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ALU 301 > Chapter 13- Congenital Heart Disease > Flashcards

Chapter 13- Congenital Heart Disease Flashcards

1
Q

There are many varieties of structural defects and combinations. What 8 conditions account for 80% of congenital heart disease casses?

A

3 obstructions: pulmonary stenosis (PS), aortic stenosis (AS) and coarctation of the aorta (COA).
Tree left-> Right shunts: atrial septal defect (ASD), ventricula septal defects (VSD), and petent ductus arteriousus (PDA)
two complex cyanotic conditions: Tetralogy of Fallot (TF) and transposition of the great arteries (TGA_

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2
Q

What congenital heart defects remain undetected until adulthood?

A

Atrical septal defect (ASD) and mild aortic valve disorders

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3
Q

Define atrial septal defect (ASD)

A

hole in the septum betweewn the Rt and Lt atria.

- significance depends on lcoation, size and presence of other congenital heart lesions.

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4
Q

What is the most common ASD, and where is it located?

A

Scandium ASD

- located in the center of the septum.

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5
Q

What are the restrictions to considering surgical intervention for repairing Scandium ASD?

A

<5mm is followed
5mm- 3.0cm closed by catheter

Closure is recommended prior to age 6 to avoid perm enlargement.

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6
Q

What is the risk to completing a closure of a large atrial defect after age 20?

A

25% change of developing atrial Fib over the next 30 years.

persisten Rt atrial/Ventrical and pulmoanry artery enlargement.

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7
Q

What is the second most common ASD and where is it located?

A

Primum ASK,

located in the Lower part of septum - usually associted with a celft mitral valve.

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8
Q

What are the restrictions for an unoperated premium atrial septal defect?

A

small defect with minimal MR is 200%.
Most require surgical repair.
- some MR progresses and pt requires valve repair or replacement 5-50 yrs after first operation

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9
Q

What are the risks with repaired primum ASD?

A
  • risk of residual mitral regurgitation.
  • with no complications mortality is 100-150%
  • severe MR require future valve replacement /repair.
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10
Q

What is the least common ASD and where is it located?

A

Sinus Venosus ASD,

located in upper portion of the septum.

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11
Q

What are the risks for repaired Sinus Venosus ASD?

A

concern: majority of pts will have the upper pulmoary vein connected to super vena cava.
Sick sinus syndrome can develop and pacing might be needed.
with no residuals, mortality is 100-150%.

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12
Q

What is a ventricual septal defect (VSD)?

A

a hole in the septum between the right and left ventricles.

- can be isolated or occur with other lesions.

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13
Q

What is a small VSD?

A

<3mm, with normal Rt ventricular and pulmoanry artery presures and small Left-to-right shunt.
if flow <20% then mortality is close to 100%.
- 50% of infant cases repair spontaneously.

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14
Q

What is a moderate VSD?

A

Rt vent and pulm art pressure 30 to 50 mmHg/

  • shunt flow modest-large.
  • some will shring, Asxs children are just followed.
  • long term mortality usually 150%
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15
Q

What is a large VSD?

A
  • unrepaired mortality is 500%
  • repaired should have occured in childhood.
  • To be considered there needs to be a holter and possible stredmill test showing reasonable exercise capacity and normal Rt ventricular pressure. NO AORTIC regurg.
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16
Q

What Eisenmenger syndrome?

A

pulmonary huypertension complication from any congenital heart disease with a left-to-right shunt.

  • commonly associated with VSD.
  • if the shunt reverses, this can result in cyanosis and digital clubbing.
    tx: vasodilator medication.
  • seldom survive >50 yo.
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17
Q

What is PAtent Ductus Arteriosus (PDA)

A

condition in which the ductus arteriosus (DA) ie: the fetal connection between the aortia and the main pulmonary artery, fails to close normally after birth.

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18
Q

How are PDAs treated?

A

catherter intervention.

- mortalityt: normal within few months of tx.

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19
Q

What is Isolated valvular pulmonary stenosis (PS)?

A

narrowing at the level of the pulmonic valve causing obstruction of blood flow from the Rt ventricle into the pulmonary artery.
- can occur alone or with congenital heart defects.

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20
Q

Define mild PS

A

peak gradient across the valve is 10-30mmHG.

- usually never intervented.

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21
Q

Define moderate PS.

A

peak gradient 31-50 mmHg.

