Transposition of the Great

Arteries

Chad Paisley, Josh Schultz, Ellie Halverson

 What is it?
● Congenital cyanotic heart defect
● Involves an abnormal arrangement
of the main arteries leading away
from the heart
● Aorta is connected to right
ventricle, while the pulmonary
artery is connected to the left
ventricle (opposite of normal
anatomy)

Elumalai, G., & Princess, J. (2016)

 What is it?
● In ‘normal’ hearts, blood returns from the body to the right side of the heart and then
goes to the lungs to get oxygenated. Blood then returns to the left side of the heart to
be pumped out to the body.
● In a person with transposition of the great vessels, the blood from the body returns to
the heart and is pumped back into the body without picking up oxygen from the lungs,
while the blood from the lungs is pumped from the heart back to the lungs without
distributing to the body

● The condition is incompatible with life unless a ventricular septal defect, atrial septal
defect or patent ductus arteriosus is present to allow for adequate mixing of blood
(Goldman & Schafer, 2016)

Elumalai, G., & Princess, J. (2016)

 What is it?
● Symptoms appear very shortly after birth
● Severity of symptoms depend on both the size and type of additional heart
defects (such as a patent ductus arteriosus, which allows blood to mix
between the two abnormal circulation patterns)
● Incidence: 20-30 per 100,000 live births
● More common in males
● Causes:
○ Viral infection/rubella
○ Alcohol consumption
○ Diabetes
○ Maternal age greater than 40
● Survival rate is greater than 90% (if fixed, if not fixed, life expectancy is only
months) Elumalai, G., & Princess, J. (2016)
 Tests to confirm
● Prior to Birth:
○ Often can be diagnosed with a fetal echocardiogram
● Post Birth:
○ On exam: mouth and skin have blue hint, can hear murmur with
stethoscope
○ Other tests
■ Pulse oximetry
■ Chest x-ray
■ Echocardiogram
■ Cardiac catheterization
■ ECG

Transposition of the great vessels

(2015)
 Treatment
● Neonate will receive IV prostaglandins to help keep ductus arteriosus open
(which allows mixing of oxygenated and deoxygenated blood)
● Surgical intervention: arterial switch procedure
○ Septostomy may be done to promote inter-circulatory mixing
○ Pulmonary artery is moved from the left ventricle to the right ventricle
○ Aorta is moved from right ventricle to the left ventricle
○ Coronary arteries are moved so they originate from aorta (allowing the heart muscle to receive
oxygen-rich blood)
○ Any other cardiac abnormalities (such as a patent ductus arteriosus, ventricular or atrial septal
defects) are closed

(Transposition of the Great Arteries (TGA), 2018)

Transposition of the
great arteries. (n.d.).
 Preoperative Assessment
● The preoperative evaluation needs to encompass the neonate's physical
condition, including significant illness, the degree of transition from fetal to
newborn physiology, maturity, and the presence of congenital anomalies
● Gestational age, birth weight, and postnatal age are important in determining
anesthetic care

● Ascertain time of last oral feeding

○ NPO Guidelines
■ Clear liquids - 2 hours
■ Breast Milk - 4 hours
■ Formula - 6 hours

(Fanaroff, Martin, & Walsh, 2015)

 Preoperative Assessment
● A complete clinical physical examination must be performed prior to OR,
including vital signs.
● The airway needs to be carefully assessed
○ Difficult intubation is a common problem in neonates with congenital defects
○ Neonates have a tendency for rapid oxygen desaturation, so difficulty with intubation can be
extremely serious

● Volume status needs to be carefully assessed due to the potential for major
shifts during surgery

(Fanaroff, Martin, & Walsh, 2015)

 Induction and Maintenance
● Prostaglandin E infusion should be continued until on cardiopulmonary
bypass
● Increased sensitivity to anesthetic induced myocardial depression

● Induction drugs should promote hemodynamic stability without adversely

affecting inter-circulatory mixing
○ Ex: Opioids such as fentanyl or sufentanil
● In addition to muscle relaxation, divided doses of benzodiazepines or
low-concentration inhaled agents can be added to promote amnesia (Cladis &
Davis, 2017)

(Holzman, Mancuso, & Polaner, 2016)

 Intraoperative Anesthetic Considerations
● Blood pressure and heart rate must be monitored closely

● Blood pressure should be kept at age-appropriate norms

● Systemic ventricular dysfunction may necessitate inotropic and vasodilator therapy to terminate
cardiopulmonary bypass

○ Dobutamine or dopamine is most often used

○ Milrinone may be used if SVR is high

■ Milrinone not only reduces LV afterload but may also contribute inotropic, lusitropic, and
PVR-reducing effects

● To maintain an age-appropriate heart rate, atrial pacing may need to be used in the post
cardiopulmonary bypass period

○ Cardiac output will be more heart rate dependent in the neonate

(Cladis & Davis, 2017)
 Postoperative Anesthetic Considerations
● CBC may be performed
● Possibility of extensive bleeding from the aortic and pulmonary suture lines
which may require the transfusion of blood product

