Congenital Pathology (Blue book)

Question 1

congenital defects early on will usually have a shunt of

Answer 1

left to right

due to the more powerful left side of the heart dominating

Question 2

later as pulmonary distance develops, the pulmonary vascular changes the shunt to

Answer 2

right to left shunt

Question 3

defects that are shunting right to left are usually inoperable because..

Answer 3

the right ventricle will not be able to pump against the increased pulmonary resistance and will fail

Question 4

corrective surgery for ASD

Answer 4

ASD closure

Question 5

3 types of ASD

Answer 5

1. sinus venous
2. ostium secundum
3. ostium primum

Question 6

sinus venosus details

Answer 6

found high in atrial septum near the SVC

associated with partial anomalous pulmonary venous drainage

Question 7

ostium secundum details

Answer 7

found in the central portion of the atrial septum in the fossa ovals

most common type of ASD

Question 8

what is the most common type of ASD

Answer 8

ostium secundum

Question 9

ostium primum details

Answer 9

found low in the septum

• also known as partial or complete AV canal
• type of endocardial cushion defect

Question 10

CorTriatriatum

Answer 10

a third atrium is created by a septum dividing the left atrium into two chambers

Question 11

what is surgical correction of corTriatriatum

Answer 11

removal of the septum dividing the chambers

Question 12

what are VSDs

Answer 12

openings between the left and right ventricle

Question 13

what is surgical correction of VSD

Answer 13

closure of the VSD

Question 14

what is a complication of VSD closure

Answer 14

complete heart block

Question 15

4 types of VSDs

Answer 15

type I - subarterial, aortic
type II - perimembranous VSD, most common
type III - inlet VSD
type IV - muscular VSD

Question 16

what is the PDA

Answer 16

connection between the descending aorta and the left pulmonary artery

Question 17

what is coarctation of the aorta

Answer 17

narrowing of the aorta that may occur at any location

• most located proximal descending aorta
• bicuspid aortic valve is often present

Question 18

corrective surgery of coarctation of the aorta

Answer 18

excision of the coarctation with end to end anastomosis if possible

Question 19

what is idiopathic hypertrophic subaortic stenosis

Answer 19

reduced size of the outflow tract from the left ventricle due to excessive muscular tissue

• caused by the presence of a membrane inside the left ventricle partial blocking flow through the valve

Question 20

what is aorto-pulmonary window

Answer 20

opening made between the aorta and the pulmonary artery characterized by a large left to right shunt

Question 21

what is truncus arteriosus

Answer 21

aorta and PA both come off the same trunks which has only one ventricular valve

-VSD must be present

Question 22

what is the corrective surgery for truncus arteriosus

Answer 22

rastelli procedure

• closure of the VSD with a valved conduit from the right ventricle to the PA. this will divert all blood from the left ventricle to the aorta and stops all flow from the right ventricle to the aorta

Question 23

what is transposition of great arteries?

Answer 23

aorta comes off the right ventricle and the pulmonary artery comes off the left ventricle, behind the aorta

Question 24

what must be present in TGA

Answer 24

VSD, ASD, or PDA

Question 25

corrective surgeries for TGA

Answer 25

1. sinning procedure and mustard procedure: both atrial partitioning procedures. fix pulmonary venous blood from the lungs to go to the RV and systemic venous return go to the LV
2. jatene procedure: switches PA and AO by making transverse incisions through both vessels and reattaching each to the opposite vessel
3. LeCompte maneuver: switches PA and AO, leaving the PA and its right and left branches in front of the AO

Question 26

what is TAPVR

Answer 26

total anomalous pulmonary venous return

oxygenated blood from the lungs returns to the right side of the heart
- ASD must be present

Question 27

what are the three types of TAPVR

Answer 27

1. supra cardiac: oxygenated blood enters a left superior vena cava, innominate vein and then the SVC (MOST COMMON)
2. cardiac: oxygenated blood enters the coronary sinus.
3. infra cardiac: pulmonary veins combine into a venous trunk that goes into the IVC or portal vein.