Lecture 11 crying babies/Congenital Heart Defects

Question 1

Turner syndrome is associated with what 2 heart defects?

Answer 1

bicuspid aortic valve, coarctation of the aorta

Question 2

Trisomy 21 (down syndrome) is associated especially with _____ (According to FA)

Answer 2

ASD (ostium primum)

Question 3

What other trisomy’s are associated with heart defects?

Answer 3

13, 18

Question 4

marfan’s syndrome is associated with _____

Answer 4

aortic root aneurysms

Question 5

digeorge syndrome is associated with truncal abnormalities such as ____ and ____

Answer 5

truncus arteriosus, tetrology of fallot

Question 6

according to pathoma, maternal diabetes is associated with what defect?

Answer 6

transposition of great vessels

Question 7

congenital rubella syndrome is associated with _____

Answer 7

patent ductus arteriosus

Question 8

Fetal alcohol syndrome is associated with ____ (according to pathoma)

Answer 8

VSD

Question 9

name the 3 shunts in fetal circulation

Answer 9

ductus venosus, foramen ovale, ductus arteriosus

Question 10

deoxygenated blood from the SVC passes through the right heart–>pulm artery–> _____ –> descending aorta

Answer 10

ducts arteriosus

Question 11

blood entering fetus through the umbilical vein is conducted via the ______ into the IVC, bypassing the liver

Answer 11

ductus venosus

Question 12

Most of the oxygenated blood reaching the heart from the IVC passes into the aorta via the _____

Answer 12

foramen ovale

Question 13

at birth: first breath –> _____ in ______ of the pulmonary vasculature, causing an increase in _____ atrial pressure, closing the ____ ____

Answer 13

drop in resistance; left;

foramen ovale

Question 14

The increase in O2 and decrease in _____ causes closure of the ductus arteriosus

Answer 14

prostaglandins

Question 15

_____ is cyanosis of the hands and feet and is normal for the first 6 months

Answer 15

acrocyanosis

Question 16

In acute/early cyanosis, there is hypoxia without ____. sometimes ____

Answer 16

hypercarbia; tachypnea

Question 17

What are the 5T’s and PS associated with early cyanosis? This is due to a ___ to ____ shunt

Answer 17

truncus arteriosus
transposition of great vessels
tricuspid atresia
tetralogy of fallot
total anomalous pulmonary venous return (TAPVR)

pulmonary atresia
single ventricle

right to left

Question 18

cyanosis is called when there is less than ___ percent saturation after 24 hours of age, or greater than ___ percent difference between upper and lower saturations

Answer 18

90, 3

Question 19

symtpoms of congestive heart failure in infants:

_____ (RR > 60), ____ _____, and tachycardia/diaphoresis

Answer 19

tachypnea;

poor feeding/poor weight gain

Question 20

physiological abnormalities that result in heart failure:

1. increased fluid load to the heart = ______
2. obstruction to ventricular emptying = increased ___
3. decreased ____
4. abnormal _____

Answer 20

preload, afterload;

myocardial contractility
rhythms

Question 21

____ is the most common congenital heart defect

Answer 21

VSD

Question 22

VSD is due to a defect in closure of the _____ _____. typically, this is the ____ portion

Answer 22

interventricular septum; membranous

Question 23

VSD causes a ___ to ___ shunt. The shunt is determined by ____ of the defect and the ______

Answer 23

left to right;

size, pulmonary vascular resistance

Question 24

VSD is clinically ____ at birth. By 2 weeks, a ____ murmur at the ____ may develop

Answer 24

normal; holosystolic murmur at the lower left sternal border

Question 25

larger lesions in the VSD can cause ____ cyanosis, ____ overload, and heart failure; long term, they can cause _____

Answer 25

late, LV; endocarditis

Question 26

what happens with small/moderate size VSDs?

Answer 26

close spontaneously

Question 27

What is the preferred method of closure of VSD?

Answer 27

percutanoues closure

Question 28

____ is the most common defect seen in holt-oram syndrome

Answer 28

ASD

Question 29

the most common ASD is due to failure of the ____ ____ to close. ASD's cause a __ to ___ shunt

Answer 29

ostium secundum; | left to right

Question 30

ASD's are typically _____ until 2 years of age, when noted on PE. Other symptoms include subtle ____ _____ (Recognized after repair)

Answer 30

asymptomatic; | exercise intolerance

Question 31

what sounds are noted in patients with ASD?

Answer 31

loud S1, | fixed split S2

Question 32

ASD repair should occur before ____ years of age, to avoid complications such as _____ embolism, atrial dysrthymias, heart failure, and pulmonary HTN

Answer 32

20; | paradoxical

Question 33

what keeps a PDA open? if the PDA is not closed within ___, it stays open typically

Answer 33

Prostaglandin E2; | 1 month

Question 34

what is used to close a PDA?

Answer 34

indomethacin

Question 35

initially, the murmur is ____. Later, the murmur is described as what?

Answer 35

systolic; | continuous, machine-like

Question 36

uncorrected PDA can result in ____ cyanosis in the ____ extremeities (called ____ cyanosis). also endocarditis, heart failure, pulmonary HTN

Answer 36

late; lower | differential

Question 37

most common coarctation of the aorta is below the origin of the ______ ____ and _____ relative to the ductus arteriosus

Answer 37

subclavian artery; | juxtaductal

Question 38

after birth: slowing of blood flow through aorta causes ___ hypertrophy and ____. If not severe, _____ may develop. If severe, _____ cyanosis occurs

Answer 38

LV, HTN; | collaterals, lower extremity

Question 39

with coarctation, blood pressure in the _____ extremity is elevated; blood pressure in the _____ extremity is decreased. pulses in the ____ extremity are delayed

Answer 39

upper, lower; lower

Question 40

if the ductus closes, the infant can go into sudden ____ with CHF. post constriction hypoperfusion leads to decreased _____ and _____ of the lower extremities

Answer 40

shock; oxygenation, acidosis

Question 41

characteristic finding on CXR of adults with coarctation

Answer 41

notched ribs due to collaterals

Question 42

____ should be administered to infants with coarctation. corrective surgery should follow

Answer 42

prostaglandin E

Question 43

4 heart problems seen in tetralogy of fallot:

Answer 43

pulmonary infundibular stenosis, RVH, overriding aorta, VSD

Question 44

tetralogy of fallot is the most common cyanotic heart defect diagnosed in _____

Answer 44

adults

Question 45

____ is the most important determinant of prognosis in patients with tetralogy of fallot

Answer 45

amount of pulmonary stenosis

Question 46

___ spells from exercise/stress occur because of increased ____ _____ obstruction

Answer 46

tet; | RV outflow

Question 47

what do infants with tetralogy of fallot do during exercise to decrease their symptoms? why?

Answer 47

squat; | squatting increases systemic vascular resistance --> increasing pulmonary flow and decreasing the right to left shunt

Question 48

characteristic finding on CXR in patients with tetralogy of fallot

Answer 48

boot shaped heart

Question 49

in transposition of the great arteries, the aorta arises from the ____ and is ____ to the pulmonary artery arising from the ____

Answer 49

RV, anterior, LV

Question 50

patient with transposition of great arteries requires ____, ____, or _____ to survive

Answer 50

VSD, ASD, patent DA

Question 51

what is the name of the procedure for fixing of transposition of the great arteries ?

Answer 51

jatene procedure (Arterial switch operation)