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

SV, HLHS, HRHS- Topic 16 and 17 Flashcards

1
Q

SV Physiology

A

Single function pumping chamber

Valves/Outflow tracts may be disrupted

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2
Q

What’s the goal of SV physiology?

A

Must control/balance PA and Aortic flow

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3
Q

HRHS aka….

A

PA with IVS (may have tricuspid atresia)

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4
Q

Types of Single Ventricle Syndromes

A

HLHS

HRHS

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5
Q

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

A

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

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6
Q

HLHS has been about _____% fatal.

A

100%

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7
Q

1983: ____________ reports the 1st successful case.

A

Dr. William Norwood

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8
Q

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

A

Baby Fae

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9
Q

1986: _________- bought time to improve results.

A

Prostaglandins (PGE1)

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10
Q

HLHS

A

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

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11
Q

HLHS is characterized by….

A 
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
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12
Q

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

A

PDA

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13
Q

In HLHS, the ASD can be ________ or __________.

A

Restrictive or non-restrictive

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14
Q

HRHS

A

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

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15
Q

WHat is the major problem in HRHS?

A

Pulmonary Valve atresia

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16
Q

Secondary problems of HRHS

A

A small TV

A hypoplastic pulmonary artery

17
Q

Is HRHS or HLHS more rare?

A

HRHS is more rare than HLHS

18
Q

What ist he survival rate for HRHS?

A

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

19
Q

Surgical Procedures for Univentricular Repair: Palliation

A

Staged Procedures

Hybrid Procedures

20
Q

Immediate Palliation for HLHS/HRHS

A 
Balloon Atrial Septostomy (Rashkind Procedure)
Blade Septectomy (Hanlon) Procedure - not used much
21
Q

Staged Procedures for SV Heart

A
  1. Norwood
  2. Bi-directional Glenn/Hemi-Fontan
  3. Competion Fontan (18 mo-2 y/o)
22
Q

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

A

Heart Transplant is performed

23
Q

Norwood Procedure: CPB Details

A

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

24
Q

What type of Systemic-PA Shunt during warming

A

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

Sano (5.0 mm shunt size-average)

25
Q

Sano Modification Procedure

A

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

26
Q

Sano Modification Shunt Construction

A

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
Q

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

A

MBTS

28
Q

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

A

Sano

29
Q

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

A

MBTS

30
Q

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

A

Sano

31
Q

Which shunt is more centrally located: MBTS or Sano?

A

Sano

32
Q

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.

A

90%

33
Q

New Phsyiology: Pulmonary Blow Flow

A

MBS

34
Q

New Physiology: Systemic Blood Flow

A

Neo-aorta

Congenital Defects & Pediatrics COPY (19 decks)

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