Embryology and Congenital Defects

Question 1

(probe) patent foramen ovale

Answer 1

20% of people
higher pressure on left side keeps membranous flap over foramen ovale
increase right pressure above left: opens foramen ovale
deoxygeneated blood skips lungs

Question 2

Tetralogy of Fallot

Answer 2

right to left shunt
VSD, RVH, pulmonary stenosis, overriding aorta
cyanotic
tet spell: relieved by squatting
Sx: low saturation without respiratory distress, blacker than normal lungs, cyanosis, harsh systolic ejection murmur along the right upper sternal border, looks like boot

Question 3

eisenmenger syndrome

Answer 3

chronic left-to-right shunt that causes progressive pulmonary HTN that eventually reverses the shunt

Question 4

truncus arteriosus

Answer 4

ascending aorta and pulmonary trunk

Question 5

bulbus cordis

Answer 5

smooth parts of ventricles

Question 6

primitive atrium

Answer 6

trabeculated part of atria

Question 7

primitive ventricle

Answer 7

trabeculated part of ventricles

Question 8

primitive pulmonary vein

Answer 8

smooth part of LEFT atrium

Question 9

left horn of sinus venosus

Answer 9

coronary sinus

Question 10

right horn of sinus venosus

Answer 10

smooth part of RIGHT atrium (sinus venarum)

Question 11

right common cardinal vein an right anterior cardinal vein

Answer 11

SVC

Question 12

fetal erythropoiesis

Answer 12

yolk sac: 3-8 weeks
liver: 6 weeks- birth
spleen: 10-28 weeks
bone marrow: 18 weeks to adult

Question 13

ductus arteriosus

Answer 13

ligamentum arteriosum

Question 14

ductus venosus

Answer 14

ligamentum venosum

Question 15

foramen ovale

Answer 15

fossa ovalis

Question 16

notochord

Answer 16

nucleus pulposus

Question 17

umbilical arteries

Answer 17

medial umbilical ligaments

Question 18

umbilical vein

Answer 18

ligamentum teres hepatis in falciform ligament

Question 19

allantois-> urachus (duct between bladder and umbilicus)

Answer 19

median umbilical ligament

Question 20

right to left shunts

Answer 20

early cyanosis
req. urgent surgery or maintenance of PDA
start with T
1. tetralolgy of fallot
2. transposition of arteries
3. truncus arteriosus (persistent)
4. total anomalous pulmonary venous return (TAPVR)
5. tricsupid atresia
6. hypo plastic left heart

Question 21

left to right shunts

Answer 21

late cyanosis

freq. : VSD>ASD>PDA
1. ventricular septal defect
2. atrial septal defect,
3. patent ductus arteriosus
4. pulmonic stenosis
5. aortic stenosis

Question 22

persistent truncus arteriosus

Answer 22

right to left shunt
fails to divide into pulmonary trunk and aorta due to lack of aorticopulmonary septum formation
most patients have VSD too

Question 23

transposition of great vessels

Answer 23

more common in first born, male, big normal looking babies
right to left shunt
failure of aorticopulmonary trunk to spiral: aorta leaves RV and pulmonary trunk leaves LV
MUST have another defect that allows mixing of blood to survive: VSD, PDA, ASD, patent foramen ovale
Sx: cyanotic, reverse cyanosis, egg on a string CXR, LOW O2 saturation: RIGHT HAND LOWER THAN RIGHT FOOT (get some saturation due to a patent foramen oval that eventually will close)

Question 24

tricuspid atresia

Answer 24

right to left shunt
absence of tricuspid valve and hypo plastic rRV
NEED both ASD and VSD to survive

Question 25

total anomalous pulmonary venous return (TAPVR)

Answer 25

right to left shunt pulmonary veins drain right heart into circulation associated with ASD and sometimes PDA to maintain CO CXR heart in a SNOW STORM

Question 26

ventricular septal defect (VSD)

Answer 26

left to right shunt MOST common usually membranous part of septum ASYMPTOMATIC AT BIRTH and may remain asymptomatic for life larger lesions: LV overload and HF Sx: HF (growth delay and feeding intolerance), high RR and HR, head bobbing, sweats and falls asleep when feeds, low freq. holosytolic murmur across precordium (blood going from left ventricle to right), diastolic murmur in left axilla (mitral have more blood in left atrium), crackles in lungs, liver slightly large, S2 hard to hear left heart dilatation Small VSD: large systolic pressure difference between ventricles: high freq. holosystolic murmur

Question 27

atrial septal defect (ASD)

Answer 27

``` left to right shunt loud S, wide fixed split S2 Sx: none to HF types: ostium secundum defect (most common), ostium primum defect (other defects often found), sinus venosus defect, common atrium ADULT S2 SPLIT complication: emolism Tx: cath lab disc ```

Question 28

patent ductus arteriosus (PDA)

Answer 28

left to right shunt (fetal: right to left (normal then changes when lung pressure decreases at birth) machine like murmur Signs: CONTINOUS MURMUR: through systolic and diastolic wide pulse pressure (diastolic drops), pulses bounding, tachycardia, low saturation of lower extremities, tachypnea, crackles in lungs (lung congestion), cardiomegaly progressive RVH and or LVH and HF untreated: late cyanosis of lower limbs Tx: indomethacin PDE will keep open complications: less blood to organs: ischemia of intestines, intestinal necrosis, kidney insufficiency or failure; risk of endocarditis

Question 29

coarctation of aorta

Answer 29

aortic narrowing near insertion of ductus arteriosus associated with: bicuspid valve, Turner syndrome, other heart defects Sx: HTN in upper extremeties and weak delayed pulse in lower extremities, collateral arteries erode ribs (notches on xray); cool mottled extremities, delayed capillary refill pre-ductal: patent ductus arteries needed post ductal: develop collateral circulation in utero

Question 30

infant normal stats

Answer 30

HR: 140 Arm BP: 65/45 pulmonary artery BP: 25/10 O2 saturation: 96-98%

Question 31

pulmonary HTN of newborn

Answer 31

pressure too high in lungs to get blood to body; common to have tricuspid regurgitation tachycardia, tachypnea, cyanosis, hepatomegaly, crackles in lungs, loud P2, holosystolic murmur along midsternal border can get aspiration from mechonium aspiration: abnormal pulmonary parenchyma on CXR want: open PDA

Question 32

innocent murmurs

Answer 32

venous hum: at clavicle looking straight ahead, blood going down SVC (goes away when turn head or press on it) PPS: peripheral pulmonary stenosis Still's murmur: something with the left ventricle: musical near lower left sternal border and apex

Question 33

systolic murmurs

Answer 33

``` PS: pulmonary stenosis AS: aortic stenosis TR: tricuspid regurgitation MR: mitral regurgitation VSD: ventricular septal defect ```

Question 34

diastolic murmurs

Answer 34

AI: aortic insufficiency PI: aortic insufficiency MS: mitral stenosis TS: tricuspid stenosis

Question 35

What heart problems might you see with DiGeorge?

Answer 35

``` conotruncal anomalies Tetralogy of Fallot truncus arteriousus transposition of great vessels Sx: hypocalcemia, immunodeficiencies, developmental issues (speech and feeding), high arching soft and hard palates ```

Question 36

Ebstein's anomaly

Answer 36

anomaly of tricuspid valve | WALL to WALL heart