Embryology and Congenital Defects Flashcards
(probe) patent foramen ovale
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
Tetralogy of Fallot
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
eisenmenger syndrome
chronic left-to-right shunt that causes progressive pulmonary HTN that eventually reverses the shunt
truncus arteriosus
ascending aorta and pulmonary trunk
bulbus cordis
smooth parts of ventricles
primitive atrium
trabeculated part of atria
primitive ventricle
trabeculated part of ventricles
primitive pulmonary vein
smooth part of LEFT atrium
left horn of sinus venosus
coronary sinus
right horn of sinus venosus
smooth part of RIGHT atrium (sinus venarum)
right common cardinal vein an right anterior cardinal vein
SVC
fetal erythropoiesis
yolk sac: 3-8 weeks
liver: 6 weeks- birth
spleen: 10-28 weeks
bone marrow: 18 weeks to adult
ductus arteriosus
ligamentum arteriosum
ductus venosus
ligamentum venosum
foramen ovale
fossa ovalis
notochord
nucleus pulposus
umbilical arteries
medial umbilical ligaments
umbilical vein
ligamentum teres hepatis in falciform ligament
allantois-> urachus (duct between bladder and umbilicus)
median umbilical ligament
right to left shunts
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
left to right shunts
late cyanosis
freq. : VSD>ASD>PDA
1. ventricular septal defect
2. atrial septal defect,
3. patent ductus arteriosus
4. pulmonic stenosis
5. aortic stenosis
persistent truncus arteriosus
right to left shunt
fails to divide into pulmonary trunk and aorta due to lack of aorticopulmonary septum formation
most patients have VSD too
transposition of great vessels
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)
tricuspid atresia
right to left shunt
absence of tricuspid valve and hypo plastic rRV
NEED both ASD and VSD to survive
total anomalous pulmonary venous return (TAPVR)
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
ventricular septal defect (VSD)
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
atrial septal defect (ASD)
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
patent ductus arteriosus (PDA)
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
coarctation of aorta
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
infant normal stats
HR: 140
Arm BP: 65/45
pulmonary artery BP: 25/10
O2 saturation: 96-98%
pulmonary HTN of newborn
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
innocent murmurs
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
systolic murmurs
PS: pulmonary stenosis AS: aortic stenosis TR: tricuspid regurgitation MR: mitral regurgitation VSD: ventricular septal defect
diastolic murmurs
AI: aortic insufficiency
PI: aortic insufficiency
MS: mitral stenosis
TS: tricuspid stenosis
What heart problems might you see with DiGeorge?
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
Ebstein’s anomaly
anomaly of tricuspid valve
WALL to WALL heart
