Abnormalities of Ventricular Outflow Flashcards

AS, Sub/Supra AS, CoA, interrupted aortic arch

1
Q

PDA usually closes within hours or days or a newborn who has achieved ___ weeks gestation

A

40 weeks

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2
Q

What is the typical origin to find a CoA?

A

Just distal to the left subclavian artery and just opposite to the PDA, where the natural occurring aortic shelf (aortic isthmus) is located.

Can occur at any level o the AO including the abdominal AO

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3
Q

What are the 3 types of CoA based upon the relationship of the PDA?

A

Preductal
Juxtaductal
Postductal

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4
Q

What is considered a significant pressure gradient across the Aortic Shelf and is indictive of CoA?

A

Any pressure gradient greater than 16mmHg is abnormal.

CW Doppler may show “shelfing” representing normal AO flow and higher velocity CoA flow with diastolic runoff

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5
Q

How does CoA affect the blood pressure?

CCI

A

Elevated upper extremity BP with lower BP in the lower extremities

upper pressures can be significantly higher by 10mmHg or more.

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6
Q

What is a sign of CoA when dopplering the abdominal AO?

A

When abdominal AO is non-pulsatile and blunted, always consider CoA.

A: Normal individual. B: Severe aortic insufficiency. C: Severe coarctation. D: Individual with tricuspid atresia and failed systemic venous to pulmonary artery connection (Fontan operation) demonstrating a low cardiac output and increased peripheral vasoconstriction.

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7
Q

What is often associated with CoA?

A

Bicuspid AV is present 50% of the time.
VSD
PDA

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8
Q

Congenital cardiac anomalies and shunts associated with CoA may include?

A

-PDA, ASD, VSD
-AS or LVOT obstruction
-Cor triatriatum
-D-TGA
-DORV

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9
Q

Can CoA develop in a newborn who recently underwent PDA ligation?

A

Yes, one should always interrogate the AO post PDA ligation on every exam.

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10
Q

What is a clinical sign on chest x-ray in the presence of CoA

A

Figure “3” sign and rib notching

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11
Q

CoA EKG often shows

A

LVH

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12
Q

Name the 4 surgical repairs for CoA Repair and the pros and cons of each.

A
  1. Balloon angioplasty done in the cath lab
  2. End-to-end anastomosis: 2 ends of the AO are sutured together, this is the preferred method because it preserves LSA.
  3. Synthetic Dacron graft/patch: Coarcted portion is resected and replaced with a patch, preserves LSA but at risk for AO aneurysm later on
  4. LSA Patch: LSA is excised from AO and used to repair coarct. There is less chance of rejection, however, loss of the LSA reduces the blood supply to the left arm.
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13
Q

Congenital AS is more prevalent in males or females?

A

Males

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14
Q

Two surgical repairs used to correct AS

A
  • Ross procedure: switch PV to AV position, insert a RV-PA conduit
  • konno procedure: Involves an incision from the aortic root down through the ventricular septum to widen the LVOT.
  • Can have a combinationof the two

Can also do a balloon valvuloplast

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15
Q

What syndrome is highly associated with supravalvular AS

A

Williams syndrome

Supravalvular AS will appear hourglass shape in the AAO in PLAX

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16
Q

Bicuspid AV is highly associated with

A

CoA

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17
Q

What is the leading cause of sudden unexplained death in young athletes?

A

Hypertrophic obstructive cardiomyopathy, (HOCM, HCM)
-subaortic stenosis

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18
Q

Congenital cardiac defect associated with subaortic stenosis are?

A

IAA
CoA
VSD’s

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19
Q

Definition of AP window and direction of shunt

A

rare supravalvular defect which there is direct communication between AAO and MPA superior to semilunar valves
-shunt is usually L-R

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20
Q

AP window can cause what affect on the heart

A

enlarged right side structures and PHTN due to volume overload

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21
Q

AP window Surgical repair usually involves?

A

Closing the AP window ASAP with a synthetic patch or small piece of pericardium.

sooner to reduce permenant damage to the lungs

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22
Q

Are there usually other cardiac defects associated with a AP window?

A

Yes, 50% of the time

  • IAA, type A
  • CoA
  • TET
  • Anomalous coronaries
  • AO atresia
  • D-TGA
  • Double AO arch
23
Q

Definition of interrupted aortic arch

A

Complete separation of the arch between the AAO and the DAO.

