Transposition of the Great Arteries (TGA)
1. Differential Diagnoses:
a. Pulmonary Atresia With Intact Ventricular Septum
b. Tetralogy of Fallot With Absent Pulmonary Valve
c. Tetralogy of Fallot With Pulmonary Atresia
d. Total Anomalous Pulmonary Venous Connection
e. Tricuspid Atresia
f. Truncus Arteriosus (Charpie, 2013)
2. Pathophysiology: A serious, but rare, congenital birth defect in which the two main arteries that leave the heart are transposed, or reversed (Mayo Clinic, 2014). The incomplete septation and migration of the truncus arteriosus during fetal development is the causative condition. The aorta arises from the right ventricle and the pulmonary artery come from the left ventricle. As a result, the aorta receives deoxygenated blood and returns it to the arterial circulation, at the same time the pulmonary artery receives oxygenated pulmonary venous blood and return it to the pulmonary circulation (Burns, Dunn, Brady, Starr, & Blosser, 2013, p. 688).
3. Epidemiology: The occurrence of TGA has overall low prevalence in the United States; however, it is the most common diagnosed congenital cyanotic heart defect in newborns, with an estimated 5-7% occurrence in patients with congenital heart disease (Charpie, 2013). The estimated overall incidence is 20-30 per 100,000 live births. In 90% of patients who have TGA, the incident is isolated and usually is not linked to any syndrome or extracardiac abnormalities (Charpie, 2013). TGA has a male predominance of 60-70% with no racial influence (Charpie, 2013).
4. Physical exam findings: Cyanosis that is presents within one hour or within the first day of life. Congestive heart failure may be present at birth and infants may or may not have a murmur at birth. S2 is loud and single as a result of the structural placement of the great arteries (Burns et al., 2013, p. 688). Infants who also have a ventricular septal defect may not have severe symptoms or mild cyanosis until pulmonary blood volume/flow increases over the first 3-6 weeks of life (Charpie, 2013)
5. Diagnostic testing and any referrals needed:
a. Immediate pediatric cardiologist referral is imperative (Charpie, 2013).
b. Chest x-ray
c. EKG (right axis deviation and RVH)
d. Echocardiogram will show transposition as well as other defects that may exist (Burns et al., 2013, p. 688).
e. Electrolytes need to be assessed and normalized if not within normal parameters. IV prostaglandin should be given to delay closure of the ductus arteriosus (Charpie, 2013).
6. Management Plans and Follow-up:
Jatene procedure first few days of life which essentially includes rerouting the arteries to their original position, further operative procedures are dependent on additional defects that may be present (Charpie, 2013). Patients will be monitored closely throughout life and SBE prophylaxis will be recommended for life (Burns et al., 2013, p. 689). Infants who are severely hypoxic will need a balloon atrial septostomy in order to increase atrial level shunt and improve mixing of oxygenated blood (Charpie, 2013).
References
Burns, C. E., Dunn, A. M., Brady, M. A., Starr, N. B., & Blosser, C. G. (2013). Pediatric primary care (5th ed.). Philadelphia, PA: Elsevier Saunders.
Charpie, J. R. (2013). Transposition of the great arteries. Retrieved from http://emedicine.medscape.com/article/900574-overview#a0199
Mayo Clinic. (2013). Transposition of the great arteries. Retrieved from: http://www.mayoclinic.org/diseases-conditions/transposition-of-the-great-arteries/basics/definition/con-20043232
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