SV, HLHS, HRHS- Topic 16 and 17 Flashcards
SV Physiology
Single function pumping chamber
Valves/Outflow tracts may be disrupted
What’s the goal of SV physiology?
Must control/balance PA and Aortic flow
HRHS aka….
PA with IVS (may have tricuspid atresia)
Types of Single Ventricle Syndromes
HLHS
HRHS
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
HLHS has been about _____% fatal.
100%
1983: ____________ reports the 1st successful case.
Dr. William Norwood
1984: ______, the little girl who received the babboon heart transplant in 1984, is probably the most prominent case of HLHS.
Baby Fae
1986: _________- bought time to improve results.
Prostaglandins (PGE1)
HLHS
severe congenital heart defect in which the left side of the heart does not develop
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
What is the only blood flow to the body in HLHS?
PDA
In HLHS, the ASD can be ________ or __________.
Restrictive or non-restrictive
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
WHat is the major problem in HRHS?
Pulmonary Valve atresia
Secondary problems of HRHS
A small TV
A hypoplastic pulmonary artery
Is HRHS or HLHS more rare?
HRHS is more rare than HLHS
What ist he survival rate for HRHS?
Survival rate predicted to be 15-30 years post - Fontan
Surgical Procedures for Univentricular Repair: Palliation
Staged Procedures
Hybrid Procedures
Immediate Palliation for HLHS/HRHS
Balloon Atrial Septostomy (Rashkind Procedure) Blade Septectomy (Hanlon) Procedure - not used much
Staged Procedures for SV Heart
- Norwood
- Bi-directional Glenn/Hemi-Fontan
- Competion Fontan (18 mo-2 y/o)
Rarely, the staged approach cannot be performed on an HLHS heart, _________ is performed.
Heart Transplant is performed
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
What type of Systemic-PA Shunt during warming
Modified B-T (3.5 mm shunt size-average)
Sano (5.0 mm shunt size-average)
Sano Modification Procedure
placement of a conduit between the RV and the PA instead of hte MOdified BT shunt
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
What has a more rocky course in the OR: MBTS or Sano.
MBTS
What has a more rocky course in the PICU: MBTS or Sano
Sano
What is a smaller, lower pressure shunt? MBTS or Sano?
MBTS
What is a large shunt with higher pressures? MBTS or SANO?
Sano
Which shunt is more centrally located: MBTS or Sano?
Sano
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%
New Phsyiology: Pulmonary Blow Flow
MBS
New Physiology: Systemic Blood Flow
Neo-aorta
