Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Echo > Cyanotic Heart defects > Flashcards

Cyanotic Heart defects Flashcards

1
Q

What are the Terrible T’s?

A
  1. Tetralogy of Fallot (TOF or Tet)
  2. Truncus Arteriosus
  3. Transposition of the great arteries (TGA or TVG)
  4. Total anomalous pulmonary venous return (TAPVR or TAPVC)
  5. Tricuspid atresia (TVA)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is tetralogy of Fallot?

A

Tetralogy of Fallot is a set of four defects all present at the same time including:

  1. VSD
  2. Overriding aorta
  3. Pulmonary stenosis (subvalve, valvular, supravalve, branch, combination or atresia)
  4. Right Ventricular Hypertrophy (RVH)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the commonality of TOF?

A

• TOF is the most common cyanotic heart defect, accounting for approx. 10 % of all CHD.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Is a patient always cyanotic? What the major determining factor of weather TFO will be cyanotic or not?

A

• TOF may not always causes cyanosis

the severity of pulmonary stenosis that creates more R-L shunting to occur

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are associated defects with tetralogy of Fallot?

A 
•	Right aortic arch (30 %)
•	Secundum ASD (25%) 
o	Referred to as pentalogy when present with all other TOF defects 
•	Persistent left SVC (11%)
•	Coronary artery arteries (10%)
•	 Complete AVSD
•	PDA
•	Absent pulmonary valve 
•	Pulmonary valve atresia 
•	Aortic valve abnormalities
o	Number of leaflets, valve dimensions, AI
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Patient history in a patient with Tet?

A

Asymptomatic (if acyanotic)
Cyanosis (Severity generally increases with time)
Dyspnea of exertion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Physical exam in a patient with Tet?

A 
o	Failure to thrive (FTT)
o	CHF
o	Squatting (to relive symptoms of SOB)
o	Cyanosis 
o	Clubbing of fingers and toes (from cyanosis)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is a “tet” spell

A

• A “tet” spell is brought on by a sudden drop in oxygen saturation and marked by a deep blue coloring of the lips, skin and nails after crying, feeding or when agitated.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What natural positioning a child will take to help relieve the symptoms of a tet spell?

A

• To relieve symptoms of a “tet” spell, a child will generally assume a child’s pose or a squat leaning forward because it increases PVR and thus decrease the magnitude of the R to L shunting via the VSD.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What will the EKG of someone with TOF show?

A
  • May be normal
  • Bi ventricular hypertrophy
  • Right axis deviation
  • RA enlargement
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How long will Patients with TOF who have not had intervention live?

A

• 20’s or 30’s and in some cases longer. However, the patient’s quality of life is severely impaired and activity levels are low.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

If left untreated, what is the natural history or likely course of progression of TOF?

A
  • Increase in severity of cyanosis and pulmonary stenosis
  • Infundibular stenosis (RVOTO), if present, may periodically occlude flow to the lungs creating hypoxic periods that increase the risk of developing or provoking arrhythmias and sudden death tet spells.
  • Retardation with growth (from lack of proper oxygen supply)
  • Increased risk of SBE
  • Increased risk of stroke
  • Anemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What does medical treatment of TOF look like?

A

o Treat tet spells with oxygen, positional changes and beta blockers (propranolol)
o Treat anemia
o SBE prophylaxis
o Prostaglandin E to keep PDA open ( in cases of severe stenosis or atresia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How is ToF surgically treated?

A

o Palliative: (improving the problem but not correcting it)
 Primarily in cases where PA branch stenosis is too severe and the vessels will not tolerate right heart CO after complete repair
• Systmeic PA shunt (modified BT shunt most common)
• Staged unifocalization (can be single or multiple stages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is done for complete repair of Tof?

A

Closure of VSD
Relief of pulmonary obstruction
• RVOT resection, transannular patch, PA conduit (PV atresia)
Repair of associated defects

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

After undergoing surgery for TOF, a patient will be reevaluated with echo. What should we as techs look for in a post op assessment?

A
  • Assess residual pulmonary stenosis
  • Assess severity of PI
  • Assess RV dimension
  • Obtain TR gradient
  • Assess for residual VSD shunting
  • Assess LV function
  • Assess Ao for AI
  • Assess repair of any additional defects
  • Evaluate for pericardial effusion (common after surgery
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is Truncus Arteriosus?

A
  • Truncus arteriosus is the presence of a single, common truck that gives rise to the coronary arteries and aortic arch
  • There will be a large membranous or outlet VSD with R to L shunting
  • The truncal valve may have anywhere from two to six leaflets and may be dilated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is Type 1 Truncus Arteriosus?

