Lecture 11 crying babies/Congenital Heart Defects Flashcards
Turner syndrome is associated with what 2 heart defects?
bicuspid aortic valve, coarctation of the aorta
Trisomy 21 (down syndrome) is associated especially with _____ (According to FA)
ASD (ostium primum)
What other trisomy’s are associated with heart defects?
13, 18
marfan’s syndrome is associated with _____
aortic root aneurysms
digeorge syndrome is associated with truncal abnormalities such as ____ and ____
truncus arteriosus, tetrology of fallot
according to pathoma, maternal diabetes is associated with what defect?
transposition of great vessels
congenital rubella syndrome is associated with _____
patent ductus arteriosus
Fetal alcohol syndrome is associated with ____ (according to pathoma)
VSD
name the 3 shunts in fetal circulation
ductus venosus, foramen ovale, ductus arteriosus
deoxygenated blood from the SVC passes through the right heart–>pulm artery–> _____ –> descending aorta
ducts arteriosus
blood entering fetus through the umbilical vein is conducted via the ______ into the IVC, bypassing the liver
ductus venosus
Most of the oxygenated blood reaching the heart from the IVC passes into the aorta via the _____
foramen ovale
at birth: first breath –> _____ in ______ of the pulmonary vasculature, causing an increase in _____ atrial pressure, closing the ____ ____
drop in resistance; left;
foramen ovale
The increase in O2 and decrease in _____ causes closure of the ductus arteriosus
prostaglandins
_____ is cyanosis of the hands and feet and is normal for the first 6 months
acrocyanosis
In acute/early cyanosis, there is hypoxia without ____. sometimes ____
hypercarbia; tachypnea
What are the 5T’s and PS associated with early cyanosis? This is due to a ___ to ____ shunt
truncus arteriosus transposition of great vessels tricuspid atresia tetralogy of fallot total anomalous pulmonary venous return (TAPVR)
pulmonary atresia
single ventricle
right to left
cyanosis is called when there is less than ___ percent saturation after 24 hours of age, or greater than ___ percent difference between upper and lower saturations
90, 3
symtpoms of congestive heart failure in infants:
_____ (RR > 60), ____ _____, and tachycardia/diaphoresis
tachypnea;
poor feeding/poor weight gain
physiological abnormalities that result in heart failure:
- increased fluid load to the heart = ______
- obstruction to ventricular emptying = increased ___
- decreased ____
- abnormal _____
preload, afterload;
myocardial contractility
rhythms
____ is the most common congenital heart defect
VSD
VSD is due to a defect in closure of the _____ _____. typically, this is the ____ portion
interventricular septum; membranous
VSD causes a ___ to ___ shunt. The shunt is determined by ____ of the defect and the ______
left to right;
size, pulmonary vascular resistance
VSD is clinically ____ at birth. By 2 weeks, a ____ murmur at the ____ may develop
normal; holosystolic murmur at the lower left sternal border
larger lesions in the VSD can cause ____ cyanosis, ____ overload, and heart failure; long term, they can cause _____
late, LV; endocarditis
what happens with small/moderate size VSDs?
close spontaneously
What is the preferred method of closure of VSD?
percutanoues closure
____ is the most common defect seen in holt-oram syndrome
ASD
the most common ASD is due to failure of the ____ ____ to close. ASD’s cause a __ to ___ shunt
ostium secundum;
left to right
ASD’s are typically _____ until 2 years of age, when noted on PE. Other symptoms include subtle ____ _____ (Recognized after repair)
asymptomatic;
exercise intolerance
what sounds are noted in patients with ASD?
loud S1,
fixed split S2
ASD repair should occur before ____ years of age, to avoid complications such as _____ embolism, atrial dysrthymias, heart failure, and pulmonary HTN
20;
paradoxical
what keeps a PDA open? if the PDA is not closed within ___, it stays open typically
Prostaglandin E2;
1 month
what is used to close a PDA?
indomethacin
initially, the murmur is ____. Later, the murmur is described as what?
systolic;
continuous, machine-like
uncorrected PDA can result in ____ cyanosis in the ____ extremeities (called ____ cyanosis). also endocarditis, heart failure, pulmonary HTN
late; lower
differential
most common coarctation of the aorta is below the origin of the ______ ____ and _____ relative to the ductus arteriosus
subclavian artery;
juxtaductal
after birth: slowing of blood flow through aorta causes ___ hypertrophy and ____. If not severe, _____ may develop. If severe, _____ cyanosis occurs
LV, HTN;
collaterals, lower extremity
with coarctation, blood pressure in the _____ extremity is elevated; blood pressure in the _____ extremity is decreased. pulses in the ____ extremity are delayed
upper, lower; lower
if the ductus closes, the infant can go into sudden ____ with CHF. post constriction hypoperfusion leads to decreased _____ and _____ of the lower extremities
shock;
oxygenation, acidosis
characteristic finding on CXR of adults with coarctation
notched ribs due to collaterals
____ should be administered to infants with coarctation. corrective surgery should follow
prostaglandin E
4 heart problems seen in tetralogy of fallot:
pulmonary infundibular stenosis, RVH, overriding aorta, VSD
tetralogy of fallot is the most common cyanotic heart defect diagnosed in _____
adults
____ is the most important determinant of prognosis in patients with tetralogy of fallot
amount of pulmonary stenosis
___ spells from exercise/stress occur because of increased ____ _____ obstruction
tet;
RV outflow
what do infants with tetralogy of fallot do during exercise to decrease their symptoms? why?
squat;
squatting increases systemic vascular resistance –> increasing pulmonary flow and decreasing the right to left shunt
characteristic finding on CXR in patients with tetralogy of fallot
boot shaped heart
in transposition of the great arteries, the aorta arises from the ____ and is ____ to the pulmonary artery arising from the ____
RV, anterior, LV
patient with transposition of great arteries requires ____, ____, or _____ to survive
VSD, ASD, patent DA
what is the name of the procedure for fixing of transposition of the great arteries ?
jatene procedure (Arterial switch operation)
