SV, HLHS, HRHS- Topic 16 and 17

Question 1

SV Physiology

Answer 1

Single function pumping chamber

Valves/Outflow tracts may be disrupted

Question 2

What’s the goal of SV physiology?

Answer 2

Must control/balance PA and Aortic flow

Question 3

HRHS aka….

Answer 3

PA with IVS (may have tricuspid atresia)

Question 4

Types of Single Ventricle Syndromes

Answer 4

HLHS

HRHS

Question 5

HLHS was first successfully treated in the mid 1980’2 by who? Where? Under who?

Answer 5

Dr. William Norwood; Philadelphia Children’s Hospital; under Dr. Aldo Castaneda

Question 6

HLHS has been about _____% fatal.

Answer 6

100%

Question 7

1983: ____________ reports the 1st successful case.

Answer 7

Dr. William Norwood

Question 8

1984: ______, the little girl who received the babboon heart transplant in 1984, is probably the most prominent case of HLHS.

Answer 8

Baby Fae

Question 9

1986: _________- bought time to improve results.

Answer 9

Prostaglandins (PGE1)

Question 10

HLHS

Answer 10

severe congenital heart defect in which the left side of the heart does not develop

Question 11

HLHS is characterized by….

Answer 11

Atretic, hypoplastic aorta and arch
Large PDA
Hypoplastic LV
Small MV and/or AV
Hopefully, an ASD allowing blood returning from lungs to reach the single ventricle

Question 12

What is the only blood flow to the body in HLHS?

Answer 12

PDA

Question 13

In HLHS, the ASD can be ________ or __________.

Answer 13

Restrictive or non-restrictive

Question 14

HRHS

Answer 14

refers to underdevelopment of the right sided structures of the heart; these defects cause inadequate blood flow to the lungs and thus, a cyanotic infant

Question 15

WHat is the major problem in HRHS?

Answer 15

Pulmonary Valve atresia

Question 16

Secondary problems of HRHS

Answer 16

A small TV

A hypoplastic pulmonary artery

Question 17

Is HRHS or HLHS more rare?

Answer 17

HRHS is more rare than HLHS

Question 18

What ist he survival rate for HRHS?

Answer 18

Survival rate predicted to be 15-30 years post - Fontan

Question 19

Surgical Procedures for Univentricular Repair: Palliation

Answer 19

Staged Procedures

Hybrid Procedures

Question 20

Immediate Palliation for HLHS/HRHS

Answer 20

Balloon Atrial Septostomy (Rashkind Procedure)
Blade Septectomy (Hanlon) Procedure - not used much

Question 21

Staged Procedures for SV Heart

Answer 21

1. Norwood
2. Bi-directional Glenn/Hemi-Fontan
3. Competion Fontan (18 mo-2 y/o)

Question 22

Rarely, the staged approach cannot be performed on an HLHS heart, _________ is performed.

Answer 22

Heart Transplant is performed

Question 23

Norwood Procedure: CPB Details

Answer 23

DHCA Procedure: on arrest, surgeon does the following:
Close PDA
Enlarge aorta ( create neo-aorta )
Add Systemic- PA Shunt during warming

Question 24

What type of Systemic-PA Shunt during warming

Answer 24

Modified B-T (3.5 mm shunt size-average)

Sano (5.0 mm shunt size-average)

Question 25

Sano Modification Procedure

Answer 25

placement of a conduit between the RV and the PA instead of hte MOdified BT shunt

Question 26

Sano Modification Shunt Construction

Answer 26

Slightly larger Gortex tube graft than that used for the modified BT shunt. Generally a 5mm tube graft is selected in contast to the 3.5 mm graft

Question 27

What has a more rocky course in the OR: MBTS or Sano.

Answer 27

MBTS

Question 28

What has a more rocky course in the PICU: MBTS or Sano

Answer 28

Sano

Question 29

What is a smaller, lower pressure shunt? MBTS or Sano?

Answer 29

MBTS

Question 30

What is a large shunt with higher pressures? MBTS or SANO?

Answer 30

Sano

Question 31

Which shunt is more centrally located: MBTS or Sano?

Answer 31

Sano

Question 32

Today, about ____ percent of babies presenting with HLHS can be expected to survive their Norwood operation; truly a success given that 20+ years ago the outlooks was hopeless.

Answer 32

90%

Question 33

New Phsyiology: Pulmonary Blow Flow

Answer 33

MBS

Question 34

New Physiology: Systemic Blood Flow

Answer 34

Neo-aorta