A

Main PA arises from trunk and then bifurcates into the LPA and RPA (60% of cases)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is Type 2 truncus arteriosus?

A

RPA and LPA arise from the posterior portion of the trunk as separate orifices (20 % of cases)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is Type 3 truncus arteriosus?

A

RPA and LPA arise separately from the sides of the trunk (20 % of cases)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is Type 4 truncus arteriosus?

A

Bronchial collateral vessels originate from the descending Ao (10% cases)
Also classified as Tetralogy of Fallot with pulmonary atresia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What defects are associated with truncus arteriosus?

A
  • Maligned VSD (100%)
  • Right aortic arch (30-50%)
  • Secundum ASD (10-30%)
  • Interrupted Aortic arch (11-19 %)
  • Persistent left SVC (6%)
  • Absent PDA
  • Truncal valve regurgitation
  • Coronary artery anomalies
  • DiGeorge syndrome
  • Maternal diabetes
  • Arch anomalies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Describe an expected history and findings from a physical exam on a patient with truncus arteriosus.

A
  • Cyanosis
  • Tachypnea
  • Poor feeding and weight gain
  • Dypsnea
  • Tachycardia
  • Systolic thrill at left sternal border
  • Wide pulse pressure
  • CHF
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What does the auscultation of someone suffering from truncus arteriosus sound like?