  • usually no sxs,
  • usually tx’ed with balloon valvuloplastic
  • mortality 125-175%, but if gradient reduces to 20 mmHG then its closer to normal.
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22
Q

Define moderate-severe PS

A

Peak gradient is 50-80 mm HG,

  • few cases without surgical intervention via valvotomy and balloon Valvuloplastic.
  • some pt may require a second intervention.
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23
Q

What is noonan’s syndrome?

A

the valve is dysplastic and stretches, rather than splits, in response to balloon valvuloplastic.
- common genetic disorder.

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24
Q

What is Severe PS?

A

pinhole valve at infancy, requires immediate relief of stenosis for survival.
- might be foramen ovale with Rt-2-Lt shunting causing Cyanosis.

25
Q

What are the favourable factors to consider 2-3 years after intervention to treat severe PS?

A
  1. residual gradient < 30mmHG
  2. Good Rt ventricular function.
  3. no Rt-2-Lt shunt at the foramen ovale
  4. no significant tricuspid regurgitation
  5. No arrythmia
26
Q

What is a Patent Foramen ovale (PFO)?

A

common finding, specially in children with PS.

mild changes in Rt ventricular function lead to great Rt-2-Lt shunting at atrial level.

27
Q

What is subvalvular pulmonary stenosis?

A

Rt ventricular outflow is obstructed by hypertrophied muscle bundles below the pulmonic valve.
- open heart surgery to repair, when gradient >40 mmHG.

28
Q

What is Aortic Valve stenosis (AS)

A

narrowing of the aortic valve that prevents the valve from opening fully and obstructs blood flow from the Lt ventricle into the aorta.
- usally prognosis worsens as pt ages.

29
Q

True or False
Congential AS is valvular in 90% of cases
subvalvular in 9% of cases and supravalvular in <1% of cases.

A

True

30
Q

A normal aortic valve has 3 leaflets of cusps. Valvular AS has what?

A

a fusion of leaflets resulting in a bicuspid valve or unicuspid valve structure.

31
Q

Are the risk associted with a bicuspid aortic valve

A

BAV occurs with individuals with no sig stenosis or mild or trival aortic regurg/
- main problem that requires intervention would be bacterial endocarditis.’

32
Q

What is a significant variable with a BAV?

A

Dilation of the ascending aorta, - part of BAV complex.

- increases risk of sevre regurg, and thus aortic dissection.

33
Q

What is considered mild, moderate, moderate-severe, severe Valvular AS in children?

A 
mild : 15-30mmHG
moderate: 20-50 mmHG
moder/sever: 50-80mmHg
Severe: >80mmHG. 
** disease gradually worsens with age.
34
Q

How is Valvular AS treated in children?

A

usuallly ballon valvuloplasty,
open heart surgery.
> residual usually ends up thickening aortic valve, future valve replacement may be required.

35
Q

What is Subvalvular aortic stenosis?

A

aortic valve is usually normal, but secondary changes lead to some regurgitation.

  • narrowing just under aortic valve.
  • at 30% risk of recurrent stenosis or incomplete removal
36
Q

How is Subvalvular aortic stenosis treated?

A

When pt is Sxs, and peak flow >40 mmHg or theres LVH.

- 1/3 of pt usually require second op in 20 years, and possible valve replacement.

37
Q

What is supravalvular aortic stenosis?

A

rare,

  • diaphragm above the aortic valve, narrowing or hypoplasia.
  • common lesion in Williams syndrome.
38
Q

What is Williams Syndrome?

A

Rare genetic disorder

Pt have a typical facial appearance with saddle shape nose, dental abnormalities and moderate retardation

39
Q

What is Coarctation of the Aorta (COA)?

A

narrowing of the thoracic aorta that causes obstruction of blood flow through the aorta. Can be associated with bicuspid aortic valve, Turner Syndrome, and intracerebral aneurysm.
- 60% of BAV pt have COA.

40
Q

How is COA treated?

A

Surgically with ballon angioplasty. some require grafts.
best age for surgical repair of Asxs is 4 years hold.
- most cases need intervention, rarely is it mild enough to leave along.
- goal is to drop pressure across the coarctation segment

41
Q

What is the mortality of a COA history?

A

long term follow-up required, and usually is 250-500% after age 35. deaths d/t CHF, TIA, MI and aortic rupture/dissection.

42
Q

Some ideal cases of COA are likely to have long-term motality close to normal. What requirements need to be met for this assessment?

A
  1. normal aortic valve, or if bicuspid no stenosis or regurg.
  2. Arm/Leg systoliv pressure difference is <10 mmHg
  3. arm BP
43
Q

What are the anatomic changes of the Tetralogy of Fallot (TF)?