24
Q

What are the three types of IAA
-percentages

A

Type A (30-40%): distal to the LSA
Type B (55-60%): occurs between the LCC and LSA
Type C (rare; less than 5%): occurs distal to the brachiocephalic artery and proximal to LCC

25
Q

Clinical sign of IAA

A

Cyanosis of the lower extremities
tachypnea
CHF
shock

26
Q

With IAA how does the lower extremity receive blood flow?

A

continuation of the main PA through the ductus arteriosus

27
Q

Nearly ALL IAA will have what type of shunt?
-direction
Don’t think about the PDA shunt

A

VSD
L-R flow

helps increase O2 flow in the PA for the DAO segment and lower extremities

28
Q

What type of anastomosis is used to surgically repair IAA?

A

end-to-end

29
Q

What type of murmur is PPS

A

Systolic murmur that radiates to the back

30
Q

When trying to figure the pressure gradients in PPS other than the PA pressures themselves what other way can you measure the gradient?

A

TR Jet
RVSP

31
Q

In PPS it’s important to measure the branches for ___ placement?

A

Stent/balloon angioplasty

32
Q

True or false, Pulmonary branch stenosis usually occurs as a result of PHTN?

A

True

vasoconstrictive response to reduce flow to the lungs due to elevated pressures in the lungs, resolve over time if no other major complications

33
Q

What is considered abnormal pressure gradient across the Pulmonary artery?

A

gradients greater than 16mmHg

normal pressures are a third of systemic pressures

34
Q

Pulmonary artery banding purpose is to

A

used to alleviate severe pulmonary over circulation in neonates

Tightened till pressures distal to the band are within normal range

35
Q

Definition of double chamber RV

A

septated RV into a proximal and distal portion by a hypertrophied muscle bundles

36
Q

What is highly associated with double RV

A

VSD
subaortic stenosis

37
Q

Best view to appreciate the double RV is

A

Subcostal coronal view with an anterior tilt to the RVOT

subcostal long axis

38
Q

Definition of pulmonary atresia

A

cyanotic defect lacking the normal opening at the level of the PV, no antegrade flow

39
Q

What are the two types of pulmonary atresia

A

membranous: thin may be able to perforate
fibromuscular: thick muscular obstruction, requires surgery

40
Q

In pulmonary atresia pulmonary flow is ____ dependent?

A

Ductal dependent

prostaglandins administered to keep PDA open

41
Q

What should be closely evaluated within the RV muscle when pulmonary atresia is present

A

myocardial sinusoids especially in cases with intact IVS, decrease color scale to rule out

coronary artery sinusoids

could be resposible for cardiac ischemia and death following repair if not identified

42
Q

Severity of pulmonary atresia with intact ventricular septum (PA-IVS) depends on?

A

Degree of RV hypoplasia
tricuspid valve stenosis
if coronary artery sinusoids are present

43
Q

What can be done to increase pulmonary flow in the setting of pulmonary atresia?

A

atrial septostomy

44
Q

Pulmonary atresia in the setting of a large VSD is commonly considered

A

to be a severe form of Tetralogy of Fallot

45
Q

In general, pulmonary atresia surgery

A

can vary drastically depend on if the septum is intact or not

can resemble Tet repair if VSD present
or a single ventricle repair if RV hypoplastic no VSD
palliative BT shunt, then transannular patch to open the RVOT and new PV

46
Q

Left aortic arch lies to the ___ of the trachea

47
Q

The branches are part of what segment of the AO arch?

A

Transverse AO

48
Q

Right aortic arch is a ___ image of the left

A

Mirror image (turn probe slightly to the right arm)

Branches in this order:
RSA
RCCA
Left side BA

49
Q

Right aortic arch is highly associated with

50
Q

In embryology, Two primitive aortic tubes encircle esophagus and trachea forming a vascular ring. Which tube should dissipate to leave a normal aortic arch?

A

Right arch should dissipate leaving the BA, and RCCA behind. And the left arch will form as normal making the LCCA and LSA

51
Q

Double aortic arch occurs when

A

Wright aortic arch fails to dissipate during development in the vascular ring continues to circle the trachea and esophagus.

52
Q

What happens when the left arch dissipates leaving the right arch

A

The right arch becomes dominant and is a mirror image of a left aortic arch

Sometimes the origin of the branches can be affected

53
Q

In the presence of a double aortic arch, what should be determined?

A

Which arch is dominant by measuring the flow across both arches
Determine the origin of the branches