SV, HLHS, HRHS- Topic 16 and 17 Flashcards Preview

Congenital Defects & Pediatrics > SV, HLHS, HRHS- Topic 16 and 17 > Flashcards

Flashcards in SV, HLHS, HRHS- Topic 16 and 17 Deck (34):
1

SV Physiology

Single function pumping chamber
Valves/Outflow tracts may be disrupted

2

What's the goal of SV physiology?

Must control/balance PA and Aortic flow

3

HRHS aka....

PA with IVS (may have tricuspid atresia)

4

Types of Single Ventricle Syndromes

HLHS
HRHS

5

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

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

6

HLHS has been about _____% fatal.

100%

7

1983: ____________ reports the 1st successful case.

Dr. William Norwood

8

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

Baby Fae

9

1986: _________- bought time to improve results.

Prostaglandins (PGE1)

10

HLHS

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

11

HLHS is characterized by....

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

12

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

PDA

13

In HLHS, the ASD can be ________ or __________.

Restrictive or non-restrictive

14

HRHS

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

15

WHat is the major problem in HRHS?

Pulmonary Valve atresia

16

Secondary problems of HRHS

A small TV
A hypoplastic pulmonary artery

17

Is HRHS or HLHS more rare?

HRHS is more rare than HLHS

18

What ist he survival rate for HRHS?

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

19

Surgical Procedures for Univentricular Repair: Palliation

Staged Procedures
Hybrid Procedures

20

Immediate Palliation for HLHS/HRHS

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

21

Staged Procedures for SV Heart

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

22

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

Heart Transplant is performed

23

Norwood Procedure: CPB Details

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

24

What type of Systemic-PA Shunt during warming

Modified B-T (3.5 mm shunt size-average)
Sano (5.0 mm shunt size-average)

25

Sano Modification Procedure

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

26

Sano Modification Shunt Construction

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

27

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

MBTS

28

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

Sano

29

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

MBTS

30

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

Sano

31

Which shunt is more centrally located: MBTS or Sano?

Sano

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.

90%

33

New Phsyiology: Pulmonary Blow Flow

MBS

34

New Physiology: Systemic Blood Flow

Neo-aorta