Congenital Heart Disease--Severson

Question 1

What is the most common etiology of atrial septal defect?

Answer 1

90%

patent foramen ovale

Question 2

Shunt

Answer 2

abnormal communication leading to blood flow diversion

Question 3

Left to right shunt timeline

Answer 3

cyanosis several months to years after birth

Question 4

Right to left shunts timeline

Answer 4

cyanosis early in postnatal life

Question 5

Normal pulmonary artery/aortic pressure

Answer 5

1/6

Question 6

Increased pulmonary pressure secondary to shunt leads to what in the lungs?

Answer 6

pulmonary fibrosis

Question 7

Stenosis

Answer 7

narrowing of blood vessel or structure

Question 8

Atresia

Answer 8

failure of structure to develop

Question 9

What is the function of the foramen ovale in the fetus?

Answer 9

right –> left shunt in fetal heart

Question 10

Atrial septal defect etiologies

Answer 10

patent forament ovale (90%)

*excessive primary septum resorption

*incompetant ovale due to hypoplastic growth of secondary septum

Question 11

What percent of atrial septal defects involve the foramen ovale?

Answer 11

90%

Question 12

Patent foramen primum etiology

Answer 12

inadequate development of primary septum

1˚ atrial septum does not reach endocardial cushions

leaves 1˚ atrial septum patent

Question 13

High atrial septal defect etiology

Answer 13

sinus venosus defect

Question 14

Pulmonary hypertention secondary to left to right shunting can result in

Answer 14

cyanosis tardive

cardiac failure

Question 15

Hematological complication of atrial septal defects/left to right shunting

Answer 15

paradoxical embolism

Question 16

Probe patent forament ovale

Answer 16

25% people have this

fusion of primary septum does not occur

can put probe in there

clinically benign

Question 17

Ventricular septal defect normal location

Answer 17

membranous portion of the interventricular septum

Question 18

Most common congetnital heart abnormality

Answer 18

ventricular septal defect

*many heal over in ~ 1 yr.

Question 19

Ventricular septal defect etiology

Answer 19

endocardial cushion fails to respond to signals

Question 20

Cyanosis etiology in ventricular septal defect etiology

Answer 20

right ventricular failure

ineffective pumping of blood/oxygenation of blood

Question 21

Patent ductus arteriosis etiology

Answer 21

ductus arteriosis does not become ligamentum arteriosum

PGE2 keeps it open

give NSAID to decrease PGE2 synthesis that is normally discontinued when O₂ reaches tissue

if you want to keep PDA open (like in transposition of the great arteries), give PGE2 IV to keep it open

blood flows from aorta to pulmonary artery

Question 22

When does the ductus arteriosis normally close?

Answer 22

beginning day 1, usually by day 3

ligamentus structure by day 30

Question 23

What infectious disease is associated with patent ductus arteriosus?

Answer 23

rubella

in first 8 weeks

after about 8 weeks, the heart is formed

Question 24

Maternal rubella complications during first 8 weeks of pregnancy

Answer 24

PDA

congenital cataracts

deafness

pulmonary stenosis, ventricular septal defects, etc.

Question 25

Murmur in patent ductus arterioris

Answer 25

“machinery-like” murmur

Question 26

Tetralogy of fallot etiology

Answer 26

ventricular septal defect

pulmonary stenosis

overriding (large) aorta

right ventricular hypertrophy

• (cyanogic, right to left)*
• **basically: blood shunted from right ventricle into aorta because anatomically pushed into*
• **aorta is overriding interventricular septum*

Question 27

Complications of tetralogy of fallot

Answer 27

paradoxical embolism

infectious endocarditis

Question 28

Transposition of the great arteries etiology

Answer 28

aorta coming off right ventricle

pulmonary artery coming off left ventricle

*incompatible with life UNLESS atrial and ventricular septal defect and associated patent ductus arteriosus are present

*shunts save you

Question 29

Transposition of great arteries occurs more often in mothers with this condition

Answer 29

diabetes

Question 30

Persistent truncus arteriosus

Answer 30

absent aorticopulmonary septum

equal oxygenation

Question 31

Coarctation of the aorta etiology

Answer 31

contriction of aorta in area of ductus arteriosis

*aorta sometimes constricts with ductus arteriosis

*constriction ofter left subclavian, left common carotid, and right brachiocephalic artery

*body compensates for this

Question 32

Characteristic features of coarctation of aorta

Answer 32

increased BP in arms

decreased BP in legs

cyanosis in legs

increased intercostal artery size

notching of ribsm erosion of body tissue

*due to kidneys sensing low BP

Question 33

Two old types of coarctation of the aorta

Answer 33

postductal (adult–95%)

preductal (infantile–5%)

Question 34

Pulmonary/aortic stenosis and atresia etiology

Answer 34

asymmetrical division of spiralling of the aorticopulmonary septum

Question 35

Down syndrome

congenital heart abnormality association

Answer 35

atrial septal defect

Question 36

Rubella

congenital heart abnormality association

Answer 36

PDA

Question 37

Known (non-maternal) diabetes

congenital heart abnormality association

Answer 37

transposition of the great arteries

Question 38

DiGeorge Syndrome (22q11)

congenital heart abnormality association

Answer 38

persistant truncus arteriosis

Question 39

Turner Syndrome

congenital heart abnormality association

Answer 39

coarcation of the aorta