Congenital Heart Disease Flashcards
____ is the greatest risk factor for having a congenital heart disease
defect in sibling or parent is the greatest risk factor for having a congenital heart disease
____ is associated with coarctation of the aorta
Turner syndrome is associated with coarctation of the aorta
describe the fetal to adult circulation transition
- at birth, the baby breathes, the lungs inflate and pulmonary resistance (right sided pressure) falls
- blood flow stops through foramen ovale (flap valve)
- ductus arteriosus closes by 15 hours of life
- prostaglandins (PGs) maintain patency (keep open)
- PGs produced in placenta, metabolized in lungs
- at birth, reduced production/increased breakdown
there is an increase in _____ with left to right shunting of blood (plethoric lung fields) and a decrease in ____ with a right to left shunting of blood (oligemic lung fields)
there is an increase in lung vascularity with left to right shunting of blood (plethoric lung fields) and a decrease in vascularity with a right to left shunting of blood (oligemic lung fields)
____ is the most common form of cyanotic congenital heart disease
Tetralogy of Fallot is the most common form of cyanotic congenital heart disease
name the 4 features of ToF
PROVe
- pulmonary stenosis (infundibular stenosis): the degree determines the prognosis
- right ventricular hypertrophy
- overriding aorta
- VSD
___ is associated with Tetralogy of Fallot
Down’s syndrome is associated with Tetralogy of Fallot
list the clinical features of ToF
- usually present by 6 months
- dyspnea on exertion, cyanosis, squatting
- polycythemia → cerebral thrombosis
- bc of persistent hypoxia → increased EPO → increased RBCs
- IE
- pulmonary vasculature decreased
- associated with Down’s Syndrome
describe transposition of the great arteries
- aorta off RV and pulmonary artery off LV
- incompatible with post-natal life, unless a shunt (VSD or PDA) is present
- the dominant clinical finding is cyanosis
- the outcome depends on the degree of blood “mixing,” tissue hypoxia and the ability of the RV to maintain the systemic circulation
describe what is seen in the image
transposition of the great vessels
RV is much thicker than LV because the RV needs to pump the blood to the systemic circulation, whereas LV pumps to pulmonary circulation (lower pressure)
describe truncus arteriosus
- failure of partitioning of embryologic truncus into aorta and pulmonary artery
- single great artery gets blood from both ventricles
- underlying VSD
- blood from both ventricles mixes
- cyanotic
describe tricuspid atresia
- complete occlusion of the tricuspid orifice
- underdeveloped RV (hypoplastic)
- mitral valve larger than normal
- R to L shunt through ASD (bypass obstruction)
- L to R shunt through VSD (gets blood in RV)
- cyanotic from birth with high mortality
describe total anomalous pulmonary venous connection (TAPVC)
- pulmonary veins fail to join the left atrium
- pulmonary return vessels drain into the left innominate vein or coronary sinus
- ASD always present, allowing oxygenated pulmonary blood to return into the LA
- R to L shunting through ASD due to lower LA pressure (no normal return) results in cyanosis
____ is the most common congenital heart lesion and is associated with ____
VSD is the most common congenital heart lesion and is associated with trisomy 21, 13, 18
the membranous portion is most commonly affected
name clinical features and complications of VSD
- clinical features:
- pulmonary HTN (bc RV receiving blood from RA + LV)
- CHF
- pansystolic murmur
- complication:
- shunt reversal leads to cyanosis = Eisenmenger complex
describe ASD
- age at presentation variable, may be asymptomatic into adulthood
- most common congenital malformation diagnosed in adults
- pulmonary vascularity increased if significant left to right shunt
describe the 3 types of ASD
describe what is seen in the image
describe patent ductus arteriosus (PDA)
- females > males
- continuous, “machine-like” murmur
- may need to keep open with prostaglandin E (kEEp it open) if associated with other malformations
- close with indomethacin (NSAID = prostaglandin inhibitor)
- shunt may reverse leading to cyanosis
describe the pathology of PDA
- connect left pulmonary artery to aortic arch
- closes with high O2 tension
- higher incidence in maternal rubella infxn
- associated with polycythemia
describe coarctation of the aorta and name the 2 types
- narrowed aortic lumen
- associated with Turner’s syndrome
- 2 types:
- preductal (infantile): has PDA
- postductal (adult): ductus arteriosus closed
describe what is seen in the image
coarctation of the aorta
describe clinical features of preductal coarctation of the aorta
- preductal (presents in infancy)
- CHF
- selective cyanosis of lower extremities (PDA)
- femoral pulses are weaker than those of the upper extremities
describe clinical features of postductal coarctation of the aorta
- postductal: presents in older children and adults
- no selective cyanosis is seen
- HTN of the upper extremities
- BP is low and pulses are weak in lower extremities
- notching of ribs due to collaterals
- intermittent claudication; arterial insufficiency
