Complex Congenital Heart Defects Flashcards
What is coarctation of the Ao? (CoAo)
Narrowing of the Ao arch or Desc Ao
Where is the most common location of CoAo?
Descending Ao
What type of congenital valvular disease do 50% of people with CoAo have?
Bicuspid AV
With a CoAo, we should assess the abdominal Ao. What kind of flow would we see in the abdo Ao?
Decreased velocity, monophasic with continuous antegrade flow through systole AND diastole
What are the 3 types of CoAo?
Postductal (inferior to DA)
Preductal (superior to DA)
Juxtaductal (at the level of the DA)
What is a truncus arteriosus?
Single, large overriding outflow track with an associated VSD
PA and AO come from a single trunk with one valve controlling flow out of the heart
What are the 4 types of Truncus Arteriosus variations?
Type I - MPA originates from TA
Type II/III - L + R PA’s originate from TA
Type IV - No PA’s, bronchial arteries originate directly from DescAo
What is transposition of the great arteries? (TGA)
Ao comes from RV
PA comes from LV
What are the two types of TGA?
CCTGA (congenitally corrected)
DTGA (complete)
What is occurring with DTGA?
Ao arises from RV, PA arises from LV
What occurs with DTGA with NO additional defect?
No defect leads to cyanosis and death, no oxygenated blood is getting to the body
What surgical procedure is required for survival with a DTGA?
Mustard procedure
What is occurring with CCTGA?
Ventricles and great arteries reverse so that the flow pattern remains correct
Pulmonary veins flow into LA then flows into RV and into the Ao
IVC flows into RA then into the LV and into the PA
What is Tetralogy of Fallot? (TOF)
4 coexisting defects:
- VSD
- Overriding Ao
- Pulmonary stenosis
- RVH
What is the most common cyanotic abnormality?
TOF
What problems are created with TOF? (caused by PS, VSD)
PS increases Rt sided pressures, creating a R to L shunt
Oxygenated and deoxygenated blood mix in the LA, sending less-oxygenated blood into the Ao = cyanosis
What can happen to the right heart after TOF repair?
Chronic PI resulting in RV enlargement and dysfunction after TOF repair
What is a double outlet RV? (DORV)
Ao and PA come from RV (also considered overriding Ao when >50% shift into RV with VSD present)
Is surgical intervention required for survival with a DORV?
Yes
What is anomalous venous return?
When the PV’s (one or more) dump into anywhere that isn’t the LA
What are the 2 types of anomalous venous return?
Total (TAPVR) - all of the PV’s dump into somewhere other than the LA
Partial (PAPVR) - some of the PV’s not dumping into the LA
What occurs with TAPVR?
All PV’s do not dump into LA and cause severe cyanosis and high pulmonary pressures, which causes a R to L shunt through the PFO
With TAPVR, what happens with the PFO shunt after birth?
The lungs expand after birth, causing the right sided pressures to drop. The shunt reverses to L to R which causes remaining LA (oxygenated) blood to be shunted to the right side, so no oxygenated blood is sent out of the left side
What is associated with PAPVR? (80% association)
Sinus venosus ASD
What are the abnormal location connections of the PV’s?
RA
IVC/SVC
Coronary Sinus
Which procedures is done to correct TOF, pulmonary atresia? (HINT: 2 procedures)
- Blalock-Taussig shunt
Subclavian A or Innominate A connected to L/RPA’s to increase pulmonary flow
- Rastelli Procedure
Connects RV to PA
What procedure is done to correct tricuspid atresia, single ventricle?
Fontan Procedure
Connection b/w systemic venous return to PA (usually IVC to PA), to increase PA flow
What procedure replaces a diseased AV?
Ross procedure (pediatrics)
Diseased AV is replaced with Pt’s own PV (autograft), then PV is replaced with cadaver valve (homograft)