A
  • Loud single S2
  • S3
  • Loud systolic ejection click from the truncal valve
  • Systolic murmur
  • Diastolic decrescendo murmur (truncal insufficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
How is the EKG of someone with truncus arteriosus altered?
* Biventricular hypertrophy * LA or biatrial enlargement * Right axis deviation
26
If left untreated, what is the likely progression of truncus arteriosus?
* CHF * Increased truncal valve insufficiency * Pulmonary artery occlusion or worsening stenosis * Pulmonary over circulation leading to pulmonary HNT
27
How is truncus arteriosus treated medically?
SBE prophylaxis | Treat heart failure and pulmonary over circulation
28
How is truncus arteriosus treated surgically?
Palliation Pulmonary artery banding Modified Ao- PA shunt
29
How is truncus arteriosus repaired?
Rastelli procedure • VSD closure, RV to PA conduit (with or without PV) PA unifocalization (type 4) and then Rastelli procedure Repair associated defects
30
What is the outcome if a conduit is used to repair a truncus arteriosus?
The conduit is a fixed size and will not grow with the patient. Most patients who have a conduit placed the first time will have to undergo an additional surgery
31
What should be included in the post op echo assessment of Truncus Arteriosus?
• Evaluate for residual shunting • Evaluate neo-aortic (truncal) valve o Size, regurgitation, stenosis etc. • Evaluate severity of RV-PA conduit stenosis • Evaluate repair of any associated defects
32
What is complete transposition of the great arteries (d-TGA)?
a condition that occurs when the great vessels arise from the wrong ventricle and cause circulation of cyanotic blood PA arises from the LV and Ao arises from the RV RV recirculates deoxygenated systemic blood back into the body while the LV recirculates oxygen rich blood from the lungs back to the lungs
33
What is the prevalence of d-TGA?
* D-TGA accounts for approx. 5% of all CHD * Male to female prevalence 3:1 * Increased incidence in diabetic mothers
34
What is survival with this defect dependent on?
• Survival is dependent on mixing of oxygenated and deoxygenated blood via shunting at the ductal, ventricular and or atrial level
35
If left untreated, what is the expected natural history of a child with d-TGA?
* Progressive hypoxia * Acidosis * CHF * Pulmonary disease (stenosis, HNT etc.) * 90 % of patients die before the age of six months without intervention
36
What is the goal of echo in evaluating for d-TGA?
Confirm diagnosis Identify any and all levels of shunting (PDA, VSD, ASD) Identify coronary artery ostia and courses Identify any and all associated lesions Determine arch sidedness
37
How is d-TGA treated, medically?
``` Prostaglandin E (to prevent PDA from closing) Treat CHF (digitalis, Lasix) ```
38
How is d-TGA treated, surgically?
Jatene repair- arterial switch Rastelli Mustard/senning PA banding (To buy time and protect against pulmonary HNT)
39
How is d-TGA treated, catheterization?
Rashkind atrial balloon septostomy | Often performed in ICU under echo guidance
40
What is total anomalous pulmonary venous return (TAPVR)?
a condition when the pulmonary veins are not connected to the left atrium, and instead enter into the right atrium
41
What will always be present with TAPVR?
ASD
42
How common is a TAPVR?
TAPVR is not common and accounts for approx. 1 % of all CHD
43
How are the types of TAPVR classified?
Supra Cardiac Cardiac infra cardiac Mixed
44
Describe supracardiac TAPVR
• Supracardiac (most common approx. 50%) | Pulmonary vein confluence drains to left sided vertical vein, to innominate vein, to right sided SVC to RA
45
Describe a Cardiac TAPVR
Cardiac (approx. 20%) | Pulmonary vein confluence drains to coronary sinus then to RA
46
Describe an Infracardiac TAPVR
Infracardiac (sub diaphragmatic- 20%) | o Pulmonary venous confluence drains to portal vein/IVC then to RA
47
Which type of TAPVR has a 4:1 male prevalence?
infracardiac
48
Which type of TAPVR is most likely to develop obstruction?
infracardiac
49
Which type of TAPVR has the highest mortality?
infracardiac
50
What defects are associated with TAPVR?
* Univentricular heart * Truncus arteriosus * D-TGA * Hypoplastic left heart * Asplenia/polysplenia * Pulmonary atresia * Coarctation of the aorta
51
Left untreated, what is the expected natural history of someone with TAPVR?
* Recurrent pneumonias * Pulmonary hypertension * Progressive congestive heart failure * Severe failure to thrive
52
What is the goal of echo in evaluating for TAPVR?
o Confirm diagnosis o Identify drainage of all 4 pulmonary veins determining type of TAPVR o Evaluate the path from venous confluence to RA for any area of stenosis o Assess atrial septal defect for restriction (should be right to left shunting o Evaluate LA & LV size
53
How is TAPVR treated, medically?
Treat symptoms of CHF Oxygen Prevention of recurrent infections and endocarditis
54
How is TAPVR treated, surgically?
Connect pulmonary venous confluence back of LA Repair ASD Ligate previous pulmonary venous drainage Note that a Rashkind atrial septostomy might be needed as a palliative procedure to increase LA flow
55
What should be included in a post op echo assessment of a patient either corrected TAPVR?
* Identify pulmonary venous flow entering LA and evaluate for any evidence of obstruction * Assess chamber sizes & function * Rule out any residual atrial shunting * Assess repair of any associated defects * Identify that previous drainage path/paths have been ligated
56
What is tricuspid valve atresia?
the underdevelopment of the tricuspid valve causing a lack of communication between the RA and RV • Has to be an ASD with R to L shunting in order for blood to migrate to the left heart
57
What is the prevalence of TV atresia and is it gender dominate?
* The prevalence of TV atresia is approx. 1-3 % of all CHD | * Slight male prevalence
58
What complications can arise from TV atresia?
* Endocarditis (15-25%) * Arrhythmias (7%) * Brain abscess (2-5%) * Progressive cyanosis * Congestive heart failure * Cerebrovascular accident
59
How is TV atresia classified?
* Type Ia: Normally related Great vessels with intact ventricular septum and PV atresia * Type Ib: Normally related Great vessels with small VSD & PV stenosis (hypoplasia) * Type Ic: Normally related Great vessels with large VSD and normal PV * Type IIa: d-TGA with VSD & AoV atresia * Type IIb: d-TGA with VSD & AoV stenosis * Type IIc: d-TGA with VSD & normal AoV * Type III: L-TGA with malposition of the Great vessels
60
What would you expect to see in the history and physical exam of a patient with TV atresia?
* Cyanosis * Clubbing * Tachycardia * Anemia * Congestive heart failure * Tachypnea * Poor feeding/failure to thrive * Hypoxic spells
61
What should be evaluated with echo when diagnosing TV atresia
``` Assess for any antegrade flow through TV Evaluate ASD for restriction Evaluate associated defects Great vessel relationship Semilunar valve function VSD ```
62
What is the medical treatment for TV atresia?
o Prostaglandin E1 to keep PDA open o Digitalis and Lasix to treat heart failure symptoms o SBE prophylaxis o Atrial septostomy
63
What is the surgical treatment for TV atresia?
``` o Aorta-pulmonary shunt & PDA ligation  Blalock-Tausig, central o PA Band o Bidirectional Glenn o Fontan ```
64
What should be evaluated in a post op assessment of corrected TV atresia?
``` • Evaluate surgical intervention o Shunt, Glenn, Fontan, PA Band, etc. • Assess LV size & function • Assess atrial shunt • Identify any associated defects • Rule out thrombus ```
65
What are some variations on a single ventricle?
• Pulmonary atresia with intact ventricular septum

Echo (2 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions