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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)
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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)
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3
Q

What is the commonality of TOF?

A

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

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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

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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
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6
Q

Patient history in a patient with Tet?

A

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

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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)
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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.

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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.

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10
Q

What will the EKG of someone with TOF show?

A
  • May be normal
  • Bi ventricular hypertrophy
  • Right axis deviation
  • RA enlargement
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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.

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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
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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

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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

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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

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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
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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
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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)

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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)

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20
Q

What is Type 3 truncus arteriosus?

A

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

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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

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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
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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
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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
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25
Q

How is the EKG of someone with truncus arteriosus altered?

A
  • Biventricular hypertrophy
  • LA or biatrial enlargement
  • Right axis deviation
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26
Q

If left untreated, what is the likely progression of truncus arteriosus?

A
  • CHF
  • Increased truncal valve insufficiency
  • Pulmonary artery occlusion or worsening stenosis
  • Pulmonary over circulation leading to pulmonary HNT
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27
Q

How is truncus arteriosus treated medically?

A

SBE prophylaxis

Treat heart failure and pulmonary over circulation

28
Q

How is truncus arteriosus treated surgically?

A

Palliation
Pulmonary artery banding
Modified Ao- PA shunt

29
Q

How is truncus arteriosus repaired?

A

Rastelli procedure
• VSD closure, RV to PA conduit (with or without PV)
PA unifocalization (type 4) and then Rastelli procedure
Repair associated defects

30
Q

What is the outcome if a conduit is used to repair a truncus arteriosus?

A

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
Q

What should be included in the post op echo assessment of Truncus Arteriosus?

A

• 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
Q

What is complete transposition of the great arteries (d-TGA)?

A

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
Q

What is the prevalence of d-TGA?

A
  • D-TGA accounts for approx. 5% of all CHD
  • Male to female prevalence 3:1
  • Increased incidence in diabetic mothers
34
Q

What is survival with this defect dependent on?

A

• Survival is dependent on mixing of oxygenated and deoxygenated blood via shunting at the ductal, ventricular and or atrial level

35
Q

If left untreated, what is the expected natural history of a child with d-TGA?

A
  • Progressive hypoxia
  • Acidosis
  • CHF
  • Pulmonary disease (stenosis, HNT etc.)
  • 90 % of patients die before the age of six months without intervention
36
Q

What is the goal of echo in evaluating for d-TGA?

A

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
Q

How is d-TGA treated, medically?

A 
Prostaglandin E (to prevent PDA from closing) 
Treat CHF (digitalis, Lasix)
38
Q

How is d-TGA treated, surgically?

A

Jatene repair- arterial switch
Rastelli
Mustard/senning
PA banding (To buy time and protect against pulmonary HNT)

39
Q

How is d-TGA treated, catheterization?

A

Rashkind atrial balloon septostomy

Often performed in ICU under echo guidance

40
Q

What is total anomalous pulmonary venous return (TAPVR)?

A

a condition when the pulmonary veins are not connected to the left atrium, and instead enter into the right atrium

41
Q

What will always be present with TAPVR?

A

ASD

42
Q

How common is a TAPVR?

A

TAPVR is not common and accounts for approx. 1 % of all CHD

43
Q

How are the types of TAPVR classified?

A

Supra Cardiac
Cardiac
infra cardiac
Mixed

44
Q

Describe supracardiac TAPVR

A

• Supracardiac (most common approx. 50%)

Pulmonary vein confluence drains to left sided vertical vein, to innominate vein, to right sided SVC to RA

45
Q

Describe a Cardiac TAPVR

A

Cardiac (approx. 20%)

Pulmonary vein confluence drains to coronary sinus then to RA

46
Q

Describe an Infracardiac TAPVR

A

Infracardiac (sub diaphragmatic- 20%)

o Pulmonary venous confluence drains to portal vein/IVC then to RA

47
Q

Which type of TAPVR has a 4:1 male prevalence?

A

infracardiac

48
Q

Which type of TAPVR is most likely to develop obstruction?

A

infracardiac

49
Q

Which type of TAPVR has the highest mortality?

A

infracardiac

50
Q

What defects are associated with TAPVR?

A
  • Univentricular heart
  • Truncus arteriosus
  • D-TGA
  • Hypoplastic left heart
  • Asplenia/polysplenia
  • Pulmonary atresia
  • Coarctation of the aorta
51
Q

Left untreated, what is the expected natural history of someone with TAPVR?

A
  • Recurrent pneumonias
  • Pulmonary hypertension
  • Progressive congestive heart failure
  • Severe failure to thrive
52
Q

What is the goal of echo in evaluating for TAPVR?

A

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
Q

How is TAPVR treated, medically?

A

Treat symptoms of CHF
Oxygen
Prevention of recurrent infections and endocarditis

54
Q

How is TAPVR treated, surgically?

A

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
Q

What should be included in a post op echo assessment of a patient either corrected TAPVR?

A
  • 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
Q

What is tricuspid valve atresia?

A

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
Q

What is the prevalence of TV atresia and is it gender dominate?

A
  • The prevalence of TV atresia is approx. 1-3 % of all CHD

* Slight male prevalence

58
Q

What complications can arise from TV atresia?

A
  • Endocarditis (15-25%)
  • Arrhythmias (7%)
  • Brain abscess (2-5%)
  • Progressive cyanosis
  • Congestive heart failure
  • Cerebrovascular accident
59
Q

How is TV atresia classified?

A
  • 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
Q

What would you expect to see in the history and physical exam of a patient with TV atresia?

A
  • Cyanosis
  • Clubbing
  • Tachycardia
  • Anemia
  • Congestive heart failure
  • Tachypnea
  • Poor feeding/failure to thrive
  • Hypoxic spells
61
Q

What should be evaluated with echo when diagnosing TV atresia

A 
Assess for any antegrade flow through TV
Evaluate ASD for restriction
Evaluate associated defects
     Great vessel relationship
     Semilunar valve function
      VSD
62
Q

What is the medical treatment for TV atresia?

A

o Prostaglandin E1 to keep PDA open
o Digitalis and Lasix to treat heart failure symptoms
o SBE prophylaxis
o Atrial septostomy

63
Q

What is the surgical treatment for TV atresia?

A 
o	Aorta-pulmonary shunt & PDA ligation
	Blalock-Tausig, central
o	PA Band
o	Bidirectional Glenn
o	Fontan
64
Q

What should be evaluated in a post op assessment of corrected TV atresia?

A 
•	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
Q

What are some variations on a single ventricle?

A

• Pulmonary atresia with intact ventricular septum

Echo (2 decks)

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