Pediatric Cardiology

Question 1

what is the most common isolated birth defect in newborns?

Answer 1

cardiac structural defects

Question 2

what maternal factors put the baby at risk for cardiac defects?

Answer 2

family hx of CHD
maternal diabetes
maternal teratogen exposure (alcohol, dilantin, rubella)

Question 3

what chromosomal abnormalities are at higher risk for CHD?

Answer 3

Trisomy 21
Trisomy 18
DiGeorge Syndrome
Turner Syndrome

Question 4

when a baby is diagnosed with a CHD, what should the next step be?

Answer 4

check chromosomes

look for other dysmorphic features or congenital abnormalities (possible syndrome)

Question 5

what are the left-to-right shunt defects?

Answer 5

VSD
ASD
PDA

Question 6

what are the right-to-left shunt defects?

Answer 6

pulmonary atresia

tricuspid atresia

Question 7

what are the obstructive defects?

Answer 7

pulmonary stenosis

aortic stenosis

Question 8

what are the mixed defects?

Answer 8

ToF
Ebstein’s anomaly of the tricuspid valve
hypoplastic left heart syndrome

Question 9

what are the “innocent murmurs”?

Answer 9

Stills murmur
pulmonary flow murmur
venous hum
carotid bruit

Question 10

what is the presentation of an ASD?

Answer 10

RA and RV enlargement
pulmonary over-circulation –> pulm HTN
usually asymptomatic

Question 11

what is heard on auscultation with ASD?

Answer 11

Grade 2-3 systolic ejection murmur
heard best at LUSB
wide fixed split S2
may have grade 1 or 2 diastolic flow murmur at LLSB

Question 12

what is the presentation of VSD?

Answer 12

surgical repair required if hemodynamically significant
smaller defects are louder and harsher
can cause CHF
may close spontaneously

Question 13

what is heard on auscultation with VSD?

Answer 13

grade 2-5 holosystolic murmur at LLSB

thrill at LLSB

Question 14

what is the presentation of PDA?

Answer 14

possibly asymptomatic

may cause fatigue, CHF and resp symptoms

Question 15

what is heard on auscultation with PDA?

Answer 15

continuous murmur
crescendo-decrescendo
grade 1-5
normal S1 and S2 may be buried in the murmur

Question 16

what is the presentation of tricuspid atresia?

Answer 16

single LV
pulmonary flow restricted
obligate ASD and VSD
early onset cyanosis or CHF

Question 17

what is heard on auscultation with tricuspid atresia?

Answer 17

holosystolic murmur at LLSB or midsternal border

murmur may be absent

Question 18

what is the presentation of pulmonary atresia?

Answer 18

obligate ASD and VSD

PDA dependent until flow can be established

Question 19

what is the presentation of aortic stenosis?

Answer 19

dyspnea, fatigue, syncope
can develop CHF
sudden death may occur

Question 20

what is heard on auscultation with aortic stenosis?

Answer 20

systolic ejection murmur at RUSB
grade 2-5
radiates to carotids
thrill at ULSB or suprasternal notch with grade 4

Question 21

what is the presentation of pulmonary stenosis?

Answer 21

usually asymptomatic

may have sx of pulmonary congestion

Question 22

what is heard on auscultation with pulmonary stenosis?

Answer 22

systolic ejection murmur at LUSB
grade 2-5
radiates to axilla
systolic ejection click may be heard at LSB

Question 23

what is the presentation of Tetralogy of Fallot?

Answer 23

VSD
overriding aorta
RVH
pulmonic valve stenosis

Question 24

what is heard on auscultation with ToF?

Answer 24

grade 3-4 long systolic ejection murmur at LUSB
may have holosystolic murmur at LLSB
systolic thrill at LUSB if 4/6
single S2

Question 25

what is Ebstein's anomaly?

Answer 25

``` displaced, leaking tricuspid valve small RV enlarged RA ASD PDA ```

Question 26

what is hypoplastic left heart syndrome?

Answer 26

underdeveloped LV underdeveloped aorta ASD PDA

Question 27

what are the staged surgeries for hypoplastic left heart syndrome?

Answer 27

1. Norwood procedure: new aorta + BT shunt 2. Bidirectional Glenn procedure; redirection of blood, BT shunt removed, ASD patched 3. Fontan procedure: ASD patch removed, baffle build in R atrium with small hole

Question 28

what is a still's murmur?

Answer 28

low-pitched "musical" LSB or between apex and LLSB normal S2

Question 29

what is a pulmonic flow murmur?

Answer 29

mid-frequency LUSB normal S2

Question 30

what is a venous hum?

Answer 30

continuous, high frequency loudest while sitting/standing heard around clavicle/base of neck goes away when supine

Question 31

who should receive a complete cardiac exam?

Answer 31

any child w/ murmur ``` infant features of CHD: FTT cyanosis feeding intolerance respiratory sx ``` ``` child features of CHD: CP on exertion syncope exercise intolerance fam hx of sudden death in young people ```

Question 32

what is a complete cardiac exam?

Answer 32

``` vitals pulses perfusion precordial inspection/palpation auscultation ```

Question 33

is splitting of heart sounds normal?

Answer 33

yes S1 split in 40-70% of people S2 split with inspiration

Question 34

is a fixed S2 split normal?

Answer 34

no - indicative of ASD

Question 35

you are examining a child for suspected CHD, when auscultating, their murmur increases in intensity when standing and again when they cough. what is your diagnosis?

Answer 35

hypertrophic cardiomyopathy

Question 36

what will you hear at the RUSB?

Answer 36

aortic stenosis | venous hum

Question 37

what will you hear at the LUSB?

Answer 37

pulm stenosis pulm flow murmurs ASD PDA

Question 38

what will you hear at LLSB?

Answer 38

``` Still's murmur VSD tricuspid valve regurgitation hypertrophic cardiomyopathy subaortic stenosis ```

Question 39

what will you hear at the apex?

Answer 39

mitral valve regurgitation

Question 40

what are the 7 S's of innocent murmurs?

Answer 40

``` sensitive - change with position or respiration short duration single small soft sweet systolic ```

Question 41

when should you refer to a cardiologist?

Answer 41

``` grade 4 murmur or above diastolic murmur increased intensity with standing symptomatic murmur obscure heart sounds weak femoral pulses clicks hyperactive precordium hx of sudden death ```

Question 42

what are the 5 cyanotic congenital heart defects?

Answer 42

One big trunk: truncus arteriosus Two interchanged vessels: transposition of great vessels Three: tricuspid atresia Four: Tetralogy of Fallot Five words: Total Anomalous Pulmonary Venous Return