is less serious

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5
Q

Determine the two hearts?

A

Left: complete AVSD
Right: Incomplete AVSD

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6
Q

Label the VSD

A
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7
Q

What is this image represent?

A

Inside a heart with patent ductus arteriosus
The label is a connection (patent ductus arteriosus)

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8
Q

What does this image represent?

A

PDA and Desc. Ao

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9
Q

What does this spectral doppler proove? (This represents flow from the Ao to PA)

A

Due to low PA pressure, there is continuous flow from Ao to PA. Peaks at mid- end systole

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10
Q

What does this image represent?

A

CCTGA L-TGA

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11
Q

What would we see in PSAX with CCTGA?

A

We would see a AV with 2 leaflet tips and a PV with 3 leaflets

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12
Q

What does this image represent?

A

Cor triatriatum

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13
Q

What does this image represent?

A

Cor Triatriatum Dexter

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14
Q

What does this image represent?

A

Partial anomalous pulmonary venous return

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15
Q

What does this image represent?

A

Ebstein anomaly

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16
Q

What does this image demonstrate?

A

Persistent Left SVC

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17
Q

If there is a persistent L-SVC, there is a good chance that the LSVC shunts blood into the RA, this would cause what?

A

RA dilation and coronary sinus dilation

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18
Q

What does this image represent?

A

Dilated coronary sinus

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19
Q

What does this image represent?

A

Persistent LSVC

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20
Q

What are 3 types of acyanotic heart defects?

A
  1. Shunt-related defects
  2. Obstructive Defects
  3. Other Acyanotic defects
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21
Q

What are 5 shunt related defects?

A
  1. Atrial septal Defects (ASD)
  2. Ventricular Septal defect (VSD)
  3. Atrioventricular septal defects (AVSD)
  4. Patent ductus Arteriosus (PDA)
  5. Congenitally corrected transposition of the great arteries
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22
Q

What are obstructive acyanotic heart defects? 2

A
  1. All involve a narrowing of a valve or vessel
  2. Non-shunt related acyanotic heart defects
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23
Q

What are 3 types of obstructive defects

A
  1. Congenital Aortic stenosis:(AS)/Bicuspid AV
  2. Pulmonary Stenosis:
  3. Coarctation of the aorta (COA)
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24
Q

Besides Shunt related and obstructive defects of acyanosis what are other defects? 4

A
  1. Cor triatriatum
  2. Partiall anomalus pulmonary venous return (PAPVR)
  3. Ebstein anomaly
  4. Persistent left superior vena cava
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25
Q

What is patent ductus arteriosus?

A

A connection between the descending aorta and origin of the left pulmonary artery

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26
Q

What is PDA caused by?

A

Failure of the ducts arteriosus to close at birth

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27
Q

PDA shunts move LT&raquo_space;> RT why?

A

Because of higher LT sided pressure, therefore AO&raquo_space;> PA

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28
Q

What is blood flow like through the PDA?

A

Continuous

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29
Q

What does PDAs sound like?

A

Machinery murmur due to the continuous blood flow

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30
Q

Depending on the size of the shunt what might happen to the PDA? 2

A
  1. PDA may be asymptomatic
  2. May cause LV volume overload
31
Q

If the PDA causes LV volume overload, what happens? 2

A
  1. Hyperdynamic LV
  2. Dilatation of the LA and LV
32
Q

How might he PDA close or get repaired? 3

A
  1. Pharmacologically - premature infants - indomethacin
  2. Surgically
  3. Percutaneously
33
Q

PDA is best imaged from what window?

A

PSAX - AV/PV Level (base level AV/PV focus)

34
Q

With the PSAX AV/PV view what does the colour doppler look like?

A

Continuous red high velocity jet in the MPA or its branches, which is best seen during diastole

35
Q

In terms of PDAs: Qp and Qs is measured at what level? 2

A
  1. Qp is measured at the level of LVOT
  2. Qs is measured at the level of the RVOT
36
Q

The magnitude of the PDA shunt is calculated as a ratio between what?

A

Qp and Qs

37
Q

In terms of shunts, if there is increase flow, which side does a normal shunt increase and which side does a PDA increase? (Of the heart)

A

Normal shunts increase on the RT side of the heart and the PDA increases flow on the LT side of the heart.

38
Q

Why is the QP:QS ratios switched for PDA?

A

Because of the flow directions of the shunts.

39
Q

Congenitally corrected TGA (L-TGA) is also called or referred to as what? 4

A
  1. Corrected transposition
  2. L-transposition
  3. L-TGA
  4. CC-TGA
40
Q

CC-TGA is characterized by what? 2

A
  1. Leftward looping of the ventricles
  2. Transposition of the great vessels
41
Q

What’s an easy way to determine CC-TGAs?

A

Both the ventricles and the great arteries are switched

42
Q

What is something we see with CC-TGAs which is abnormal with the Ao?

