Cyanotic CHD

Question 1

Interrupted aortic arch

Answer 1

this is the most severe form of coarc

O2 blood goes through Ao to upper body

Need ASD and PDA: mixed blood goes to lungs and lower body

Question 2

TAPVR

Answer 2

TAPVR = Total Anomalous Pulmonary Venous Return

the PVs do not connect to LA;
they drain directly or indirectly into RA

Need ASD or VSD or PDA - mixed blood to lungs and body

Question 3

Prostaglandin

Answer 3

drug given at birth to keep PDA open

Question 4

Tetralogy of Fallot

Answer 4

Comprised of things:

1. Overriding Ao (overrides VSD and shifted to R)*
2. VSD
3. RVOT obs and/or PS - determines severity
4. RVH - bc of #3.

*still more than 50% Ao from LV (otherwise DORV)

Question 5

What is the most common cyanotic CHD?

Answer 5

Tetralogy of Fallot

Question 6

What is the most hemodynamically significant factor of Tetralogy of Fallot?

Answer 6

The RVOT obs/PS

the bigger the RVOT obs, the more deO2 blood goes to body = more severe disease

Question 7

DORV

Answer 7

DORV = Double Outlet RV

both great vessels arise from RV +
VSD is only outlet from LV
(may have PS - if yes, than same as ToF but Ao more on R side)

4 types of DORV based on location of great arteries relative to VSD:
subaortic
subpulmonary
doubly committed
noncommitted

Question 8

DORV: subaortic

Answer 8

AoV is closest to VSD

good - mostly O2 blood to body

Question 9

DORV: subpulmonary

Answer 9

PV is closest to VSD

bad - mostly deO2 blood to body

Question 10

DORV: doubly committed

Answer 10

both AoV and PV close to VSD

mixed O2 to body and lungs

Question 11

DORV: non-committed

Answer 11

both AoV and PV far away from VSD

mixed O2 to body and lungs

Question 12

Hemodynamics of DORV?

Answer 12

deO2 blood through Ao to body

excess blood to pulm sys - Right HF

Question 13

D-TGA

Answer 13

Complete TGA (D = Die)

the great vessels have switched spots (bc truncus arteriosus did not spiral)

single discordance (ventriculoarterial)
R and L sides in parallel

commonly associated with VSD :)

at birth: atrial septostomy and prostaglandin

Question 14

What would a SAX at AoV look like in a patient with D-TGA?

Answer 14

PV in the “middle”
AoV anterior and to the right of the PV

To ID AoV vs PV, sweep and look for bifurcation (PA) or no split (Ao)

Question 15

Truncus Arteriosus

Answer 15

Common trunk with 1 semilunar valve (truncal valve) that overrides an outlet VSD

common trunk branches: coronaries, MPA, Ao

caused by failure of truncus arteriosus to divide

Question 16

hemodynamic consequences of truncus arteriosus?

Answer 16

Mixed blood to body and coronaries = reduced EF

too much blood to lungs = PHTN and cardiomegally

Question 17

HRHS

Answer 17

HRHS = Hypoplastic Right Heart Syndrome

Tricuspid Atresia
Pulmonary Atresia: PA-IVS, PA-VSD

Question 18

HRHS: Tricuspid Atresia

Answer 18

No TV and hypoplastic RV

Required for survival:
ASD or PFO
VSD and/or PDA

LV pumps mixed blood to both body and lungs (if VSD, excess blood to lungs…)

Question 19

HRHS: PA-IVS

Answer 19

PA-IVS = Pulmary Atresia Intact Ventricular Septum

Involves:
1. Complete RVOT obs (atretic PV)
2. Intact IVS
3. varying degrees TV and RV hypoplasia

Required for survival:
ASD
PDA

Question 20

HRHS: PA-VSD

Answer 20

PA-VSD = Pulmonary Atresia Ventricular Septal Defect

Involves:
1. Atresia of PV and underdevelopment of RVOT (this determines severity)
2. Large VSD
3. Overriding Ao

(50% of patients have ASD/PFO)

*Considered the most severe form of ToF

Question 21

Which condition is considered the most severe form of ToF?

Answer 21

PA-VSD

Question 22

HLHS

Answer 22

HLHS = Hypoplastic Left Heart Syndrome

under development of L heart - Ao complex

Includes any of: MS / Mitral Atresia, AS / Aortic Atresia, Hypoplasia of LV, hypoplasia of Ao arch

Required for survival:
PFO/ASD
PDA