L26- CVS Pathology V Flashcards
list the 8 common congenital heart diseases
- tetrology of fallot
- transposition of great arteries (or vessels)
- truncus arteriosus
- VSD
- ASD
- PDA
- coarctation of aorta
- cardiac neoplasm
Turner Syndrome is associated with (1) congenital heart disease
Trisomy 21 (Down syndrome) is associated with (2)
Rubella is the main environmental factor to cause congenital heart disease, associated with (3)
1- coartication of aorta
2- AV canal, ASD, VSD
3- PDA
______ is by far the most common congenital heart disease
VSD (42% of all, ASD is 2nd at 10%)
list the R to L shunts in utero
foramen ovale: RA –> LA
ductus arteriosus: pulmonary trunk –> aorta
describe the transition from fetal to adult circulation
- at birth, baby’s breathing inflate lungs –> dec pulmonary resistance
- pressure change closes flap valve of foramen ovale
- ductus arteriosus (15 hrs of life): PGs (via placenta) keep it open –> at birth PGs are metabolized in lung –> reduction via dec production and inc breakdown => closes DA
what are the categories of congenital heart malformations
- R-to-L shunts (cyanotic at birth or later)
- L-to-R shunts
- obstruction
describe pulmonary vascularity in relation to congenital heart disease
Dx clue:
-inc lung vascularity indicates L –> R shunt (plethoric lung fields)
-dec lung vascularity indicates R –> L shunt (oligemic lung fields)
what are the R-to-L shunt malformations seen at birth
(cyanosis at birth)- 5 T’s
- Tetrology of Fallot
- Transposition of the Great Vessels
- Truncus Arteriosus
- Tricuspid Atresia
- Total Anomalous Pulmonary Venous Connection
what are the R-to-L shunt malformations seen well after birth (mos-yrs)
(cyanosis later in life)- 3 D’s
- VSD
- ASD
- PDA
list the 4 features of Tetrology of Fallot
- VSD
- pulmonary stenosis (infundibular stenosis)
- overriding aorta
- RVH
the prognosis of Tetralogy of Fallot is dependent on…..
degree of pulmonary stenosis
-if severe, cyanosis is evident immediately
Tetrology of Fallot usually presents at (1), its timing is dependent on (2). It is often associated with (3) disorder.
1- w/in 6 mos
2- degree of severity of pulmonary stenosis
3- Down syndrome
- (1) is one of the hallmark symptoms of ToF as it causes (2)
- (3) is evident with increased activity
- (4) is evident on labs and can possibly cause (5)
- ToF is a (6) type shunt, therefore there is an increased risk of (7)
1/2- squatting- inc PVR / pressure in aorta –> pushes blood into pulmonary circulation
3- dyspnea
4/5- polycythemia inc risk of cerebral thrombosis
6/7- R-to-L, inc risk of infective endocarditis and systemic embolization
Transposition of the Great Arteries is defined as (1) and is only compatible with life if (2) is present. The dominant clinical finding is (3). The prognosis is dependent on (4).
1- aorta from RV, pulmonary trunk from LV
2- VSD, PDA
3- cyanosis
4- degree of mixing => tissue hypoxia /// ability of RV to maintain systemic circulation
Truncus Arteriosus is defined as (1), accompanied with (2) necessary for compatibility with life. The main blood flow abnormality is (3), possibly causing (4).
1- failure of partitioning of embryonic truncus (supposed to => aorta + pulm. trunk)
2- VSD (=> one ventricular chamber with mixed blood)
3- over-perfusion to lungs
4- pulmonary HTN