RVOT, LVOT, & Aortic Arch Anomalies Flashcards

1
Q

Subvalvular obstruction involves hypertrophy of the ____ and usually occurs alongside other defects.

A

Infundibulum

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2
Q

____ obstruction may be in the form of a bicuspid pulmonary valve with varying degrees of pulmonary valve stenosis or atresia.

A

Valvular

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3
Q

Obstruction above the valve is usually in the form of ____ stenoses of the branch pulmonary arteries.

A

Discrete

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4
Q

There are two major forms of pulmonary valve stenosis:

A
  • Fusion of the raphe, often with a bi-leaflet pulmonary valve which domes
  • Severely thickened valve tissue with a tri-leaflet valve
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5
Q

____ will usually develop if RVOT obstruction is present.

A

RVH

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6
Q

The degree of RVH is dependent upon the amount of ____.

A

Obstruction

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7
Q

Most infants with an RVOT obstructions are ____.

A

Asymptomatic

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8
Q

Since the RV is not built to withstand high ____, early surgical intervention is necessary to reduce the stress on the RV.

A

Pressure

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9
Q

If there is a moderate to severe stenosis of the RVOT, they will have a loud ____ murmur.

A

Systolic

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10
Q

Pulmonary ____ is the complete obstruction of the RVOT with an atretic pulmonary valve.

A

Atresia

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11
Q

With pulmonary atresia, infants have no pulmonary blood flow except what is provided by the ____.

A

PDA

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12
Q

What are the two types of pulmonary atresia?

A
  • Those with an intact IVS
  • Those with a large VSD
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13
Q

Pulmonary atresia with intact IVS (PAIVS):

  • Associated with a poor ____.
  • Since there was not any blood exiting the RV during fetal development, the TV may be either severely ____ or ____.
  • The RV is often ____ because of the lack of circulation in utero so it’s growth is restricted
  • The RV will usually contract ____.
A
  • Prognosis
  • Stenotic/atretic; insufficient
  • Hypoplastic
  • Minimally
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14
Q

Pulmonary Atresia with Intact IVS:

  • The smaller the RV and the more hypoplastic the TV, the poorer the ____
  • There is a PFO or ____ ASD with a right-to-left shunt present (Venous return passes through PFO to get to LA and LV)
  • There must be a ____ to supply blood flow to the lungs
A
  • Prognosis
  • Secundum
  • PDA
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15
Q

____ is given to keep PDA open.

A

Prostaglandin

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16
Q

Placement of a systemic-to-pulmonary artery ____ is the most common procedure for PAIVS.

A

Shunt

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17
Q

What are most helpful views for visualizing RVOT obstruction?

A
  • PSAX view at aortic level
  • PLAX view of the RV, RVIT, and RVOT
  • Subcostal/Apical 4 view of the RV
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18
Q

What are the steps to assessing an RVOT obstruction on echo?

A
  • Assess RV size, function, and hypertrophy
  • Assess TV morphology and function
  • Evaluate PDA
  • Evaluate IVS
  • Evaluate ASD
  • Measure main PA and branches
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19
Q

An LVOT obstrcution is any lesion that impedes the forward flow of blood from the LV to the ____.

A

Aortic arch

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20
Q

What are the three main types of LVOT obstruction?

A

Subvalvular, Valvular (bicuspid or stenosis), and Supravalvular

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21
Q

The mildest form of aortic valve disease is the ____

A

Bicuspid valve

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22
Q

Speed with which bicuspid valve calcification occurs depends upon:

A
  • Severity of initial obstruction
  • Calcium metabolism/cholesterol levels
  • Gender (males more frequently)
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23
Q

Calcification of aortic valve isn’t seen in the ____ population.

A

Pediatric

24
Q

What are the three major forms of subvalvular AS?

A

Discrete, fibromuscular, and tunnel

25
Q

Discrete subaortic AS is when there is a fixed fibrous membrane located immediately ____ aortic valve.

A

Below

26
Q

Fibromuscular subaortic AS is when there is a fibromuscular ring involving ____ and ____ areas of LVOT.

A

Anterior; posterior

27
Q

____ is when there is a fibromuscular narrowing of LVOT.

A

Tunnel

28
Q

What is the most common type of subvalvular AS?

A

Discrete

29
Q

Supravalvular AS is associated with ____ Syndrome.

A

Williams

30
Q

What are some characteristics of Williams syndrome?

A
  • Abnormal facial characteristics (elfin look)
  • Low I.Q.
  • Abnormalities of calcium metabolism
31
Q

What are the three major types of supravalvular AS?

A

Discrete, hourglass, and tubular/diffuse

32
Q

Discrete supravalvular AS is when there is a fixed fibrous membrane immediately ____ the aortic valve.

A

Above

33
Q

Hourglass supravalvular AS is when there is a narrowing at the ____.

A

STJ

34
Q

Tubular/diffuse supravalvular AS is when there is a uniform narrowing of entire ____ aorta.

A

Ascending

35
Q

When assessing for an LVOT obstruction, a Doppler velocity over ____ m/s is suspicious for MS and/or AS.

A

2 m/s

36
Q

What are the three most common types of aortic arch anomalies?

A
  • Coarctation of the aorta
  • Interrupted aortic arch
  • Right aortic arch
37
Q

____ of the aorta is defined as the discrete narrowing of the descending thoracic aorta located generally just opposite the insertion of the ductus arteriosus causing LV pressure overload.

A

Coarctation

38
Q

What are the three types of coarct?

A

Preductal, juxtaductal, and postductal

39
Q

A preductal coarct is located ___ the ductus insertion.

A

Above

40
Q

A juxtaductal coarct is located ___ the ductus insertion

A

At the level of

41
Q

A postductal coarct is located ___ the ductus insertion.

A

Below

42
Q

With ____ you will usually see a different blood pressure between arms and legs.

A

Coarctation

43
Q

Use ____ to determine location and extent of coarct.

A

SSN

44
Q

Use ____ Doppler to demonstrate high peak systolic velocity across coarct.

A

CW

45
Q

What are some treatment options for coarctation?

A
  • Percutaneous balloon angioplasty and stent
  • Resection with end-to-end anastomoses
  • Dacron patch
  • Dacron graft
46
Q

Turner’s syndrome affects ____ exclusively – absence of an X chromosome.

A

Females

47
Q

Webbed neck, short stature, swelling of hands and feet, and infertility are all side effects of having ____ syndrome.

A

Turner’s

48
Q

Shone’s complex is a rare CHD complex consisting of 4 defects:

A
  1. Coarct of Aorta
  2. Valvular and Subvalvular AS
  3. Parachute MV
  4. Supravalvular Mitral Ring
49
Q

A ____ aortic arch is characterized by an absence of a segment between the aortic arch and the descending thoracic aorta.

A

Interrupted

50
Q

Interrupted aortic arch is associated with:

A

VSD, AV deformity, and subaortic stenosis

51
Q

Blood flow of an interrupted aortic arch is as follows:

A

LV > Ascending aorta > neck vessels
PA > PDA > Descending aorta > lower body

52
Q

Surgical ____ of upper and lower segments of aorta is used to treat an interrupted aortic arch.

A

Anastomosis

53
Q

A right aortic arch crosses over the right main bronchus, passing to the ____ of the trachea.

A

Right

54
Q

A right aortic arch Usually courses to the right side of the ____.

A

Spine

55
Q

Right aortic arches are most commonly seen in children who have ____.

A

TOF