Abnormalities of Ventricular Outflow

Question 1

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

Answer 1

40 weeks

Question 2

What is the typical origin to find a CoA?

Answer 2

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

Question 3

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

Answer 3

Preductal
Juxtaductal
Postductal

Question 4

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

Answer 4

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

Question 5

How does CoA affect the blood pressure?

CCI

Answer 5

Elevated upper extremity BP with lower BP in the lower extremities

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

Question 6

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

Answer 6

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.

Question 7

What is often associated with CoA?

Answer 7

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

Question 8

Congenital cardiac anomalies and shunts associated with CoA may include?

Answer 8

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

Question 9

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

Answer 9

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

Question 10

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

Answer 10

Figure “3” sign and rib notching

Question 11

CoA EKG often shows

Answer 11

LVH

Question 12

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

Answer 12

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.

Question 13

Congenital AS is more prevelant in males or females?

Answer 13

Males

Question 14

Surgical procedure used to correct AS is done via ___ procedure

Answer 14

Ross procedure
-switch PV to AV position, insert a RV-PA conduit

Can also do a balloon valvuloplasty, konno procedure (essently the ross with widening the LVOT)

Question 15

What syndrome is highly associated with supravalvular AS

Answer 15

Williams syndrome

Supravalvular AS will appear hourglass shape in the AAO in PLAX

Question 16

Bicuspid AV is highly associated with

Answer 16

CoA

Question 17

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

Answer 17

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

Question 18

Congenital cardiac defect associated with subaortic stenosis are?

Answer 18

IAA
CoA
VSD’s

Question 19

Definition of AP window and direction of shunt

Answer 19

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

Question 20

AP window can cause what affect on the heart

Answer 20

enlarged right side structures and PHTN due to volume overload

Question 21

Surgical repair usually involves?

Answer 21

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

sooner to reduce permenant damage to the lungs

Question 22

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

Answer 22

Yes, 50% of the time

• IAA, type A
• CoA
• TET
• anomalous coronaries
• AO atresia
• D-TGA
• Double AO arch

Question 23

Definition of interrupted aortic arch

Answer 23

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

Question 24

What are the three types of IAA
-percentages

Answer 24

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

Question 25

Clinical sign of IAA

Answer 25

Cyanosis of the lower extremities
tachypnea
CHF
shock

Question 26

With IAA how does the lower extremity recieve blood flow?

Answer 26

continuation of the main PA through the ductus arteriosus

Question 27

Nearly ALL IAA will have what type of shunt?
-direction

Answer 27

VSD
L-R flow

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

Question 28

What type of anastomosis is used to surgically repair IAA?

Answer 28

end-to-end

Question 29

What type of mumur is PPS

Answer 29

Systolic murmur that radiates to the back

Question 30

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

Answer 30

TR Jet
RVSP

Question 31

In PPS its important to measure the branches for ___ placement?

Answer 31

Stent/balloon angioplasty

Question 32

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

Answer 32

True

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

Question 33

What is considered abnormal pressure gradient across the Pulmonary artery?

Answer 33

gradients greater than 16mmHg

normal pressures are a third of systemic pressures

Question 34

Pulmonary artery banding purpose is to

Answer 34

used to alleviate severe pulmonary overcirculation in neonates

Tightened till pressures distal to the band are within normal range

Question 35

Definition of double chamber RV

Answer 35

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

Question 36

What is highly associated with double RV

Answer 36

VSD
subaortic stenosis

Question 37

Best view to appreciate the double RV is

Answer 37

Subcostal coronal view with an anterior tilt to the RVOT

subcostal long axis

Question 38

Definition of pulmonary atresia

Answer 38

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

Question 39

What are the two types of pulomary atresia

Answer 39

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

Question 40

In pulmonary atresia pulmonary flow is ____ dependant?

Answer 40

Ductal dependant

prostaglandins administered to keep PDA open

Question 41

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

Answer 41

myocardial sinusoids, decrease color scale to rule out

coronary artery sinusoids

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

Question 42

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

Answer 42

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

Question 43

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

Answer 43

atrial septostomy

Question 44

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

Answer 44

to be a severe form of tetralogy of fallot

Question 45

In general pulmonary atresia surgery

Answer 45

can vary drastically depending 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