Congenital Heart Dz

Question 1

VSD

Answer 1

Most common cause of congenital heart dz.

Question 2

Apert’s Syndrome

Answer 2

VSD. Cranial deformities. Fusion of fingers and toes

Question 3

Down’s Syndrom

Answer 3

VSD, ASD

Question 4

Fetal Alcohol Syndrome

Answer 4

VSD, ASD

Question 5

TORCH

Answer 5

Toxo. Other (syphillis), Rubella, CMV, HSV.

Question 6

Cri du chat

Answer 6

VSD. 5p deletion

Question 7

Trisomies 13 and 18

Answer 7

13 - Patau
18 - Edwards
VSD

Question 8

Harsh, holosystolic murmur. Heard best at lower, left sternal border

Answer 8

VSD

Question 9

Frequent resp infections, dyspnea, FTT, CHF. Softer, more blowing murmur. systolic thrill, crackles, hepatomegaly, narrow S2, inc P2. Mid-diastolic apical rumble (inc flow across mitral valve).

Answer 9

Large VSD

Question 10

VSD diagnosis

Answer 10

• ECHO
• EKG = LVH (maybe LVH and RVH)
• CXR = cardiomegaly, Inc pulm vasc markings

Question 11

VSD tx

Answer 11

Small - most close spon (monitor with ECHO), abx only if previous closure with prosthetic material.
Surgery - if fail medical management, children <1 with PHTN, older children with persistent, large VSD
Med Management - CHF w/ diuretics, inotropes, ACEIs

Question 12

ASD, absent radii, first degree heart block

Answer 12

Holt-Oram Syndrome

Question 13

Fixed, widely-split S2. right ventricle heave

Answer 13

ASD

Question 14

Eisenmenger’s Syndrome

Answer 14

Right-to-Left shunting, cyanosis.

Leads to PHTN and shunt reversal.

Question 15

ASD EKG

Answer 15

RVH. R atrial enlargement. PR prolongation.

Question 16

ASD CXR

Answer 16

cardiomegaly. Inc pulm vasc markings

Question 17

ASD tx

Answer 17

90% close spon

Question 18

ASD surgical repair

Answer 18

infants with CHF. Pt with more than 2:1 pulm to systemic blood flow

Question 19

Cont “machinery murmur” @ 2 LICS. Loud S2. Wide pulse pressure. Bounding peripheral pulses.

Answer 19

PDA

Question 20

PDA dx

Answer 20

color flow Doppler, showing flow from aorta to pulm artery

Question 21

PDA tx

Answer 21

indomethacin (unless PDA is needed for survival or indomethacin is contraindicated, IV hemorrhage)
-If indomethacin fails or child is >6-8mo, surgical closure is required.

Question 22

Coarc - most common site

Answer 22

just below the subclavian (98%)

Question 23

Coarc pts with bicuspid aortic valve

Answer 23

2/3

Question 24

Murmur over the back, between the scapulae

Answer 24

Coarc

Question 25

CXR - "3" sign and rib notching

Answer 25

Coarc

Question 26

Coarc tx

Answer 26

PGE1 - keeps ductus open | Monitor for restenosis, aneurysm, and dissection

Question 27

Most common cyanotic congenital heart lesion in a newborn

Answer 27

Transposition of the great vessels

Question 28

CATCH 22

Answer 28

DiGeorge Syndrome ``` Cardiac abnormalities (transposition) Abnormal facies Thymic aplasia Cleft palate Hypocalcemia ``` 22q11 deletion

Question 29

Transposition s/s

Answer 29

Cyanosis within first few hours | tachypnea, progressive hypoxemia, extreme cyanosis

Question 30

Transposition dx

Answer 30

ECHO | CXR - narrow heart base, "egg-shaped silhouette" and Inc pulm vasc markings

Question 31

Transposition tx

Answer 31

PGE1 - maintain PDA Balloon septostomy Surgical correction is definitive

Question 32

Tetralogy of Fallot - components

Answer 32

Pulm stenosis, overriding aorta, RVH, and VSD

Question 33

Most common cyanotic congenital heart disease in children

Answer 33

Tetralogy of Fallot

Question 34

Tetralogy - risk factors

Answer 34

maternal PKU, DiGeorge Syndrome

Question 35

Tetralogy - H/PE

Answer 35

Freq absent at birth - dev over first 2yrs of life | 4-6 mo: CHF, manifests as diaphoresis with feeding or tachypnea

Question 36

Child squats for relief during hypoxemic episodes

Answer 36

Tet spells | squatting INC systemic vascular resistance

Question 37

Tetralogy dx

Answer 37

ECHO and catheterization CXR - boot-shaped heart, dec vasc markings ECG - RVH and r-axis deviation

Question 38

"Boot-shaped" heart with DEC pulm vasc markings

Answer 38

Tetralogy

Question 39

Tet spell tx

Answer 39

O2, propranolol, phenylephrine, knee-chest position, fluids, morphine

Question 40

Tetralogy tx

Answer 40

PGE1, surgery