Acyanotic Congenital Heart Defects Flashcards
1
Q
What is cyanosis? 2
A
- A blue, gray, or dark purple discolouration of the mucous membranes caused by low blood oxygen (hypoxia)
- Blood with low oxygen levels circulating to the body
2
Q
What is cyanosis caused by?
A
Oxygenated blood mixing in with unoxygenated blood
3
Q
Cyanosis defects does not allow for what?
A
Adequate oxygenation, therefore is more serious
4
Q
With acyanotic, babies still receive adequate oxygen despite what?
A
The defect therefore is less serious
5
Q
Determine the two hearts?
A
Left: complete AVSD
Right: Incomplete AVSD
6
Q
Label the VSD
A
7
Q
What is this image represent?
A
Inside a heart with patent ductus arteriosus
The label is a connection (patent ductus arteriosus)
8
Q
What does this image represent?
A
PDA and Desc. Ao
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
10
Q
What does this image represent?
A
CCTGA L-TGA
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
12
Q
What does this image represent?
A
Cor triatriatum
13
Q
What does this image represent?
A
Cor Triatriatum Dexter
14
Q
What does this image represent?
A
Partial anomalous pulmonary venous return
15
Q
What does this image represent?
A
Ebstein anomaly
16
Q
What does this image demonstrate?
A
Persistent Left SVC
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
18
Q
What does this image represent?
A
Dilated coronary sinus
19
Q
What does this image represent?
A
Persistent LSVC
20
Q
What are 3 types of acyanotic heart defects?
A
- Shunt-related defects
- Obstructive Defects
- Other Acyanotic defects
21
Q
What are 5 shunt related defects?
A
- Atrial septal Defects (ASD)
- Ventricular Septal defect (VSD)
- Atrioventricular septal defects (AVSD)
- Patent ductus Arteriosus (PDA)
- Congenitally corrected transposition of the great arteries
22
Q
What are obstructive acyanotic heart defects? 2
A
- All involve a narrowing of a valve or vessel
- Non-shunt related acyanotic heart defects
23
Q
What are 3 types of obstructive defects
A
- Congenital Aortic stenosis:(AS)/Bicuspid AV
- Pulmonary Stenosis:
- Coarctation of the aorta (COA)
24
Q
Besides Shunt related and obstructive defects of acyanosis what are other defects? 4
A
- Cor triatriatum
- Partiall anomalus pulmonary venous return (PAPVR)
- Ebstein anomaly
- Persistent left superior vena cava
25
What is patent ductus arteriosus?
A connection between the descending aorta and origin of the left pulmonary artery
26
What is PDA caused by?
Failure of the ducts arteriosus to close at birth
27
PDA shunts move LT >>> RT why?
Because of higher LT sided pressure, therefore AO >>> PA
28
What is blood flow like through the PDA?
Continuous
29
What does PDAs sound like?
Machinery murmur due to the continuous blood flow
30
Depending on the size of the shunt what might happen to the PDA? 2
1. PDA may be asymptomatic
2. May cause LV volume overload
31
If the PDA causes LV volume overload, what happens? 2
1. Hyperdynamic LV
2. Dilatation of the LA and LV
32
How might he PDA close or get repaired? 3
1. Pharmacologically - premature infants - indomethacin
2. Surgically
3. Percutaneously
33
PDA is best imaged from what window?
PSAX - AV/PV Level (base level AV/PV focus)
34
With the PSAX AV/PV view what does the colour doppler look like?
Continuous red high velocity jet in the MPA or its branches, which is best seen during diastole
35
In terms of PDAs: Qp and Qs is measured at what level? 2
1. Qp is measured at the level of LVOT
2. Qs is measured at the level of the RVOT
36
The magnitude of the PDA shunt is calculated as a ratio between what?
Qp and Qs
37
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)
Normal shunts increase on the RT side of the heart and the PDA increases flow on the LT side of the heart.
38
Why is the QP:QS ratios switched for PDA?
Because of the flow directions of the shunts.
