Complex Congenital Heart Defects

Question 1

What is coarctation of the Ao? (CoAo)

Answer 1

Narrowing of the Ao arch or Desc Ao

Question 2

Where is the most common location of CoAo?

Answer 2

Descending Ao

Question 3

What type of congenital valvular disease do 50% of people with CoAo have?

Answer 3

Bicuspid AV

Question 4

With a CoAo, we should assess the abdominal Ao. What kind of flow would we see in the abdo Ao?

Answer 4

Decreased velocity, monophasic with continuous antegrade flow through systole AND diastole

Question 5

What are the 3 types of CoAo?

Answer 5

Postductal (inferior to DA)
Preductal (superior to DA)
Juxtaductal (at the level of the DA)

Question 6

What is a truncus arteriosus?

Answer 6

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

Question 7

What are the 4 types of Truncus Arteriosus variations?

Answer 7

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

Question 8

What is transposition of the great arteries? (TGA)

Answer 8

Ao comes from RV
PA comes from LV

Question 9

What are the two types of TGA?

Answer 9

CCTGA (congenitally corrected)
DTGA (complete)

Question 10

What is occurring with DTGA?

Answer 10

Ao arises from RV, PA arises from LV

Question 11

What occurs with DTGA with NO additional defect?

Answer 11

No defect leads to cyanosis and death, no oxygenated blood is getting to the body

Question 12

What surgical procedure is required for survival with a DTGA?

Answer 12

Mustard procedure

Question 13

What is occurring with CCTGA?

Answer 13

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

Question 14

What is Tetralogy of Fallot? (TOF)

Answer 14

4 coexisting defects:

1. VSD
2. Overriding Ao
3. Pulmonary stenosis
4. RVH

Question 15

What is the most common cyanotic abnormality?

Answer 15

TOF

Question 16

What problems are created with TOF? (caused by PS, VSD)

Answer 16

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

Question 17

What can happen to the right heart after TOF repair?

Answer 17

Chronic PI resulting in RV enlargement and dysfunction after TOF repair

Question 18

What is a double outlet RV? (DORV)

Answer 18

Ao and PA come from RV (also considered overriding Ao when >50% shift into RV with VSD present)

Question 19

Is surgical intervention required for survival with a DORV?

Answer 19

Yes

Question 20

What is anomalous venous return?

Answer 20

When the PV’s (one or more) dump into anywhere that isn’t the LA

Question 21

What are the 2 types of anomalous venous return?

Answer 21

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

Question 22

What occurs with TAPVR?

Answer 22

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

Question 23

With TAPVR, what happens with the PFO shunt after birth?

Answer 23

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

Question 24

What is associated with PAPVR? (80% association)

Answer 24

Sinus venosus ASD

Question 25

What are the abnormal location connections of the PV’s?

Answer 25

RA
IVC/SVC
Coronary Sinus

Question 26

Which procedures is done to correct TOF, pulmonary atresia? (HINT: 2 procedures)

Answer 26

1. Blalock-Taussig shunt

Subclavian A or Innominate A connected to L/RPA’s to increase pulmonary flow

2. Rastelli Procedure

Connects RV to PA

Question 27

What procedure is done to correct tricuspid atresia, single ventricle?

Answer 27

Fontan Procedure

Connection b/w systemic venous return to PA (usually IVC to PA), to increase PA flow

Question 28

What procedure replaces a diseased AV?

Answer 28

Ross procedure (pediatrics)

Diseased AV is replaced with Pt’s own PV (autograft), then PV is replaced with cadaver valve (homograft)