● Occasionally, reducing pulmonary artery and aortic pressures may be used to

reduce the amount of bleeding

(Holzman, Mancuso, & Polaner, 2016)

 Postoperative Anesthetic Considerations
● 12 Lead ECG
● Evidence of myocardial ischemia after coronary reimplantation should be
treated aggressively and should prompt immediate reevaluation of the
anastomoses, as well as the possibility of coronary kinking or external
coronary compression by clot or hemostatic packing material

● Evidence of myocardial ischemia is typically in the form of focal ECG

abnormalities such as S-T/T wave changes, but it can also include various
dysrhythmias and forms of AV block

(Cladis & Davis, 2017)

 Postoperative Anesthetic Considerations
● Continue to monitor blood pressure and heart rate closely, and assess for
signs of left ventricular failure
● The left ventricle may have limited ability to support the systemic circulation
after the ASO as a result of myocardial ischemia, inadequate LV mass, poor
protection of the LV during aortic cross-clamping

● Echocardiography can be useful in identifying dysfunction

(Cladis & Davis, 2017)

 Questions
1. What is “transposition of the great vessels”?
a. Aorta is connected to right ventricle, while the pulmonary artery is connected to the
left ventricle
b. Elevated aortic and pulmonic blood pressures
c. The umbilical cord has two veins and one artery
d. Pulmonary hypertension in the neonate has forced blood to flow backwards through a
patent ductus arteriosus

2. What is the first sign a practitioner may notice if a neonate has transposition of the great
vessels?

a. Apneic periods
b. Hazy chest x-ray
c. Bluish tint around neonate’s mouth
d. Low motor tone
 Questions
3. Which of the following is the main surgical treatment for transposition of the great vessels?

a. Truncus Arteriosus Repair

b. Arterial Switch
c. Aortopulmonary Window Repair
d. Sano Modification of the Norwood Procedure

4. What IV medication should be given preoperatively and be continued until on

cardiopulmonary bypass?

a. Beta blockers such as labetalol

b. ACE inhibitor
c. Nitric Oxide
d. Prostaglandin E
 Questions
5. What of these is not a risk factor for transposition of the great vessels of vessels?

a. Maternal age greater than 40

b. Maternal use of tobacco
c. Maternal Diabetes
d. Maternal viral infection
 Answers to Questions
1. What is “transposition of the great vessels”?
a. Aorta is connected to right ventricle, while the pulmonary artery is connected to the
left ventricle
b. Elevated aortic and pulmonic blood pressures
c. The umbilical cord has two veins and one artery
d. Pulmonary hypertension in the neonate has forced blood to flow backwards through a
patent ductus arteriosus

2. What is the first sign a practitioner may notice if an neonate has transposition of the great
vessels?

a. Apneic periods
b. Hazy chest x-ray
c. Bluish tint around neonate’s mouth
d. Low motor tone
 Answers to Questions
3. Which of the following is the main surgical treatment for transposition of the great
vessels?

a. Truncus Arteriosus Repair

b. Arterial Switch
c. Aortopulmonary Window Repair
d. Sano Modification of the Norwood Procedure

4. What IV medication should be given preoperatively and be continued until on

cardiopulmonary bypass?

a. Beta blockers such as labetalol

b. ACE inhibitor
c. Nitric Oxide
d. Prostaglandin E
 Answers to Questions
5. What of these is not a risk factor for transposition of the great vessels of vessels?

a. Maternal age greater than 40

b. Maternal use of tobacco
c. Maternal Diabetes
d. Maternal viral infection
 References
Cladis, F., & Davis, P. (2017). Smith's anesthesia for infants and children (9th ed.). Philadelphia, Pa.: Mosby.

Elumalai, G., & Princess, J. (2016). “Transposition of great arteries” Embryological Basis and its Clinical Importance”

Goldman, L., & Schafer, A.I. (2016) Goldman-Cecil medicine (25th ed.). Philadelphia, PA: Elsevier/Saunders

Haschek, W. M., Rousseaux, C. G., Wallig, M. A., Bolon, B., Ochoa, R., & Mahler, B. W. (2013). Haschek and Rousseaux's
handbook of toxicologic pathology (Third edition. ed.). Amsterdam ; Boston: Academic Press.

Holzman R., Mancuso, T., & Polaner, D. (2016). A practical approach to pediatric anesthesia (2nd ed.). Philadelphia, Pa:
Wolters Kluwer

Transposition of the great arteries (TGA). (2018). Retrieved February 12, 2018, from
http://www.stanfordchildrens.org/en/topic/default?id=transposition-of-the-great-arteries-tga-90-P01823

Transposition of the great vessels. (2015, October 22). Retrieved February 12, 2018, from
https://medlineplus.gov/ency/article/001568.htm

Transposition of the great arteries. (n.d.). Retrieved February 14, 2018, from
https://www.rch.org.au/cardiology/heart_defects/Transposition_of_the_Great_Arteries/

Fanaroff, A., Martin, R., & Walsh, M., (2015). Fanaroff and Martin's neonatal-perinatal Medicine (10th Ed.) Philadelphia, PA:
Elsevier/Saunders