39
Congenitally corrected TGA (L-TGA) is also called or referred to as what? 4
1. Corrected transposition
2. L-transposition
3. L-TGA
4. CC-TGA
40
CC-TGA is characterized by what? 2
1. Leftward looping of the ventricles
2. Transposition of the great vessels
41
What's an easy way to determine CC-TGAs?
Both the ventricles and the great arteries are switched
42
What is something we see with CC-TGAs which is abnormal with the Ao?
Typically the Ao is anterior and to the left of the PA but in this situation it is switched around
43
What is double discordance (AV + VA)? 2
1. When the Morphological RV now pumps out to the aorta
2. When the Morphological LV now pumps out to the pulmonary artery
44
With CCTGA/ L-TGA, The TV often leaks because of what? What does this result in?2
1. The RV was not built to handle systemic arterial pressures
2. This results in TV annulus dilating leading to TR
45
What are symptoms of CCTGA/ L-TGA?2
Depends on
1. Degree of TR (systemic AV valve), VSD
2. Severity of outflow tract obstruction
*Patients may be asymptomatic*
46
What are some associated lesions/anomalies seen with CCTGA/ L-TGA? 4
1. **VSD**
2. Tricuspid valve anomalies
3. Pulmonary outflow tract obstruction
4. Conduction defects
47
What is Cor Triatriatum?
A perforated membrane that partitions to the left or right atrium into two chambers
48
The size of the perforation of Cor Triatriatum determines what?3
1. Severity of obstruction
2. Symptoms
3. Age of presentation
49
In terms of Cor Triatriatum, what does a small orifice mean?2
1. Less flow
2. More symptoms
50
In terms of Cor Triatriatum, what does large orifice mean?
1. More flow
2. Less symptoms
51
What is Cor Triatriatum sinister? What is it characterized by?
1. Divided left atrium
2. Characterized by a perforated membrane in the left atrium
52
Where are the pulmonary veins in Cor triatriatum sinister?2
1. The pulmonary veins come together posteriorly
2. Membrane between the PV confluence and the mitral valve
53
What are symptoms of Cor Triatriatum sinister? (What is it similar to?)
Similar to valvular mitral stenosis
54
What are some echo findings of Cor Triatriatum sinister?3
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
Cor triatriatum sinister is assocaited with what?
ASD
56
What is Cor Triatriatum dexter?
1. Divided right atium
2. Characterized by a perforate membrane in the RA
57
How common is Cor Triatriatum?
Extremely rare
58
What are echo features of Cor Triatriatum dexter?2
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
What does PAPVR stand for?
Partial Anomalous pulmonary venous return
60
What happens with PAPVR?4
1-3 of the PVS are connected to
1. systemic vein (SVC/IVC)
2. RA
3. Coronary sinus
4. LT innominate vein
60
If there is a single PV connected to the SVC, this is associated with what?
Sinus venosus ASD
60
What are some configurations of PAPVR?3
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
PAPVR features are similar to those found in patients with an ASD and include what?3 (what we see with PAPVR)
1. Hypertrophy and dilation of the RA and RV
2. Dilatation of the PA
3. RVVO
61
What does Atrialization of RV cause?3
1. May cause PFO/ASD
2. May become cyanotic if shunt reverses
3. TR
61
What are echo features of Ebstein anomaly?4
1. **Apical displacement** of one or all TV leaflets
2. Leaflets **tethered** to myocardium
3. Atrialization of RV
4. Small RV, Large RA
62
What is Ebsteins anomaly associated with?5
1. ASD/ VSD/ PFO
2. PDA
3. PS/MS
4. Tetraology of fallot
5. D-TGA
63
What is a persistent left SVC?
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
In terms of Persistent Left SVC, The L-SVC commonly enters where? and it rarely enters where?
1. Coronary sinus
2. LA
65
Approximately how often does a persistent Left SVC occur?
0.3% of the population and is higher in patients with other CHDs
66
What is persistent left SVC associated with?
Any type of ASD but the coronary sinus ASD is most common
67
Does Persistent Left SVC affect physiology of the heart?
Nope it remains the same
68
What are some echo features of persistent L-SVC? 2
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
