Congenital heart disease 3

Question 1

The patient scheduled for a Fontan procedure MOST likely has a diagnosis of:
a. truncus arteriosus
b. Ebstein’s anomaly
c. transposition of the great arteries
d. hypoplastic left heart syndrome

Answer 1

d. hypoplastic left heart syndrome

Question 2

Ebstein’s anomaly is characterized by

Answer 2

a downward displacement of the tricuspid valve
right atrial dilation
“atrialization” of the right ventricle
usually an ASD or PFO

Question 3

In transposition of the great arteries, each great vessel arises from

Answer 3

the wrong ventricle

Question 4

Transposition of the great arteries produces a circulation in

Answer 4

parallel rather than in series

Question 5

_____________ is a medical emergency.

Answer 5

Transposition of the great arteries

Question 6

With transposition of the great arteries, the RV gives rise to

Answer 6

the aorta (poorly-oxygenated circuit)

Question 7

With transposition of the great arteries, the LV gives rise to

Answer 7

the pulmonary artery (well-oxygenated circuit)

Question 8

Hypoplastic left heart syndrome is a

Answer 8

single-ventricle lesion

Question 9

Hypoplastic left heart syndrome is corrected with

Answer 9

staged surgical procedures culminating with the Fontan operation

Question 10

With hypoplastic left heart syndrome, __________ should be avoided

Answer 10

anything that increases PVR

Question 11

With hypoplastic left heart syndrome, the patient has

Answer 11

a single left ventricle that pumps blood into the systemic circulation

Question 12

Truncus arteriosus is characterized by

Answer 12

a single artery that gives rise to the pulmonary, systemic, and coronary circulations

Question 13

Truncus arteriosus typically also comes with

Answer 13

a VSD

Question 14

With truncus arteriosus, there is only one artery which means

Answer 14

there is no specific pathway for blood to enter the pulmonary circulation before being pumped into the systemic circulation

Question 15

The most common congenital defect of the tricuspid valve is

Answer 15

Ebstein’s anomaly

Question 16

With Ebstein’s anomaly, ______________- can be severe

Answer 16

tricuspid regurgitation

Question 17

Describe the onset of IV drugs with Ebstein’s anomaly.

Answer 17

may be prolonged due to the pooling of drugs in the enlarged RA

Question 18

__________ is common in the postoperative period with Ebstein’s anomaly

Answer 18

RV failure

Question 19

Maintenance of _______________ is critical with Ebstein’s anomaly

Answer 19

RV function (risk of CHF)

Question 20

Transposition of the great arteries relies on ______________ to be compatable with life

Answer 20

ASD, VSD, or PFO until surgery performed

Question 21

Treatments for transposition of the great arteries includes:

Answer 21

keep PDA open with prostaglandin infusion
Rashkind procedure- interarterial pathway to allow some oxygenated blood to reach systemic circ.
Intraatrial baffle and arterial switch procedures- definitive surgical correction

Question 22

Anatomic features of hypoplastic left heart syndrome include

Answer 22

hypoplastic LV
hypoplastic aortic arch
mitral and aortic stenosis or atresia
ductal-dependent circulation

Question 23

The goal of surgical correction with hypoplastic left heart syndrome is

Answer 23

separating the pulmonary and systemic circulations

Question 24

What are the three surgeries needed to correct a hypoplastic left heart?

Answer 24

Norwood stage 1
Norwood stage 2
Norwood stage 3 (Fontan procedure)

Question 25

When does Norwood stage 1 occur?

Answer 25

neonatal period

Question 26

Surgical goals of Norwood stage 1 include

Answer 26

aortic reconstruction- aortic arch now arises from the pulmonary trunk
pulmonary arteries disconnected and use to create shunt from subclavian artery or right ventricle

Question 27

When does Norwood stage 2 occur?

Answer 27

3-6 months of age

Question 28

Surgical goals of Norwood stage 2 include

Answer 28

the shunt from the first procedure is taken down and new connection is made between the SVC and pulmonary arteries

Question 29

When does Norwood stage 3 occur?

Answer 29

2-4 years

Question 30

What is the surgical goal of Norwood stage 3?

Answer 30

conversion to Fontan circulation- the IVC is connected to the pulmonary artery with a conduit

Question 31

Important anesthetic considerations after the Fontan procedure:

Answer 31

patient has a single ventricle the pumps blood into the systemic circulation
pulmonary blood flow is passive from SVC/IVC
increased PVR is detrimental to pulmonary blood flow
PPV reduces blood flow and spontaneous ventilation is preferred
patients are preload dependent!