Congenital Heart Disease Flashcards
What are the most common cardiac malformations?
ventricular septal defect 42%
atrial septal defect 10%
pulmonary stenosis 8%
PDA &%
Name the 3 main causes (all genetic) that cause congenital heart malformations.
single gene mutations
small chromosomal deletions
whole chromosome abnormalities
Name the 2 physiologic shunts of the fetus.
(right to left shunts)
foramen ovale
ductus arteriosus
What causes physiologic closing of the PDA?
prostaglandins that are normally produced in the placenta are not produced after birth and the neonatal lungs metabolize PG as well
How can pulmonary vascularity serve as clue regarding neonatal fetal shunting?
vascularity is increased with left to right shunting (greater pulmonary pressures- can be present without cyanosis)
pulmonary vascularity may be decreased with right to left shunting (decreased pulmonary pressures)
List the cyanotic heart defects.
Terrible T's: Tetralogy of Fallot Trunks Ateriosus Tricuspid Atresia Total Anomalous Pulmonary Venous Connection Transposition of the Great vessels
Explain Eisenmenger’s Syndrome.
with initial fetal left to right shunt, you can get reversal of the shunt due to increased pulmonary pressures (causing cyanosis in adults)
What are the 4 components of Tetralogy of Fallot.
**subpulmonic stenosis
RVH
Ventral septal defect
overiding aorta
clinically look for a “boot shaped heart” and decreased pulmonary vasculature
What are the clinical features of Tetralogy of Fallot?
usually apparent by 6mo dyspnea, cyanosis polycythemia which may present as a cerebral thrombosis infective endocarditis (reduced survival without treatment)
List the left to right congenital heart defects.
the D’s
atrial septal defect
ventricular septal defect
patent ductus arteriosus
VSD, the most common congenital heart lesion is associate with what etiology?
associated with all the trisomies
90% are membranous, less likely to close spontaneously (unlike 50% of small muscular defects)
What are the clinical features of VSD?
pulmonary HTN, CHF, pansystolic murmur
reversal can lead to cyanosis and Eisenmenger complex
What is the most common congenital cardiac malformation diagnosed in adults?
atrial septal defect
represents 10% of congenital septal defects
Contrast the location of osmium secundum and esteem primum defects.
ostium primum defects are low
ostium secudium defects are usually in the middle of the septum
Describe the classic murmur of a patent ductus arteriosus and how would you cause closure of a PDA?
continuous ‘machinery-like murmur is classic for PDA, you can cause closure with indomethacin or keep it open with prostaglandin E
PDA is often caused by which of the TORCH infections?
maternal rubella infection, PDA will eventually result in pulmonary HTN
Name 3 examples of obstructive congenital anomalies.
coarctation of the aorta
pulmonary stenosis and atresia
aortic stenosis and atresia
Describe how coarctation of the aorta is associated with the following:
a congenital syndrome
another CHD
another serious medical condition
coarctation of the aorta is associated with
Turner Syndrome
50% cases associated with a bicuspid aortic valve, also VSD or ASD
associated with berry aneurysm
What are the two type of coarctation of the aorta?
preductal (infantile) PDA: more severe and more proximal
postductual (adult): more distal, at level of a closed DA aka ligamentum arteriosum
Contrast the clinical features of preductal and post ductal coarctation
preductal presents in infancy as selective cyanosis of the lower extremities and congestive heart failure
postductal presents in older children and adults as notching of the ribs due to collaterals, intermittent claudication and low pressures of the lower extremities
