Congenital Defects Ii

Front 1

Normal direction of shunt in PDA?

Back 1

Left to right

Front 2

Sx of PDA?

Back 2

if little premie, LH failure

Front 3

Changes in heart chambers of PDA?

Back 3

LV and LA enlargement (volume overload)

Front 4

Changes in O2 sats in PDA?

Back 4

increasd O2 in PA

Front 5

CXR changes in PDA?

Back 5

enlarged LA and LV; Increased pulmonary vascularity

Front 6

Murmurs in PDA?

Back 6

Continuous murmur at Aortic region; If infant or adult with PHTN, systolic only

Front 7

Sx of PDA are dependent on what?

Back 7

the degree of L to R shunting

Front 8

Tx of PDA?

Back 8

indomethacin (inhibits PGI to foster closure), Surgical ligation and division, catheter occlusion;

Front 9

What is an endocardial cushion defect (AV septal defect)?

Back 9

Complete absence of AV septum, lets all chambers communicate, theres only one common AV valve

Front 10

Where does narrowing of the aorta usually occur in coarctation of the aorta?

Back 10

near the ductus

Front 11

What does coarctation of the aorta appear with in 50% of cases?

Back 11

other cardiac abnormalities

Front 12

2 types of narrowing in aortic coarctation?

Back 12

Discrete - abrupt focal narrowing; Tubular hypoplasia - narrowing of an entire segment;

Front 13

2 types of Aortic coarctation with regards to location of the narrowing?

Back 13

Preductal (infantile), Post Ductal (adult)

Front 14

Preductal aortic coarctation presents with symptoms when and compensates how?

Back 14

early in life, increases blood flow through a PDA

Front 15

Post ductal aortic coarctation presents with Sx when and how does it compensate?

Back 15

adulthood, collateral circulation through intercostal aa's and mamaries

Front 16

Chamber changes in coarcation of the aorta?

Back 16

LV hypertrophy (pressure overload)

Front 17

CXR findings in coarctation of the aorta?

Back 17

LV hypertrophy, notching of ribs

Front 18

Murmurs in coarctation?

Back 18

systolic ejection murmur, (best heard between scapulae)

Front 19

PE findings in aortic coarctation?

Back 19

upper extremity hypertension, late diminished femoral pulses

Front 20

Best method for dx of coarctation?

Back 20

MRI, (can also use transesophageal ultrasound)

Front 21

Tx of aortic coarctation?

Back 21

open with balloon, place stent

Front 22

4 morphological features of tetrallogy of fallot?

Back 22

VSD, Aorta overriding the VSD, Obstruction of right vent outflow, RV hypertophy;

Front 23

Type of shunt in tetrallogy?

Back 23

right to left, (can be bidirectional, depends on severity of RV outflow obstruction)

Front 24

Pressure abnormalities in tetrallogy?

Back 24

RV, LV and aortic pressures all equal

Front 25

Heart sound abnormalities in tetrallogy?

Back 25

Diminished or inaudible P2 (from pulm stenosis)

Front 26

Murmur in tetrallogy?

Back 26

Systolic ejection murmur in pulm area from PS

Front 27

CXR findings of tetrallogy?

Back 27

RV hypertrophy, diminished pulm vasc

Front 28

EKG findings of tetrallogy?

Back 28

RVH, RAD

Front 29

Tx of tetrallogy?

Back 29

Total corrective surgery; Palliative - blalock taussig subclavian to PA anastomosis

Front 30

What is a congenitally corrected transposition of the GA's?

Back 30

Atria and arteries switched; Essentially your just using the wrong ventricles to pump

Front 31

What is necessary for an infant to survive with uncorrected transposition of the GA's?

Back 31

some type of shunt

Front 32

Sx of uncorrected transposition?

Back 32

severe hypoxemia, HF

Front 33

Heart sound findings of TGA?

Back 33

loud S2 (RV doing systemic), RV heave;

Front 34

CXR findings of TGA?

Back 34

Increased vascularity, Cardiomegaly if large shunt

Front 35

EKG findings of TGA?

Back 35

RVH, RAD

Front 36

Dx of TGA usually made with what?

Back 36

Echo

Front 37

Tx of TGA?

Back 37

Palliative - give PGIs to keep ductus open, make an ASD with a balloon to allow mixing; Corrective - surgery

Front 38

Valvular atresia may be accompanied by what other symptom?

Back 38

hypoplasia of the heart on the affected side

Front 39

What type of VSD will give you the biggest murmur?

Back 39

a small one

Front 40

VSD's need to be repaired before what age to prevent eisenmengers?

Back 40

age 2

Front 41

Kids with tetrallogy might do what and why?

Back 41

squat, increases venous return

Front 42

When will you hear a murmur with a VSD and when will you not?

Back 42

you'll hear one if the theres a high prssure diff on each side, otherwise no

Front 43

What is the basic concept of anomalous pulmonary venous return?

Back 43

Pulmonary veins drainging into systemic circulation

Front 44

Down syndrome associated with what heart defects?

Back 44

AV septal

Front 45

Turner syndrome associated with what heart defects?

Back 45

Left sided obstructive

Front 46

Williams syndrome associated with what heart defects?

Back 46

supravalvular AS

Front 47

Digeorge syndrome associated with what heart defects?

Back 47

outflow tract problems

Front 48

Holt oram syndrome associated with what heart defects?

Back 48

ASD/VSD

Front 49

Noonan syndrome associated with what heart defects?

Back 49

PS