Lecture 8 "Congenital Heart Disease"

Question 1

what percentage of CHDs are caused by chromosomal abnormalities?

Answer 1

10%

Question 2

what are the two chromosomal diseases associated with CHDs?

Answer 2

Turner syndrome

DiGeorge syndrome

Question 3

what are four symptoms of CHD in neonates?

Answer 3

dyspnea
poor feeding
poor growth
heart murmur

Question 4

at what diameter is an ASD not a problem

Answer 4

< 0.5 cm

Question 5

at what ASD diameter does L to R flow start

Answer 5

2 cm

Question 6

by what age does a PFO close

Answer 6

6 months

Question 7

what is the most common CHD

Answer 7

ventricular septal defect

Question 8

in a VSD, when pulmonary vascular occlusive disease (PVOD) occurs, what happens to flow?

Answer 8

it goes R to L causing a cyanotic lesion

Question 9

between what two vessels is a PDA?

Answer 9

pulmonary artery and aorta

Question 10

what is a murmur like for a PDA?

Answer 10

continuous and machine-like

Question 11

what medication is used to close a PDA?

Answer 11

indomethacin

Question 12

what induction agents are used in the NICU for patients undergoing a VATS for PDA repair?

Answer 12

pancuronium and ketamine

Question 13

narrowing of the descending thoracic aorta is known as what?

Answer 13

coarctation of the aorta

Question 14

what disease is coarctation of the aorta commonly associated with?

Answer 14

turner syndrome

Question 15

if uncorrected, coarctation of the aorta will lead to what?

Answer 15

LVH

Question 16

how is coarctation of the aorta treated?

Answer 16

keeping the PDA open with PGE1

surgical excision of the area with end-to-end anastomosis

Question 17

what are the four components of tetralogy of Fallot?

Answer 17

ventriculo-seltal defect
subvalvular pulmonic stenosis
overriding aorta
RVH

Question 18

what is a physical sign of a chronic cyanotic lesion?

Answer 18

clubbing of fingernails and toenails

Question 19

what does the VSD in TOF not cause a murmur?

Answer 19

it is too large

Question 20

what do spells of systemic vasodilation cause in TOF?

Answer 20

R to L flow

Question 21

how do you treat spells of systemic vasodilation in TOF?

Answer 21

100% O2
compress femoral arteries
morphine for pain
fluid bolus 15-30 cc/kg
NaHCO3 for acidosis
phenylephrine infusion

Question 22

what drugs do you not want to give a patient with TOF?

Answer 22

beta blockers

Question 23

how does O2 saturation compare in the pulmonary and systemic vascular beds if a patient has single ventricle physiology?

Answer 23

it will be the same in the two vascular beds

Question 24

hypoplasia of the LA and LV leading to single ventricle physiology is known as what?

Answer 24

hypoplastic left heart syndrome (HSLS)

Question 25

in HSLS, systemic perfusion is dependent on what?

Answer 25

PDA flow, keep it open with PGE1

Question 26

what are the three surgical sages of HSLS?

Answer 26

Norwood Glenn Fontan

Question 27

what is it called when the aorta comes off the RV, and the pulmonary artery comes off the LV?

Answer 27

transposition of the great arteries

Question 28

why is transposition of the great arteries a big problem?

Answer 28

it places the two circulations in parallel, not in series

Question 29

how is flow maintained in transposition of the great arteries?

Answer 29

PDA and PFO are kept open

Question 30

if a neonate is born with a chronic saturation around 70% what is the suspected CHD?

Answer 30

transposition of the great arteries

Question 31

what is Eisenmenger syndrome?

Answer 31

large L to R flow leading to pulmonary HTN and ultimately R to L flow, creating a cyanotic lesion

Question 32

what are three symptoms of Eisenmenger syndrome?

Answer 32

dyspnea on exertion pulmonary edema cyanosis

Question 33

how is Eisenmenger syndrome treated?

Answer 33

``` repair of lesion pulmonary vasodilator (prostacyclin, PDE-5 inhibitor, endothelin receptor antagonist) ```

Question 34

what are the hematological effects of a CHD?

Answer 34

polycythemia and abnormal coagulation