Cyanotic Congenital Heart Defects Flashcards
1
Q
What does this image represent?
A
Truncus arteriosus
2
Q
What does this image represent?
A
Complete transposition o the great arteries (D-TGA)
3
Q
What does this image represent?
A
D-TGA
4
Q
What does this image represent?
A
D-TGA in echo
5
Q
What does this image represent?
A
Tricuspid artresia
6
Q
What does this image represent?
A
Tricuspid Atresia
7
Q
What does this image represent?
A
Tetralogy of fallot
8
Q
What does this image represent?
A
Tetralogy of Fallot
9
Q
What does this image represent?
A
Total anomalous pulmonary Venous return
10
Q
What does this image represent?
A
Various confirgurations of TAPVR
11
Q
What does this image represent?
A
Pulmonary atresia - intact IVS
*The arrow is pointing to the tricuspid valve because it shows that there is hypoplasia of the RV and RV
12
Q
What does this image represent ?
A
Hypoplastic left heart syndrome
13
Q
What does this image represent?
A
Hypoplastic left heart syndrome
14
Q
What does this image represent?
A
Double outlet right ventricle (DORV)
15
Q
What does this image represent?
A
Double outlet right ventricle
16
Q
What does this image represent?
A
Coarctation of the aorta
17
Q
What does this image represent?
A
Coarctation of Aorta
18
Q
What is cyanosis?
A
A blue, gray, or dark purple discolouration of the mucous membranes caused by low oxygen (Severe hypoxia)
19
Q
What is cyanosis caused by? 2
A
- Oxygenated blood mixing with unoxygenated blood
- Blood with low oxygen levels circulating to the body
20
Q
What are S/S of cyanosis CHD? 9
A
- Cyanosis of lips, toes, fingers, Tips of nose
- Blueish skin
- Hyperventilation
- Rounded, clubbed fingers
- Tachycardia
- Sweating
- SOB
- Chronic respiratory infections
- Failure to thrive
21
Q
What is the 5 Ts and these?
A
8 main congenital heart defects considered to be cyanotic
22
Q
Name the 5 Ts and these?
A
The 5 Ts
- Truncus arteriosus
- Complete transposition of the great arteries
- Tricuspid atresia
- Tetralogy of fallot
- Total anomalous pulmonary venous return
These
6. Pulmonary atresia
7. Hypoplastic left heart syndrome
8. Double outlet right ventricle
23
Q
What is Truncus arteriosus?
A
Single large vessel arising from the base of the heart
24
Q
What does this single large vessel of Truncus arteriosus supply?
A
The coronary, pulmonary, and systemic circulation
25
What are some findings of Truncus arteriosus? 3
1. Large VSD
2. Large common artery (As and PA)
3. PFO
26
With Truncus arteriosus, the common trunk with a single semilunar valves arises from the heart, do what?
Overrides the VSD
27
The common valve may have how many cusps?
1-6
28
What are some things that arise from the single trunk of the Truncus arteriosus? 3
1. Coronary arteries
2. Main pulmonary artery
3. Aortic arch
29
What is the incidence rate of complete transposition of the great vessels between men and women?
3:1 incidence
30
What is another way to refer to Complete transposition of the great arteries ?
D-TGA
31
With D-tga, the Ao and the PA arises from which ventricles?
1. The morphological RV
2. Morphological LV
32
Where in relation is the Ao and the PA to each other? (D-TGA)
They are parallel
33
What are some things that we would see with D-TGA? 3
1. PFO/ASD
2. Transposed PA and Ao
3. PDA
34
What are important things to remember about D-TGA? 3
1. Vessels run parallel
2. Shunt dependent
3. Fatal if not corrected
35
What are some echo features to know about D-TGA? 2
1. AV anterior + to the RT of MPA
2. Both seen in SAX in PSAX view and LAX in PLAX view
36
In terms of D-TGA, Conal ridges grow how?
In parallel rather than spiral fashion inside the Truncus arteriosus
37
In terms of D-TGA, the anterior aortic valve connects where?
The right ventricle
38
In terms of D-TGA, the posterior pulmonic valve connects to what?
The left ventricle
39
How is pulmonary and systemic circulation with D-TGA? What does this mean? 2
1. They are now parallel
2. Mixing of blood occurs through the PDA and PFO
40
What is Tricuspid atresia?
1. Absence of a direct communication between the right atrium ad the right ventricle
41
What is the floor of the RA with Tricuspid atresia?
Floor of the RA is entirely muscular with complete separation form the hypoplastic RV
42
With Tricuspid Atresia, What is flow like?
Flow crosses the ASD and back across the VSD to the RV, then out the PA or Ao
43
What are some things that are associated with Tricuspid Atresia? 3
1. Shunt dependent
2. Hypoplastic RV
3. TGA
44
What is tetralogy of fallot?
Complex set of congenital heart defects, which involves 4 anomalies of the heart
45
Tetralogy of fallot accounts for how much of CHD?
10% of all CHD
46
What is the most common cyanotic heart defect?
Tetralogy of fallot
47
Can tetraogy of fallot be acyanotic?
Yes
48
What is a tetralogy spell?
Periodic spells of extreme cyanosis
49
What are some sonographic features of tetralogy of fallot? 4
1. Large VSD
2. Overriding Aorta (overriding the IVS)
3. Pulmonary stenosis
4. Hypertrophy of right ventricle
50
Tetralogy of fallot can also be associated with what? 2
1. ASD
2. Arial arrhythmias common
51
What is the hemodynamics of tetralogy of fallot? What does this mean? 4
1. Increase PS = Increased shunting across IVS into Ao
2. Path of least resistance
3. Systemic circulation overloaded with low oxygenated blood
4. Cyanosis
52
IF ToF occurs with pulmonary atresia what is the prognosis?