A
  1. ventricular septal defect
  2. Rt ventricular outflow obstruction
  3. anterior displacement of the aorta (overriding aorta)
  4. Rt Ventricular hypertrophy (RVH).
44
Q

What are the two main defects of TF?

A 
large VSD (diameter >50%) 
RV flow obstruction.
45
Q

RV outflow can be subvalvular PS, both subvalvular and valvular PS or only valvular PS. How is the severity determined?

A

by severity of PS.

- mild PS may not be cyanotic.

46
Q

How is TF repaird?

A

Childhood intervention : closing the VSD and relief of the RV outflow tract obstruction.

47
Q

Some adult have TF treated with palliative surgery and not reapired. What does this mean?

A

procedure including: the Blalock-Taussing Waterson, and Potts shunts or Gortex grafts from the aorta to the pulmonary artery.
- may require multiple interventions.

48
Q

What factors should be considered in UW pt with TFs?

A
  1. age at repair (6months = good, unless mild then 6-10 yo)
  2. size of pulmonary arteries.
  3. outflow path across the pulmoary valve ring
  4. marked Rt ventricular dilation from severe Pulm/tricupsid regurd.
  5. Arrythmias-
  6. Presence of Rt Bundle Branch Block and Lt anterior Hemiblock.
  7. Several anatomic and repair variations that have an impact on the outlook-
49
Q

What occurs in the Transposition of the great arteries (TGA)?

A

the aorta and pulmonary artery are reversed. Aorta arises from the Rt ventricle carrying un-oxygenated blood to the body.. The pulmonary artery arises from the Lt ventricule taking blood with O2 back to the lungs.

  • Bi-directional shunting occurs at the forament ovale and/or a VSD.
  • biggest risk is arrhythmias and heart failure.
50
Q

Early survival for TGA has improved d/t Rashkind Ballon Atrial septostomy (BAS) what is this?

A

surgery preformed in infants to enlarge the foramen ovale by sharply pulling a balloon catheter from the Lt to Rt atrium.

51
Q

The tx for TGA from 1966 to 1980 was done via atrial Switch operations. There were two of these atrial operations. Define them.

A
  1. Senning operation-
  2. Mustard operation

There were VERY poor prognosis and ++ surgical complications surfacing 10 years after the tx.

52
Q

Noting the failure with atrial Switch operations, they changed the tx of TGA to Arterial Switch Operations, in late 1980’s.

A

needs to be done in first 2 weeks of life.
- aorta is moved from the top of the Rt ventricle to the Lt ventricle.
Coronary arteries are also moved. Pulmonary artery is moved from top of the Lt ventricule to Rt venticle.
Ventricular function is preserved.
- 25% of pt develpp MR,
- complication is future coronary artery complications.
- procedure is only 25 yo.

53
Q

What is Tricuspid Atresia?

A

complete blockage of the tricuspid valve.

  • surgical correction includes aortic to pulmonary artery shunt, superior vena cava connection to the Rt pulmonary artery, or a fontan operation.
  • survial >50 yo is rare.
54
Q

What is Pulmonary Atresia with VSD?

A

Here the Rt ventricular connection to the pulmonary artery is blocked.

  • severe form of TF.
  • If pulmoary arteries are not a reasonable size surgery cant take place.
55
Q

What is Pulmonary Atresia with Intact septum

A

Rare cyanotic lesion with pulmoary valve obstuction, variable developement of Rt ventricle and small tricuspid valve.

  • most cases have sevre pulm regurg unless they get valve replacement.
  • mortality 300% in those >20 yo.
56
Q

What are Aortic and Mitral Atresia?

A

lesions are the basis for hypoplastic left heart syndrome.

- 60% of pt reach 16 yo, but most dont make 50.

57
Q

What is a univentricular Heart - single Ventricle?

A

these cases are often palliated with Fontan operation, wehre Rt atrium or vena cava is connected difrectly to the pulm artery and the single ventricle ejects bloo through aorta only.
- 500%

58
Q

What is a Fontan Operation

A

used to palliate cases of tricuspid atresia and sometimes Rt ventricular disorders (ebstein maldormation of the tricuspid valve and univentricular hearts).

  • superior vena cava is connected to the Rt pulmonary artery and a lateral conduit or tunnel, directs inferior vena cava bloog to the pulmonary artery.
  • good for number of years after operation but then mortality is usually 500%

ALU 301 (14 decks)

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