A

Typically the Ao is anterior and to the left of the PA but in this situation it is switched around

43
Q

What is double discordance (AV + VA)? 2

A
  1. When the Morphological RV now pumps out to the aorta
  2. When the Morphological LV now pumps out to the pulmonary artery
44
Q

With CCTGA/ L-TGA, The TV often leaks because of what? What does this result in?2

A
  1. The RV was not built to handle systemic arterial pressures
  2. This results in TV annulus dilating leading to TR
45
Q

What are symptoms of CCTGA/ L-TGA?2

A

Depends on
1. Degree of TR (systemic AV valve), VSD
2. Severity of outflow tract obstruction
Patients may be asymptomatic

46
Q

What are some associated lesions/anomalies seen with CCTGA/ L-TGA? 4

A
  1. VSD
  2. Tricuspid valve anomalies
  3. Pulmonary outflow tract obstruction
  4. Conduction defects
47
Q

What is Cor Triatriatum?

A

A perforated membrane that partitions to the left or right atrium into two chambers

48
Q

The size of the perforation of Cor Triatriatum determines what?3

A
  1. Severity of obstruction
  2. Symptoms
  3. Age of presentation
49
Q

In terms of Cor Triatriatum, what does a small orifice mean?2

A
  1. Less flow
  2. More symptoms
50
Q

In terms of Cor Triatriatum, what does large orifice mean?

A
  1. More flow
  2. Less symptoms
51
Q

What is Cor Triatriatum sinister? What is it characterized by?

A
  1. Divided left atrium
  2. Characterized by a perforated membrane in the left atrium
52
Q

Where are the pulmonary veins in Cor triatriatum sinister?2

A
  1. The pulmonary veins come together posteriorly
  2. Membrane between the PV confluence and the mitral valve
53
Q

What are symptoms of Cor Triatriatum sinister? (What is it similar to?)

A

Similar to valvular mitral stenosis

54
Q

What are some echo findings of Cor Triatriatum sinister?3

A
  1. Membranes across the LA
  2. PVs may be connected superiorly and inferiorly to the membrane
  3. If 1 or 2 PVs connect inferiorly to the septation, there are less symptoms
55
Q

Cor triatriatum sinister is assocaited with what?

A

ASD

56
Q

What is Cor Triatriatum dexter?

A
  1. Divided right atium
  2. Characterized by a perforate membrane in the RA
57
Q

How common is Cor Triatriatum?

A

Extremely rare

58
Q

What are echo features of Cor Triatriatum dexter?2

A
  1. Suspected when a linear structure is visualized in the right atrium that does not resemble a Eustachian valve
  2. Assess membrane/ orifice with colour and spectral doppler
59
Q

What does PAPVR stand for?

A

Partial Anomalous pulmonary venous return

60
Q

What happens with PAPVR?4

A

1-3 of the PVS are connected to
1. systemic vein (SVC/IVC)
2. RA
3. Coronary sinus
4. LT innominate vein

60
Q

If there is a single PV connected to the SVC, this is associated with what?

A

Sinus venosus ASD

60
Q

What are some configurations of PAPVR?3

A
  1. LT sided PVs may connect to the coronary sinus and/or the left innominate vein
  2. RT sided PVs may connect to the RA, SVC/ IVC
  3. Single PV connected to the SVC
60
Q

PAPVR features are similar to those found in patients with an ASD and include what?3 (what we see with PAPVR)

A
  1. Hypertrophy and dilation of the RA and RV
  2. Dilatation of the PA
  3. RVVO
61
Q

What does Atrialization of RV cause?3

A
  1. May cause PFO/ASD
  2. May become cyanotic if shunt reverses
  3. TR
61
Q

What are echo features of Ebstein anomaly?4

A
  1. Apical displacement of one or all TV leaflets
  2. Leaflets tethered to myocardium
  3. Atrialization of RV
  4. Small RV, Large RA
62
Q

What is Ebsteins anomaly associated with?5

A
  1. ASD/ VSD/ PFO
  2. PDA
  3. PS/MS
  4. Tetraology of fallot
  5. D-TGA
63
Q

What is a persistent left SVC?

A

The L- SVC is formed by the confluence of the left jugular and subclavian veins and descends inferiorly parallel to the right SVC in most cases

64
Q

In terms of Persistent Left SVC, The L-SVC commonly enters where? and it rarely enters where?

A
  1. Coronary sinus
  2. LA
65
Q

Approximately how often does a persistent Left SVC occur?

A

0.3% of the population and is higher in patients with other CHDs

66
Q

What is persistent left SVC associated with?

A

Any type of ASD but the coronary sinus ASD is most common

67
Q

Does Persistent Left SVC affect physiology of the heart?

A

Nope it remains the same

68
Q

What are some echo features of persistent L-SVC? 2

A
  1. The flow of L-SVC blood into the coronary sinus results in dilation of the coronary sinus
  2. Best imaged in the A4C view with posterior angulation which shows the coronary sinus nicely
69
Q
A