test 6 surgical treatment

Question 1

Balloon atrial septostomy (BAS)

Answer 1

• has been used with some success to decompress the venous circuit and improve cardiac output in cases of a restrictive inter-atrial communication.
• Since an ASD is imperative to survival – it is better if it is non-restrictive

Question 2

Goal of surgical repair

Answer 2

 Establish an unobstructed communication between the pulmonary venous confluence and the left atrium
 Interrupt connections with systemic venous circulation
 Close the ASD

Question 3

Newborn period for newborns with Total Anomalous Pulmonary Venous Return and obstructed pulmonary veins

Answer 3

• Surgical repair is performed EMERGENTLY
• Some of these children will actually require extracorporeal life support (ECMO) prior to surgery because of their marked hemodynamic instability
• Obstructive TAPVR is one of the true pediatric emergencies

Question 4

Children with TAPVR without obstruction to the pulmonary veins

Answer 4

• typically undergo surgical repair electively days or weeks after the diagnosis is made.
• In these children, although the surgery is not emergent, there is generally little benefit to be gained by waiting more than one or two months.

Question 5

PAPVR repair

Answer 5

• normally corrected without complications

Question 6

TAPVR repair

Answer 6

• still carries significant morbidity and mortality in low volume centers, (due to the severe hemodynamic and metabolic compromise).
  • 10-15% of patients undergoing repair of TAPVR require multiple interventions due to recurrent stenosis after initial successful correction, (with an increasingly poor outcome at each representation).

Question 7

Process of TAPVR

Answer 7

• In Total Anomalous Pulmonary Venous Return, the pulmonary veins frequently return to a common confluence behind the LA.
• The surgical repair takes advantage of this fact. The common PV confluence is connected to the back of the LA, resulting in a normal connection of PV->LA
• All other abnormal routes for pulmonary venous drainage (are tied off).

Question 8

Supracardiac repair

Answer 8

• go on bypass and cool all the way down to 18 degrees to circ arrest for better access
• ligate the vertical vein
• open up where the 4 pulmonary veins come into the LA and the pulmonary vein and sew it together
• cut connection between pulm vein and SVC
• open RA to close ASD

Question 9

Intracardiac Repair

Answer 9

• cool to 28 degrees because they don’t have to lift the heart or pull everything out of the way
• go in through the atrium
• attach baffle from the coronary sinus and re-route the blood to the LA

Question 10

Infracardiac Repair

Answer 10

• cool to 18 degrees to circ arrest
• pull the heart up toward the babies face
• ligate the veins at the diaphragm
• open up the main pulmonary vein
• open up the back side of the LA
• Sew the two together
• as the kids grow, the heart grows and can distort the anastamosis site (results in return obstruction)
• USE SUTURELESS REPAIR

Question 11

CPB Considerations

Answer 11

 Arterial: Aortic
 Venous:
         PAPVR: Bicaval
         TAPVR: Single Atrial
• Hypothermia: Circulatory arrest will be utilized for TAPVR
         mild for PAPVR

Question 12

Changes happening with hypothermia and TAPVR

Answer 12

• In the past, almost all infants with TAPVC were repaired using profound hypothermia and circulatory arrest
• now it can be performed with bicaval cannulation and low flow hypothermic perfusion.

Question 13

CASE Notes

Answer 13

• These children are very sick
• Pre and post ECMO are a BIG POSSIBILITY
• Birth weights tend to be small
• The pulmonary blood flow undergoes a BIG change and can shock the body

Question 14

Prognosis

Answer 14

• surgical mortality or death rate is higher when surgery is performed emergently in critically ill newborns with obstructed pulmonary venous return.
• This is because they are very sick going to surgery.
• Critically ill newborns who do survive the surgery may require a prolonged period of post-operative intensive care.
• Pulmonary hypertension must decrease in order for complete restoration of normal circulation
• Impressive acidosis may develop