Congenital Heart Disease- Gupta Flashcards
When do congenital defects occur during pregnancy?
4-9th weeks ususally
Causes of congenital defects (maternal factors)?
- infection, rubella
- alcohol
- radiation
- teratogenic drugs
What common birth defect has a strong association with congenital heart malformation?
Down’s syndrome
What heart malformations are common with Down’s syndrome?
- AV septal defect (aka Endocardial cushion defect)
- Ventricular septal defect
- Persistent ductus arteriosus
- TOF
What are some left to right shunts?
- ASD (and patent foramen ovale)
- VSD (most common)
- Patent ductus arteriosus
- AV septal defect
What is VSD associated with?
pansystolic murmur
What is a patent ductus arteriosus associated with?
rubella, continuous “machine” murmur
T or F. Cyanosis is a symptom of left to right shunts
F. It’s not.
What is Eisenmenger syndrome caused by?
prolonged left-to-right shunting with volume and pressure overloads eventually causing pulmonary HTN and secondary right-sides pressure that exceed those on the left, leading to a reversal of the shunt and the development of cyanosis
What is an ASD?
an abnormal fixed opening in the atrial septum that allows unrestricted blood flow between the atrial chambers
The majority (90%) of ASDs are so-called ____ ____ defects.
ostium secundum
What is ostium secundum ASD?
growth of the septum scandium is insufficient to occlude the second ostium
Significance of a foramen ovale?
paradoxical embolism
T or F. Most VSDs are in the muscular septum
F. 90% are in the membranous septum, and the remaining 10% are in the muscular septum
What is the ductus arteriosus?
pulmonary artery and aorta connection allowing blood entering the pulmonic circulation to be diverted to the body (lungs are high resistance, systemic is relatively low)
soon after birth, the ducts normally closes
What does a patent ductus arteriosus result in?
if the ductus remains open, there is a left-to-right shunt potentiating congestive failure
What are some right-to-left shunts?
- Tetrology of Fallot (TOF)
- Transposition of the great arteries
- Persistent truncus arteriosus
“blue babies”
What are the main characterizations of TOF?
- pulmonary stenosis
- overriding aorta (aorta can get blood from either ventricle)
- right ventricular hypertrophy
- ventricular septal defect
How does the heart appear with TOF?
boot shaped from right ventricular hypertrophy
When would a patent ductus arteriosus be helpful?
transposition of the great arteries
What is a persistent truncus arteriosis?
primitive trunks does not divide into the pulmonary artery and aorta properly resulting in a single large arterial trunk that overlies a large ventricular septal defect
accounts for 1-2%of congenital heart anomalies
What is a common cause of persistent truncus arteriosis?
35% of patients have 22q11 deletion syndrome, which includes DiGeorge syndrome and velocardialfacial syndromes
What are the two forms of aortic coarctation?
infantile form-preductal (before DA)
adult form- postductal
Aortic coarctation is more common in men or women?
Men
Aortic coarctation in women is associated with what?
Turner’s syndrome
50% of cases also have what?
bicuspid aortic valves
Impact of preductal aortic coarctation?
- typically has a PDA (unoxygenated blood to the aorta)
- dilated pulmonary trunk
- right ventricular hypertrophy
- cyanosis of lower half of the body
Impact of postductal aortic coarctation?
- no PDA
- constriction at the ligament arteriosum
- aortic arch and branch dilation
- left ventricle hypertrophy
Is high BP is the upper body and low BP in the lower body associated with pre- or postductal AC?
postductal
What is another effect of post ductal AC?
increased pressure will increase renal pressure leading rib notching (erosion of the inferior margins of the ribs)
aka Roesler sign
Is rib notching always seen?
only in long standing cases, and therefore not seen in infancy (unusual in patients under 5 y/o)
Aortic coarctation can lead to what?
atherosclerosis
Association with Down syndrome?
septal defect
Association with rubella?
PDA
Association with Turner syndrome?
coarctation of the aorta
Association with 22q11.2DS?
TOF
Association with fetal alcohol syndrome?
VSD/ASD
Association with maternal DM types I and II?
transposition of the great vessels