Severe
53
With ToF what can we use to keep the ductus arteriosus open?
Prostaglandin
54
What is TAPVR?
Total anomalous pulmonary venous return
55
What is TAPVR?
Pulmonary veins have no connection to the left atrium
56
What TAPVR, PVs drain where?
Directly or directly into the right atrium
57
What are 3 configurations of TAPVR?
1. Supracardiac
2. Cardiac
3. Infracardiac PV connection
58
What are some important facts to remember about TAPVR? 3 (anatomically)
1. Shunt dependent
2. DIalted RA/RV
3. Small LA
59
What is the treatment for TAPVR? 3
1. Atrial septostomy
2. Anastomosis
3. ASD closure
60
What is the prognosis for Atrial sepptostomy with TAPVR? 2
1. Short term
2. Puncture the IAS to allow blood mixing, this creates a ASD
61
What is Valvular atresia?
1. Congenital absence or closure of a normal body opening or tubular structure
62
Where might valvular atresia occur? Most commonly where?
1. On any valve or vessel
2. Most often pulmonary or tricuspid valves
63
How common is Pulmonary atresia - intact IVS?
Very rare
64
In terms of pulmonary atresia - intact IVS, we might see what happening to the TV and RV?
Hypoplasia
65
What does the interventricular septum look like with pulmonary atresia - intact IVS ?
Intact interventricualr septum
66
What is some thing we need to remember about Pulmonary atresia - intact IVS?
Shunt dependent
67
What is the right ventricular outflow like with pulmonary atresia - intact IVS ?
Complete obstruction of right ventricular outflow
68
What is a ductal dependent lesion?
When an ASD/PFO + PDA is necessary for survival, If there is no ASD, and IAS puncture can be performed in utero
69
In terms of Pulmonary atresia - intact IVS, the PDA is especially helpful, why?
IT brings some blood to the nuts for re-oxygenation
70
Pulmonary atresia - intact IVS usuall requires what after birth?
Urgent surgery
71
What is Pulmonary atresia with VSD? 3
1. Underdevelopment of the RVOT, PV, PA and its branches
2. Large VSD
3. Overriding Aorta (partially committed to both ventricles)
72
What is considered the most severe end of spectrum for ToF?
Pulmonary atresia with VSD
73
In terms of pulmonary atresia with VSD, Prostaglandins are used to do what? What happens if it doesn’t work?
1. Keep the ductus arteriosus patent
2. If the ductus arteriosus closes spontaneously, the baby can go into shock and die
74
In terms of Pulmonary atresia with VSD, ASD/PFO is present in how many patients?
50%
75
In terms of Pulmonary atresia with VSD, surgical treatment will depend on what?
The anatomy of the PA
76
What are some sonographic features of hypoplastic left heart syndrome? 6 (size, flow type, associations, abnormalities seen)
1. Very small LV <10mm
2. Aortic stenosis or atresia
3. Mitral valve atresia
4. Associated with Hypoplastic aortic arch
5. Associated with coarctation of the aorta
6. ASD
77
What is Hypoplastic left heart syndrome? 2
1. Underdevelopment of the left heart- aorta complex
2. Obstruction of the systemic cardiac output + inability to support systemic circulation
78
Hypoplastic Left heart syndrome is dependent on what?
PDA
79
What is the treatment for Hypoplastic Left heart syndrome?
Norwood + Fontan procedures
80
With hypoplastic left heart syndrome we see retrograde/ biphasic flow why? Where do we see this? Where is it commonly seen
1. Low Aorta pressure relative to the PA pressure
2. Seen in the aortic Aortic arch, Ascending aorta, Coronary arteries
81
In terms of hypoplastic left heart syndrome, A small amount of oxygenated blood comes back from where?
The lungs to cross the ASD
82
What does the hypoplastic right ventricle look like sonographically? 2
1. Small RV
2. Small or absent pulmonary artery
83
In terms of hypoplastic right ventricle, a small or absent PV/PA and a small RV affect what?
The flow to the lungs
84
Is the hypoplastic right ventricle disorder shunt dependent?
Yes
85
What is double outlet right ventricle (DORV)?
Both great arteries arise from the morphological RV
86
With DORV neither semilumar valve is in fibrous continuity with what?
The AV valve
87
What do we see with blood in terms of DORV?
Oxygenated blood mixing with deoxygenated blood
88
What does a VSD represent in terms of DORV?
Only outlet from the LV
89
In terms of DORV, What is the occurrence rate of PV stenosis or Subpulmonary stenosis?
Might be present
90
With DORV, if there is an increase in PS what does this mean?
Less flow to the lungs, which means its a worse prognosis
91
What is a coarctation of the aorta?
A congenital luminal narrowing of the aorta due to a posterior “shelf”
92
With Coarctation of aorta when might we see cyanosis?
When severe
93
Where does Coarcation of aorta usually occur?
Near the ductus arteriosus which may still be patent
94
What is the hemodynamics of coarcation of aorta,if there is a PDA?
Much of the blood ejected into the aorta shunts into the PA through the ductus arteriosus if present, then back to the left heart.
95
What is the sonographic features of coarctation of aorta? What view is it best seen in? Where is it seen? 3
1. Narrowed proration of aortic arch
2. Best seen in the SSN view
3. Narrowing may be seen in the pre-ductal, ductal, or post ductal region
96
In terms of coarctation of Aorta, we should use colour doppler to look for what?
Flow occurring in both systole and diastole
97
What is Coarcation of the aorta associated with? 3
1. Bicuspid AV
2. AVSD
3. VSDs
